Thyroid
Differentiated Thyroid Cancer
Typically, well-differentiated thyroid cancer is indolent in nature and slowly progressive. It uncommonly represents a risk to the patient’s life if treated in a timely and appropriate manner. It is one of the most treatable and curable cancers. Surgery is the primary treatment. After surgery, treatment with radioactive iodine is indicated in cases with high and intermediate risk of recurrent disease. Chemotherapy and radiation therapy, which are common therapies in other cancers, are not often used in well-differentiated thyroid cancer.
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Papillary thyroid carcinoma (PTC) is the most common thyroid cancer (about 80% of all thyroid cancers). Most cases present as a painless nodule that is relatively firm. Sometimes it is found on an ultrasound or CT scan done for other reasons. The diagnosis is usually made by fine needle aspiration biopsy (FNA). Generally, PTC has an excellent prognosis.
Female sex, age under 45, small tumors (< 4cm) and tumors confined to the thyroid gland are favorable features. These tumors are generally consider a lower risk situation. Male sex, advanced age (55+), larger tumors (>4 cm) and tumors that invade into local tissues are more worrisome findings. These tumors are considered a higher risk situation. Each case is individualized, but with surgery and (when indicated) radioactive iodine treatment, the vast majority of patients do well (5 year survival rate is nearly 100%, 20 year survival rate > 90%).
Surgery is the cornerstone of therapy and frequently curative. In cases of PTC where the nodule is > 2 cm, total thyroidectomy is often recommended and appropriately treated with total thyroidectomy, although some patients may be managed with a thyroid lobectomy. New literature suggests that for tumors up to 4 cm meeting certain criteria, a thyroid lobectomy may be acceptable as treatment. However, total removal of the thyroid gland can facilitate postoperative surveillance and management. By removing the entire gland, screening with lab work and treatment with radioactive iodine pills to identify and sometimes destroy any microscopic tumor cells can be performed. By removing all thyroid tissue, a blood test (thyroglobulin) can be used to detect recurrence of thyroid cancer. Thyroglobulin is made by thyroid tissue and by thyroid cancer cells. If all thyroid tissue is removed, then the thyroglobulin level should not be detectable. If the thyroglobulin level goes up after thyroid removal, this can indicate some thyroid cancer tissue may still be present and progressing.
Click on the following links for additional discussion regarding papillary thyroid carcinoma, as well as more information regarding pre-op care, post-op care, risk of surgery, description of operation.
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FTC is the second most common type of thyroid cancer. FTC is more common in areas of iodine deficiency. It is less common in the US, representing 10-15% of all thyroid cancer. It is more common in women than men. It usually presents as an asymptomatic mass, similar to PTC. Unlike PTC, FTC it is less likely to be multifocal and less likely to spread to local lymph-nodes. When it does spread, it more commonly spreads through the blood stream (hematogenous), with frequent targets in the lungs or bone. Overall the prognosis is good, with > 99% 5 year survival with locally confined disease, down to 67% with distant disease. Unlike PTC, it is difficult to diagnose FTC on fine needle aspiration biopsy (FNA). FNA can only show that a nodule is a follicular neoplasm, which means that it is an abnormal growth involving a follicular pattern of growth. Follicular neoplasms have a risk of being cancerous in 20-30% of cases. Cancer can typically only be confirmed by removal of the thyroid lobe that contains the nodule, followed by careful pathologic evaluation of the entire specimen. Due to the extensive examination required, intraoperative examination by a pathologist (frozen section) typically cannot definitively determine whether this type of nodule is cancerous or benign. Typically, several days are needed before for a final diagnosis is reported by pathology.
Therefore, with a biopsy report of follicular neoplasm, we usually recommend either repeat biopsy with molecular testing to try to rule out malignancy, or a thyroid lobectomy for diagnosis and oftentimes treatment of this class of nodules. Proceeding with a total thyroidectomy is typically only recommended in cases confirmed as FTC that have additional thyroid nodules present, or pathologic characteristics suggesting that more advanced disease and closer surveillance are needed.
Following surgery, radioactive iodine uptake and ablation may be recommended if a total thyroidectomy had been performed. After therapy patients are given suppressive doses of thyroid medication and followed in the same manner as PTC.
More info on differentiated thyroid cancer can be reviewed on the AAES Patient Education Website.
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Hurthle cell thyroid cancer is thought to be a variant of follicular thyroid carcinoma. Overall the same diagnostic issues exist and the treatment approach is similar to that of FTC. Hurthle cell thyroid cancer may be more aggressive than FTC. It also has a tendency to metastasize through the blood, with common sites in the lungs, bones, and brain. Hurthle cell cancer often does not respond to radioactive iodine uptake and ablation like PTC or FTC, and therefore, detection and treatment of distant or recurrent Hurthle cell carcinoma may be challenging due to unique feature of this tumor.
Current survival estimates for Hurthle cell carcinoma is 85% at 5 years and 71% at 10 years, although the details of any single patient’s specific disease characteristics and tumor behavior will be more important to consider in their plan for monitoring and treatment.
More information on the management of Hurthle cell carcinoma can be reviewed on the AAES Patient Education Website.
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Incidental thyroid cancer is a term applied to a small, previously unidentified thyroid cancer (typically papillary thyroid carcinoma) found on pathologic examination of thyroid tissue removed for benign disease. Frequently, this type of thyroid cancer is < 1 cm, which is known as micro papillary thyroid carcinoma. Our discussion on this page is an effort to help our patients understand the implication of this finding.
Autopsy studies tell us that 3-4% of the adult population harbors micro papillary carcinoma of the thyroid without apparent ill effect. Approximately 1 in 15 patients who undergo thyroidectomy for benign disease are found to have an incidental 1-2 mm focus of thyroid cancer on careful pathologic examination. Based on extensive clinical experience the vast majority of these incidental carcinomas are not thought to be clinically relevant and cause no discernible harm to the patient.
Pathologist don’t consider this entity to be different from larger tumors (> 1 cm) but surgeons and other clinicians do. Papillary cancer greater than or equal to 1 cm has a possibility of spread to surrounding tissue, including lymph nodes and distant metastasis. While more aggressive behavior is occasionally seen in small thyroid cancers, typically a mass < 1 cm (certainly those < 5 mm) have a low risk of local or distant spread of disease.
Typically, patients with a micropapillary thyroid cancer with no other disease can be observed following thyroid lobectomy alone. If there is additional structural disease identified by ultrasound, it will need to be evaluated and possibly biopsied to rule out additional foci of thyroid cancer.
As mentioned above, occasionally a tiny focus of thyroid cancer can still represent the initial diagnosis of more serious and widespread disease, so careful evaluation of your particular situation by a qualified physician is important for your follow up.
In conclusion, incidentally noted micro papillary cancer found on pathologic inspection of thyroid tissue removed for benign disease often does not require further treatment. Each case should be considered individually. All patients should be followed yearly with careful physical exam. Whether ultrasound screening during follow-up is helpful or cost effective is unknown but seems a reasonable approach to consider.
Medullary Thyroid Cancer
Medullary Thyroid cancer (MTC) is considered along with other well differentiated thyroid cancers, but is different in that the cells of origin are the para-follicular cells. These are cells in the thyroid gland that are not involved in making thyroid hormone. Para-follicular cells make a hormone called calcitonin. It may play a role in calcium regulation. The importance of calcitonin in humans is unknown. MTC only accounts for about 5% of thyroid cancer, but up to 25% can be associated with an inherited mutation that is passed from parents to children. In addition to MTC, these mutations can predispose patients to a variety of other tumors. For this reason, germline genetic testing is often considered in patients diagnosed with MTC.
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Medullary Thyroid cancer(MTC) is considered with other well differentiated thyroid cancers, but is different in that the cells of origin are the para-follicular cells. These are cells in the thyroid gland that are not involved in making thyroid hormone. Para-follicular cells make a hormone called calcitonin. It may play a role in calcium regulation. The importance of calcitonin in humans is unknown. MTC accounts for about 5% of thyroid cancer. Generally it occurs equally between men and women.
There are two forms of MTC, hereditary and sporadic. About 25% of MTC is inherited, usually as a part of a Multiple Endocrine Neoplastic syndrome (MEN 2). When MTC is diagnosed the patient should be evaluated for an inherited syndrome. Some syndromes have an associated tumor called a pheochromocytoma that needs to be managed prior to surgery on the thyroid. If that evaluation is negative then MTC is considered to be sporadic. Sporadic MTC usually presents as a solitary nodule while the inherited form is multi-centric and typically present at a younger age.
MTC is treated with a total thyroidectomy with a central neck (level 6) lymph node dissection. Metastatic disease is more common with MTC and its prognosis is worse than other well differentiated thyroid cancers. Your preoperative tumor markers (calcitonin and CEA) level may suggest the risk of metastatic disease. Calcitonin levels less than 40 are associated with a lower risk of lymph node metastasis. Calcitonin levels greater than 150 should be evaluated for potential distant metastasis. Since MTC does not absorb iodine, radioactive iodine is not effective in its treatment and surveillance. However, the combination of surveillance labwork and structural imaging has been the mainstay of surveillance following surgical removal.
Newer management of MTC includes the detection of a somatic mutation called RET (rearrangement during transfection). If this is specifically present in the tumor, it can be targeted by a newer class of multikinase inhibitors directed towards that mutation. Selpercatinib (Retevmo) is one of those medications which has had very promising effect on this class of tumors with this particular mutation.
You can read more about MTC in the AAES website.
Anaplastic Thyroid Cancer
Anaplastic thyroid carcinoma is a rare, very aggressive, and frequently lethal form of thyroid cancer that primarily afflict elderly patients with untreated forms of differentiated thyroid cancer. It is thought that the accumulation of mutations over time ultimately leads to the changes that create this very aggressive and almost universally lethal form of thyroid cancer. Fortunately, this only comprises about 1.7% of all thyroid carcinomas, occurring in about 1-2 patients per 1,000,000.
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Anaplastic thyroid carcinoma is a rare, lethal form of thyroid cancer that primarily afflict elderly patients with untreated forms of differentiated thyroid cancer. It is thought that the accumulation of mutations over time ultimately leads to the changes that create this very aggressive and almost universally lethal form of thyroid cancer. Fortunately, this only comprises about 1.7% or all thyroid carcinomas, occurring in about 1-2 patients per 1,000,000.
The median length of survival for patients diagnosed with anaplastic thyroid carcinoma is 5 months. The 1 year survival is approximately 20% according to the 2012 American Thyroid Association Guidelines for Management of Patients with Anaplastic Thyroid Carcinoma, although this is possibly an overestimate.
Unlike differentiated thyroid cancers like papillary thyroid carcinoma and follicular thyroid carcinoma, anaplastic thyroid carcinoma has changed so much it does not resemble thyroid tissue at all, even when viewed under the microscope. Also, unlike management of differentiated thyroid cancers, the management of anaplastic thyroid carcinoma requires rapid diagnosis and treatment to obtain maximal benefit from therapy, as it grows very quickly.
The rarity and rapid progression to lethality has made development of treatment options difficult, as the coordination and implementation of plans from multiple providers can be a difficult task to perform quickly.
The first thing that needs to occur is for there to be adequate suspicion for this rare disease to warrant evaluation by a specialist that is familiar with this rare illness and can guide you quickly through the diagnosis and treatment options.
Signs and symptoms concerning for anaplastic thyroid carcinoma
Age > 65; it is rare for patients to be younger than 50 years old
Known or history of existing thyroid cancer like papillary, follicular, or Hurthle cell carcinoma
New onset rapid enlargement of neck mass
New onset or worsening symptoms of hoarseness, choking, pain, or difficulty breathing
Many other things can cause the above symptoms and are much more common. However, it is worth considering further evaluation if these symptoms persist or worsen despite standard treatment of the other causes, particularly if there is a neck mass associated with these symptoms. If there is enough suspicion of a rapidly progressive cancerous process, you will need imaging, diagnosis (by biopsy), and possibly surgery, radiation therapy, chemotherapy, or all of the above, based on how advanced the disease process is at diagnosis. It is best to establish the diagnosis in advance of surgery, but sometimes it is reasonable to proceed with surgery for diagnostic purposes.
You may require imaging in the form of ultrasound, CT scan, and PET scan before and/or after surgery. If you are a candidate for surgery, you will likely require laryngoscopy in advance of surgery to establish if your vocal cords are working normally prior to surgery. You may also be asked to undergo upper endoscopy or bronchoscopy to evaluate your esophagus or trachea for evidence of invasion prior to proceeding to surgery
The surgical procedure can be very extensive, including removal of any of the adjacent structures near the thyroid gland. These can include removal of parathyroid tissue, muscle, portions of the trachea, larynx, jugular vein, or other structures. You may require prolonged intubation or tracheostomy as a result of the surgery. You may require the placement of a feeding tube during or after surgery. The details of what may be required for removal, as well as a discussion of the relative benefit of extensive surgery, are individual decision points that depend on the extent of your disease and should be discussed with your surgeon prior to proceeding with surgery.
If the tumor can be removed without unreasonable morbidity, you will likely be offered radiation and/or chemotherapy after surgery. This is called adjuvant therapy.
Occasionally, if there is disease that will make surgery very difficult, you may be asked to undergo radiation and/or chemotherapy first, and then be reassessed for possible surgical removal. This is called neoadjuvant therapy.
More frequently, if the tumor is not surgically removable, you may be offered radiation and/or chemotherapy to try to slow progression of the disease around the neck and other distant sites. This is called palliative therapy.
There are many unique aspects of each patient’s disease such that any once care plan and recommendations will likely differ from another.
Regardless of any of the above scenarios, if diagnosed with anaplastic thyroid carcinoma, consultation with a palliative care specialist – a physician and medical team that specializes in reviewing end-of-life planning, is advisable due to the lethality and rapid progression. They will provide support and guidance, as well as a clear outline of the different things that can be done throughout the course of the illness.
Thyroid lymphoma
Thyroid lymphoma is another aggressive and rare form of thyroid cancer which is often considered along with anaplastic thyroid cancer when evaluating a large, rapidly growing thyroid mass. It is most often a form of B cell lymphoma. It occurs in with a frequency of 1-2 cases per 1 million. It is often seen in association with Hashimoto’s thyroiditis. Rapid diagnosis of this disorder is needed as the treatment is not surgical, but primarily chemotherapy and radiation. When correctly diagnosed and treated, survival rates are good, at 75% and higher 5 years after diagnosis.
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Thyroid lymphoma is a rare but rapidly progressive thyroid cancer which can present in a manner oftentimes resembling anaplastic thyroid carcinoma. A classic presentation of this disorder is of a rapidly progressive and newly symptomatic thyroid mass in the setting of longstanding autoimmune thyroiditis (Hashimoto's thyroiditis). It has an increased frequency among women, as well as in later ages, greater than 60 years old. Evaluation of thyroid lymphoma is much the same as evaluation of other thyroid disorders. Physical examination, a careful history, ultrasound, and biopsy or all instrumental in the diagnosis of this condition. Often times a core needle biopsy or surgical biopsy is necessary to provide the information for full identification of the type of lymphoma, as this will affect the treatment regimen. Additional imaging such as CT scan and special lab tests, as well as PET scan can be helpful in determining the extent of disease once a diagnosis has been made.
The primary treatment of thyroid lymphoma is not surgical, but rather involves chemotherapy and external beam radiation. On occasion, surgery may be recommended, but typically this disorder is managed very effectively with radiation and chemotherapy as the primary modes of treatment.
More information about thyroid lymphoma can be obtained on the AAES Patient Education website.
Thyroid Nodules
Thyroid nodules are a very common cause of referral to our practice. Thyroid nodules are common in the general population. While exact numbers are unknown, estimates indicate that about a third of the population will develop thyroid nodules between the ages of 20 and 50, half of the population will develop a thyroid nodule by 60 years old. Thyroid nodules are much more common in women than in men, occurring 4-5 times more frequently. They are more frequent following exposure to ionizing radiation and in settings of iodine deficiency.
Most will initially be identified on physical examination by a medical provider, or on self-examination by patient. However, ultrasound has provided a tool that substantially increases the sensitivity of detecting small, nonpalpable thyroid nodules easily.
About 5% of thyroid nodules being identified as malignant. 3-5% of patients with thyroid hyperfunction can be attributable to a thyroid nodule which is producing too much thyroid hormone (toxic thyroid nodule). The majority of thyroid nodules are nonfunctioning and benign. However, determining whether the nodule is benign or malignant, functional or nonfunctional, or whether it needs to be treated requires evaluation by a knowledgeable specialist.
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Significant thyroid nodules are solid and larger than 1 cm (about 1/2 inch) in greatest dimension. Thyroid nodules are very common and are not usually cancer. Small nodules may come and go. Generally, nodules over 1-2 cm have about a 20% chance of enlarging over time Some nodules may have both solid and fluid components. These are called complex cystic nodules. These should be evaluated carefully in a manner similar to solid nodules. Entirely fluid filled nodules are called simple cysts. Simple cystic masses are not malignant but can be symptomatic and require treatment.
All new thyroid nodules should be evaluated by an experienced physician. From a surgical standpoint, once a thyroid nodule is discovered 3 question should be answered.
Is it over functioning?
Is it cancer?
Is it a source of local symptoms?
Is it over functioning? This is usually answered by checking a thyroid function test called TSH (Thyroid Stimulating Hormone) level. An elevated TSH level indicates decreased thyroid function. A decreased TSH level indicates elevated thyroid function. If there is evidence of over function, then nuclear medicine test called a thyroid uptake scan is recommended. If an overactive nodule (toxic nodule) is found, treatment options for thyroid hyperfunction can be discussed. These include: medical therapy with anti-thyroid medication, and possibly radioactive iodine ablation (RAIA). In selected situations surgical removal or radiofrequency ablation of the nodule may be appropriate. An overactive nodule is rarely malignant. If the nodule is not over active (hot), then the next two questions become important.
Is it cancer? This question is best answered by a fine needle aspiration biopsy (FNA). The biopsy result provides direct sampling of the nodule, and is the gold standard for identifying cancerous cells in the thyroid. If the biopsy suggest cancer then surgery is indicated. If it is benign then it may be appropriate to observe the nodule. We will talk about FNA and thyroid cancer in greater detail on other pages. If the nodule is not over active and the FNA indicates it is not cancer, then the third question becomes important.
Is it a source of local symptoms? If the patient is having local symptoms thought to be due to the compressive effects of the nodule, then surgery may be indicated. These symptoms can include: choking, hoarseness, voice change, pressure, globus (a feeling that there is something constantly caught in the throat), pressure with lying flat, looking up, or reaching up, or difficulty breathing. Additionally, if the patient has no compression related symptoms, but the mass is large and visually unsightly, surgical intervention can be very helpful in resolving the issue.
If the answer to all three of these questions is “no” then the nodule can usually be watched. About one third of nodules less than an inch shrink in size. If a nodule increases in size, it may require biopsy again or surgery may need to be considered. Surgical intervention is appropriate if there is a question of cancer, if the nodule is causing local symptoms and in selected cases of an over functioning nodule.
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Thyroid ultrasound provides an experienced practitioner with extremely accurate and reproducible imaging at the point of care to help with diagnosis and treatment of a number of conditions. Ultrasound is performed in the office. For ultrasound to be performed effectively, the neck should be easily accessible and exposed. Please wear loose fitting clothing around the neck to provide easy access to the neck for ultrasound. Ultrasound gel is applied to the neck and the linear ultrasound probe is placed on the neck to evaluate for structural abnormalities related to the diagnosis in question. This could include thyroid masses, lymph nodes, parathyroid abnormalities, or other structural defects in question. Thyroid ultrasound is so effective at detecting abnormalities related to your condition that it is an essential component of your physical evaluation, and needs to be performed in-person, which is the main reason an in-person visit is necessary for initial consultation.
The TI-RADS scoring system through radiology has recently been employed to try to separate thyroid nodules into ones that can be safely observed versus ones that should be biopsied. This system was developed around 2017 and relies upon sonographic features which restored by an ultrasound technologist. The aggregate score will help to stratify the nodule from the standpoint of necessity to biopsy. More information about this system is located on this website regarding the TI-RADS scoring system. The specific goal of the thyroid system was to try to decrease the number of necessary biopsies of thyroid nodules, and decrease patient discomfort. However, careful evaluation by an experienced practitioner is still necessary to truly understand the potential risk of malignancy, and while the TI-RADS system has been helpful in clarifying some elements of malignant risk related to a thyroid nodule, is the opinion of our practice that direct evaluation by thyroid specialist is still warranted when the nodule is identified.
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FNA is a common procedure done in the office with local anesthesia. It is the best way to diagnose thyroid cancer. Its accuracy approaches 95%. A small amount of local anesthetic is injected into the skin and the underlying tissues. This is typically the most uncomfortable part of the procedure. A small needle is inserted into the nodule usually under ultrasound guidance. There is a mild aching sensation when the biopsy is taken. The actual procedure takes only a few minutes. Patients tolerate it well. Most patients comment that the worse part is the anxiety about the procedure not the discomfort. Some bruising may occur but significant issues with bleeding are rare. Any mild residual discomfort is easily handled with ice and tylenol. Narcotic pain medication is not required. Be sure to tell us if you are on blood thinners. Aspirin, plavix or aggrenox are okay to continue, but coumadin, Xarelto, Eliquis, and other medications in the anticoagulation class should be stopped before your biopsy to avoid excessive bleeding.
Following your biopsy, we will need to discuss one of four possible results:
The biopsy was likely benign.
The biopsy indicates malignancy was present or highly suspected.
The biopsy was indeterminate.
The biopsy was non-diagnostic (inadequate specimen collection). Not enough material was obtained to allow the pathologist to provide a diagnosis.
These results can be confusing and should be discussed with your physician to help decide how best to proceed.
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Once your biopsy result has returned, we will arrange for an appointment to review the results in detail, as well as to map out a plan of care with regard to your particular nodule and its pathology.
The pathologist assigns the results of a needle biopsy to one of 6 categories:
Category 1: non-diagnostic (inadequate collection of material for examination)
Category 2: very likely benign
Category 3: atypia of undetermined significance (approximately 10-30% of cancer)
Category 4: follicular neoplasm (approximately 20-40% risk of cancer)
Category 5: suspicious for malignancy (60-80% risk of cancer)
Category 6: positive for thyroid cancer (>99% risk of cancer)
Management of each of these entities depends on the specific pathology that was identified, the risk of cancer, the size of the nodule, local symptoms that you are experiencing, additional pathology present in the remainder of the thyroid gland, age, general health, and a multitude of other factors that require careful consideration before embarking on a specific plan. This discussion is best had in person as a lot of information is exchanged and patient-specific considerations are clarified.
Typically, with diagnoses of thyroid cancer, thyroid lobectomy and total thyroidectomy, both with or without central compartment lymphadenectomy are options for management. The specific options will depend on the type of thyroid cancer encountered and distribution of pathology within the thyroid gland as well as adjacent lymph nodes.
Additional testing of indeterminate nodules (Bethesda category 3 and Bethesda category 4) can be performed to clarify the potential for malignancy. This is done through additional biopsy obtained material and sent to the lab for molecular testing. There are number of different companies which perform this molecular testing at our request upon notification of an indeterminate result. These companies are Afirma, Thyroseq, and ThygeNEXT/ThyraMIR. Our preferred tests are through Afirma and Thyroseq. These can help to clarify decision making regarding surgery versus observation in these categories of thyroid nodules by giving a better understanding of the potential risk of malignancy. In typical practice, this type of test is most useful in a patient with a smaller sized indeterminate thyroid nodule with no local symptoms, no additional pathology, and strongly seeking to avoid surgical intervention. These tests are helpful in narrowing the relatively wide reported range of malignancy to a more concrete number, but they cannot eliminate the risk of malignancy.
For more information regarding these tests, read this American Thyroid Association Bulletin on molecular testing.
Autoimmune Thyroid Disease
Autoimmune thyroid disease is a broad category of conditions in which your immune system has misidentified a part of your thyroid gland as a target for attack. The target of attack will determine if you become hypofunctional (Hashimoto's thyroiditis) or hyperfunctional (Graves' disease). Both of these states can lead to significant symptoms and illness if left untreated.
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Hashimoto’s Thyroiditis (chronic lymphocytic thyroiditis) is an autoimmune thyroid condition of the thyroid gland. It is a common cause of goiter and probably the most common cause of hypothyroidism in the United States. It is relatively common, affecting up to 5% of the population, and the cause is unknown. It affects women more frequently than men, and the frequency of occurrence increases with age. Its overall prevalence has been increasing over time for unknown reasons.
Hashimoto’s thyroiditis is an autoimmune disease. What this means is that your immune system has misidentified a portion of the thyroid gland, thinking it is a foreign invader. As result, immune cells proliferate and attack the thyroid gland, most frequently targeting the thyroperoxidase protein, but occasionally also targeting thyroglobulin or other protein components of the thyroid glandular structure. Typically, the resultant damage to the thyroid gland leads to local inflammation, scarring, contraction, and loss of function over time. The thyroid gland can develop a very abnormal appearance as it continues to be injured and regenerate over time. Nodules and pseudo nodules (structures that look like nodules, but are just the result of local damage) can develop within the thyroid gland over time as well. Previous teaching was that there was no increase in risk of malignancy associated with Hashimoto's thyroiditis, but recent retrospective studies indicate that the risk of thyroid cancer within a nodule with Hashimoto's thyroiditis is about 60% or greater than the general population risk. For this reason, thyroid nodules are frequently biopsied and followed carefully in Hashimoto's thyroiditis.
The diagnosis of Hashimoto’s thyroiditis is made by a combination of clinical findings and labwork. Patients may oftentimes have vague complaints of fatigue, brain fog, malaise, as well as local complaints of tenderness of the neck, enlarged and palpable lymph nodes, dysphagia (difficulty with swallowing), globus (feeling of something caught in the throat), requiring excessive clearing of the throat, choking, hoarseness, and a number of other vague and nonspecific complaints that are not otherwise remedied through standard means. Typically, the TSH level can be abnormal in Hashimoto's thyroiditis. Early on in the natural history, the TSH number may be very small, indicating thyroid hyperfunction (or too much thyroid hormone). In the middle portion of its natural history, the TSH number may be normal. Towards the end of its natural history, the TSH number becomes higher than the upper limit of normal, indicating hypofunction (or too little thyroid hormone).
If there is enough clinical suspicion for Hashimoto's thyroiditis, additional lab work can be performed. In addition to thyroid function studies, checking for the presence of thyroid autoantibodies such as anti-thyroperoxidase and anti-thyroglobulin antibodies can confirm the diagnosis. While elevation in these antibodies confirms the diagnosis, antibody elevation is not always present in this condition.
Treatment
Typical recommendation regarding the management of Hashimoto's thyroiditis included checking thyroid function and starting thyroid hormone replacement therapy when thyroid function is found to be declining by lab testing, and there is clinical evidence of hypothyroidism. This will help normalize any feelings of unwellness related to the majority of patients with Hashimoto's thyroiditis. However, Hashimoto's thyroiditis results in a multitude of symptoms which really seem to be related to 3 categories.
First, there is loss of thyroid function as indicated above. This is identified through lab work and clinical evaluation. This is remedied by the addition of thyroid hormone to your medication regimen.
Second, there are local symptoms of irritation and compression that are related to the inflammatory nature of this condition. When the thyroid gland becomes injured by the immune system, the adjacent tissue also becomes inflamed and affected by this tissue injury cycle. The thyroid itself changes in its appearance and texture, from a softer, more compliant structure into a hard noncompliant and contracted gland. This transition can lead to local symptoms of irritation such as hoarseness, as well as compressive and restrictive symptoms such as choking, difficulty breathing, difficulty swallowing, throat clearing, and a feeling of constant pressure in the neck, or as if something was caught in the throat.
Third, there are generalized symptoms of malaise and general unwellness, including symptoms of fatigue not corrected by thyroid hormone replacement, brain fog, lassitude, body aches, and other systemic symptoms not explained or treated by any other known diagnosis. This is understandably a very vague category of complaints, but in patients with significantly elevated thyroid autoantibodies, there seems to be relationship between the degree of immunologic response to the thyroid gland and the appearance of these vague symptoms which seem to progress and decrease the quality of life with no clear source or treatment plan.
While Hashimoto's thyroiditis and its most mild form typically leads to loss of thyroid function, there is an increasing frequency of local and generalized symptoms related to this disorder. Removal of the entire thyroid gland can oftentimes help with the symptoms by removing the heightened immunologic response related to the thyroid gland. However, this decision to proceed with surgery should be carefully reviewed with the patient, with care to explore other causes of symptoms that may be attributed to Hashimoto's thyroiditis, but are actually related to other known or undiagnosed medical conditions.
Generally, surgery is frequently avoided in Hashimoto’s thyroiditis unless there is good reason to intervene. The inflammatory response of the disease may lead to slightly increased risk of recurrent nerve injury and post-op hypoparathyroidism when surgery is required. When surgery is required, total thyroidectomy is typically recommended. When the burden of symptoms and illness related to Hashimoto’s thyroiditis from its loss of function, local compressive symptoms, and systemic symptoms grow to substantially interfere with the quality of life, surgery should be considered.
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Patient states that an autoimmune disorder involving autoantibodies directed towards the thyroid receptor which activates growth and production of thyroid hormone from the thyroid gland. In a manner similar to that seen in Hashimoto's thyroiditis, the immune system mistakingly identifies the receptor mechanism as a target for attack, producing autoantibodies which triggered the mechanism and the thyroid to increase growth and production of thyroid hormone. The end result is significant thyroid hormone excess with an enlarged diffuse goiter and a multitude of symptoms related to both of these issues. The classic presentation of Graves' disease is of a young woman with new onset of sweating, rapid heart rate, palpitations, anxiety, weight loss, with tremor, bulging eyes, and elevated pulse at rest. This malady does affect women more frequently than men, but can affect both.
The diagnosis is made by checking thyroid function which oftentimes reveals significant suppression of the TSH lab value to an undetectable level, along with substantial elevation of the free T4 and free T3 levels. Autoantibodies towards the thyrotropin receptor site (TRAb) can be detected, confirming the diagnosis.
Graves' disease is initially managed by controlling the hyperfunctioning thyroid state with medications. The medication of choice is methimazole, although propylthiouracil (PTU) can be used early in pregnancy, or by patients if methimazole is not tolerated. Addition of a beta-blocker medication to help with rapid heart rate and high blood pressure can also be very helpful. Spontaneous normalization of thyroid function can occur, with the disappearance of thyroid autoantibodies. However, if thyroid hyperfunction persists, patient will need to continue on antithyroid medications such as methimazole or PTU, and sometimes a beta-blocker medication to help control symptoms.
If symptoms of thyroid hyperfunction or attaining a euthyroid state proves to be challenging, definitive management by eliminating the thyroid gland is indicated. The 2 modalities for definitive management of the thyroid are I-131 radioactive iodine ablation and total thyroidectomy.
Radioactive iodine has the benefit of avoiding surgery. However, there are some contraindications towards administering radioactive iodine. It should not be administered to pregnant patients or patients were hoping to become pregnant within the next 12 months. It can worsen thyroid eye disease, and therefore is avoided in patients with significant thyroid eye disease. The normalization of thyroid function can take some time to occur, rather than surgical removal of the thyroid gland, which has a more rapid normalization of thyroid function. It may also require more than 1 dose to manage very large diffuse goiters related to Graves' disease. Finally, if there are nodules with any concerning features, radioactive iodine typically does not manage these nodules well unless the nodules are also hyperfunctioning.
Total thyroidectomy is the surgery of choice for Graves' disease. It has the benefit of rapid normalization of thyroid function. Additionally, there is no negative effect towards the progression of thyroid eye disease, and can have substantial improvement in resolving thyroid eye disease, which is thought to be related to the amount of circulating thyroid autoantibody present in the body. The risks of thyroidectomy for Graves' disease include bleeding, nerve injury, hoarseness, and loss of parathyroid function. These of the same risks incurred by thyroidectomy for any reason, but their frequency can be higher in the setting of Graves' disease due to large inflamed goiters, hypervascularity, and overall a more technically challenging operation. The decision to proceed with thyroidectomy for Graves' disease also requires good preoperative preparation to ensure excellent outcomes, in addition to finding a competent an experienced surgeon who is comfortable managing your situation.
Goiter
Goiter is a lay term used to describe an enlarged thyroid gland. This could be related to a nodule, diffuse enlargement, cancer, Graves' disease, hyperfunction, Hashimoto's thyroiditis, etc. Most typically, the term goiter is used in reference to indicate a diffusely enlarged gland oftentimes with multiple nodules.
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Multi-nodular goiter (MNG) is a medical term describing an enlarged, lumpy thyroid gland. While iodine deficiency is a common cause world-wide, this is not true in the US. In the US, the cause is usually multifactorial and, in a given individual, frequently hard to determine. It is more common in women than men. It may run in families. Most goiters are not a cause of symptoms and don’t require any treatment. When a goiter is found three questions need to be answered.
Is it cancer? The vast majority of MNGs are not cancer. In an otherwise uncomplicated goiter the risk of cancer is not significantly greater than in a patient with a non goitrous thyroid gland. Multiple nodules are usually present. Large nodules more than 1/2 inch in size should be considered for biopsy (FNA). A rapid change in size of the goiter or nodule may suggest malignancy but usually does not. These findings should be evaluated by an experienced physician to exclude cancer.
Is the function of the thyroid gland normal? Most goiters do not affect the function of the thyroid gland. However a goiter may secrete too much thyroid hormone. This is called a toxic goiter. An I-131 thyroid scan may be indicated to help evaluate this. Frequently, a toxic goiter is treated surgically. On occasion it may be treated medically. If surgery is indicated then control of the over functioning gland is required pre-operatively. This is usually done with anti-thyroid medication. In other situations the function of the gland is under-active and this is commonly treated with thyroid hormone replacement therapy. Unless the goiter is causing local symptoms, surgery is not usually required when the goiter is under active. In patients with variation in the function of the thyroid, evaluation by an endocrinologist or experienced primary care physician may be indicated.
Is the goiter causing local symptoms? A goiter may grow large enough to compress adjacent structures like the airway or swallowing tube(esophagus). Patients may experience difficulty swallowing, choking sensation, voice change or difficult breathing. Commonly the symptoms are worse when lying down.
If the answer to either question one or three is “yes “ then surgery may be appropriate. If the answer to question two is “yes” then an evaluation by an endocrinologist should be considered. If a toxic goiter is found then medical therapy with anti-thyroid medication and/or radioactive iodine may be appropriate. Surgery may also be indicated. Generally, if cancer can be excluded and the goiter is not causing local symptoms and there is no alteration in function of the thyroid gland then observation is usually recommended.
Occasionally the use of thyroid hormone in pill form is recommended to try to reduce the size of the goiter. This involves giving slightly higher doses of thyroid hormone than would normally be present in the body in an effort to reduce stimulation of the thyroid from the pituitary gland( the gland that regulates the thyroid). Unfortunately this approach doesn’t work well in many clinical situations and there are potential side effects of giving above normal doses of thyroid hormone. For these reasons, in simple goiters, thyroid hormone therapy is not commonly recommended. Hashimoto’s thyroiditis, a chronic inflammatory condition of he thyroid gland, and a common cause of MNG, may be effectively treated with thyroid hormone.
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The term toxic thyroid nodule first to the thyroid nodule which is producing too much thyroid hormone. This is referred to as Plummer's disease. This is initially diagnosed with suppressed TSH and elevation in T4 and T3, suggesting thyroid hyperfunction. This lab derangement in association with symptoms of hyperfunction indicates symptomatic hyperthyroidism. An ultrasound will oftentimes reveal nodules within the thyroid gland, some of which are hypervascular. A thyroid uptake scan performed through nuclear medicine can confirm the diagnosis of a single or multinodular thyroid gland with toxic (hyperfunctioning) thyroid nodules.
Typically, this is managed by control of thyroid hyperfunction through the use of antithyroid and beta-blocker medications if symptomatic, as well as radioactive iodine ablation or surgical removal if the nodule persists and its hyperfunction, and becomes difficult to manage, or if definitive management is desired by the patient.
Radiofrequency ablation of the toxic thyroid nodules can also be an effective management strategy to destroy the hyperfunctioning nodule and restore normalization of thyroid function. Not all patients are candidates for this option, and limitations to the durability of normalization of function related to RFA versus surgical removal are important considerations.
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Substernal goiter refers to enlargement of the thyroid gland which descends into the thoracic cavity. The thyroid gland can easily drop into the chest through the thoracic inlet and grow to become enormous. While substernal goiter is can be symptomatic, oftentimes they are not until they become very large. Once the goiter begins to grow into the chest, it should be removed as continued progression in size will typically lead towards enlargement within the chest. Substernal goiters may require a transthoracic approach to safely remove the goiter. This may require a median sternotomy or thoracotomy. More recently, surgical techniques permitted removal of very large substernal goiter is through an entirely cervical approach. If an approach to the lateral chest is required, the use of thorascopic approaches and robotic thorascopic surgery have minimized the extent of surgical exposure required to safely perform this operation. However on occasion, full or partial sternotomies and thoracotomies may still be required to safely remove massive goiter involving the thoracic cavity. We recommend a careful discussion with an experienced thyroid surgeon to discuss all of the options in advance of proceeding with surgery.
Parathyroid
Hyperparathyroidism
Hyperparathyroidism refers to the excess production of parathyroid hormone (PTH), which has multiple effects on the body. Why this is happening is important to understand in offering the correct treatment for a condition involving parathyroid hyperfunction
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The parathyroid glands are located in the neck behind the thyroid gland. They are separate glands from the thyroid. Generally, there are four glands. Two on each side, in the superior and inferior position. Normally they are about the size of an apple seeds.
The parathyroid glands make a hormone called parathyroid hormone (PTH). This hormone regulates the calcium level in the blood. About 98% of calcium is in the bone, about 2% is in the blood stream. In the bone, calcium helps with structural support. In the blood, it mediates normal nerve conduction and muscle contraction, and within cells it is involved in intracellular signaling and messaging. Calcium is a very important mineral, which is why the level in the bloodstream is so closely regulated. PTH is involved in this regulation, and in the normal state, causes movement of calcium from the bones into the blood in response to low calcium in the blood. As the calcium level in the blood increases, this is detected by calcium sensing receptors which then decrease the amount of PTH being secreted into the bloodstream. The PTH level drops and the calcium level stops rising. This works much like how the temperature in your house is maintained by the interaction between your thermostat and your furnace to keep your home temperature within a certain range.
Parathyroid glands can be important from a surgical standpoint if they become over active. When this happens, too much PTH is produced and this leads to movement of calcium from the bones into the blood stream. The calcium level in the blood becomes elevated. Over time this can result in a significant loss of calcium in the bones (osteopenia and osteoporosis). Elimination of excess calcium from the blood occurs through the urine, which can lead to elevated calcium in the urine, increased risk of forming kidney stones and loss of renal function.
A variety of non-specific symptoms may develop including severe fatigue, bone aching, muscle weakness, poor mental processing (sometimes so severe it may be confused with dementia, Alzheimer’s disease, and other similar disorders), depressed mood, abdominal pain, hyperacidity, ulcers, and elevated blood pressure. Chronic increased PTH production (hyperparathyroidism) has been associated with an increased risk of kidney failure and increased risk of death from heart disease.
There are 3 types of hyperparathyroidism:
Primary hyperparathyroidism
Secondary hyperparathyroidism
Tertiary hyperparathyroidism
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Primary hyperparathyroidism (1°HPT) is an elevated PTH level cased by a primary problem with the parathyroid gland itself. That is to say, an overgrowth of abnormally functioning parathyroid cells causes too much PTH to be produced. This happens because the calcium sensing receptors are reduced and calcium particles can’t turn off the parathyroid glands. Therefore the glands are always” turned on” and constantly produce too much PTH leading to the health problems we discussed earlier. Genetic abnormalities in the parathyroid cells cause a decrease in these receptors and a overgrowth of abnormally functioning cells. If this happens to only one gland it is an adenoma. If all the glands are overgrown it is called hyperplasia.
There are a number of different presentations of this condition, including:
Single adenoma
Multigland hyperplasia
Ectopic parathyroid gland
Recurrent/persistent disease
These processes are much more involved, and are covered in more detail on our page for Parathyroid Disease.
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Secondary hyperparathyroidism (2°HPT) is overactive parathyroid glands due to conditions in the body which promote the hyperfunction of the parathyroid glands. The most common causes are kidney failure and vitamin D deficiency, but in patients with malabsorption from other medical conditions or prior surgery (such as Roux-en-Y gastric bypass or duodenal switch) 2°HPT is characterized by low normal or normal calcium levels and elevated PTH levels. This is different from primary hyperparathyroidism (1°HPT) which is characterized by elevated calcium in association with elevated PTH levels.
With kidney failure a variety of changes occur in the body’s physiology that result in low calcium levels, low vitamin D activity and elevated phosphate levels. These alterations stimulate the parathyroid glands to release PTH. This chronic stimulation leads to enlargement of all the glands (hyperplasia). Usually, 2°HPT due to kidney failure is controlled with diet, medication and dialysis. Selected patient with symptoms of severe fatigue, bone pain, difficult to control hyperphosphatemia and PTH levels greater than 1000 are considered for surgery. This decision is made in consultation with the surgeon and nephrologist (kidney specialist).
Low vitamin D is another common cause of 2°HPT. A detailed discussion of vitamin D physiology is beyond our scope. Simply, vitamin D leads to increased calcium levels and a deficiency in vitamin D results in low calcium which then stimulates the parathyroid glands. Again, the calcium levels are in the low normal to normal range and the PTH levels are elevated. Treatment with vitamin D reverses these problems. Surgery does not play a role in treatment of this kind of 2°HPT.
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Tertiary hyperparathyroidism (3°HPT) develops in some cases of long standing 2°HPT when prolonged hypocalcemia and parathyroid stimulation drives the development of independently functioning parathyroid glands. Hypercalcemia develops and again the PTH levels are significantly elevated, much in the way this balance is disrupted in primary hyperparathyroidism. This situation occurs most commonly in patients with chronic renal failure. The classic situation occurs when a patient with kidney failure (and 2°HPT) receives a kidney transplant. The abnormal physiology that leads to 2°HPT is corrected with the transplant but some of the hyperplastic parathyroid glands have developed autonomous function after years of hyperstimulation. Treatment is typically surgical. A neck exploration is performed and 3 to 3-1/2 glands are removed, allowing for normalization of parathyroid function and calcium. The cure rate approaches 98%. In this scenario, the improvement in calcium and PTH can lead to improved quality of life and increased kidney transplant survival.
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Item description
Secondary Hyper- parathyroidism
Secondary hyperparathyroidism (2°HPT) is overactive parathyroid glands due to extra – parathyroid factors that stimulate otherwise normal parathyroid glands to be overactive. The two most common causes are kidney failure and vitamin D deficiency. Most recently, there is increasing incidence of secondary hyperparathyroidism related to malabsorptive bariatric procedures (such as Roux-en-Y gastric bypass, duodenal switch). 2°HPT is characterized by low normal or normal calcium levels and elevated PTH levels. Remember, 1°HPT is usually characterized by elevated calcium and elevated PTH levels.
With kidney failure, a variety of changes occur in the body’s physiology that result in low calcium levels, low vitamin D activity and elevated phosphate levels. These alterations stimulate the parathyroid glands to release PTH. This chronic stimulation leads to enlargement of all the glands (hyperplasia). Usually, 2°HPT due to kidney failure is controlled with diet, medication and dialysis. Selected patient with symptoms of severe fatigue, bone pain, difficult to control hyperphosphatemia and PTH levels greater than 1000 are considered for surgery. This decision is made in consultation with the surgeon and nephrologist (kidney specialist).
Low vitamin D is another common cause of 2°HPT. A detailed discussion of vitamin D physiology is beyond the scope of this website. Simply, vitamin D helps to maintain calcium levels, and a deficiency in vitamin D results in low calcium which then stimulates the parathyroid glands to try to raise the calcium back to normal. Again, the calcium levels are typically in the low normal to normal range, and the PTH levels are elevated. Treatment with vitamin D reverses these problems and normalizes the PTH. Surgery does not play a role in treatment of this kind of 2°HPT.
Tertiary Hyper- parathyroidism
3°HPT develops in some cases of long standing 2°HPT when prolonged hypocalcemia causes the development of independently functioning parathyroid glands. Hypercalcemia develops and again the PTH levels are significantly elevated. This situation occurs most commonly in patients with chronic renal failure. The classic situation occurs when a patient with longstanding dialysis-dependent kidney failure (and 2°HPT) receives a kidney transplant. The abnormal physiology that led to 2°HPT is corrected with the transplant but the hyperplastic parathyroid glands continue to over-function due to the development of autonomous parathyroid hormone secretion. Treatment is first with medications, but often requires surgical parathyroidectomy. Typically a standard neck exploration is performed and 3 and1/2 glands are resected. The cure rate approaches 98%. As with other cases of hyperplasia, re-operation may uncommonly be required in recurrent disease.
Adrenal
Functional Adrenal Mass
The adrenal gland can develop both benign and malignant growths, starting in either the adrenal medulla or the adrenal cortex. If the growths secrets a hormone, they are called functioning tumors. If no hormone is secreted they are considered non-functioning tumors. Benign tumors may be functional or non-functional and malignant tumors may be functional or non-functional The adrenal gland can be a frequent sight of metastatic disease from cancer that starts in other areas of the body, and on occasion this may be surgically relevant. We will discuss the most important benign and malignant tumors of the adrenal glands, any functional syndromes that occur with them,and their evaluation and treatment.
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Pheochromocytoma (pheo) is a rare tumor of the central part of the adrenal gland. As described on other pages, this portion of the adrenal gland is called the medulla. It makes epinephrine (adrenalin) and other vasoactive hormones call catecholamines. These hormones control the bodies’ “flight or fight” reflex. Tumors developing here may be malignant, but are usually benign.
“Pheos” over produce these hormones in an unpredictable and unregulated manner. This results in a unique clinical syndrome that includes episodic heart palpitations, headaches and sweating, accompanied by severe high blood pressure. Some patients experience tremors and anxiety. The elevated blood pressure maybe sustained or episodic.
The diagnosis of a pheo involves the patient having the symptoms described above accompanied by significantly elevated blood pressure. Biochemical testing is done to detect elevated levels of catecholamines and their breakdown products. This can be problematic, because these levels may vary a lot in the normal situation as well as when a pheo is present. Usually, we use a 24 hour collection of urine to measure catecholamines breakdown products called metanephrines and VMA. The diagnosis may be tricky because these hormones may not be consistently secreted and many common prescription medications may influence the results. For example, a class of medications called beta-blockers (used to treat high blood pressure) may result in false elevation of these hormones (a false positive result). While calcium channel blockers (also used to treat high blood pressure) may falsely reduce the measured catecholamines (a false negative result). Therefore, evaluation by an experienced physician or endocrinologist is important. In uncertain situations, a carefully controlled blood test for metanephrines may be helpful. Other more complicated blood testing (clonidine suppression test) may be helpful but is not commonly used.
Usually, in suspicious but unproven cases, we obtain an I-131 MIBG whole body scan. This scan reliably shows the tumor position as well as confirms it as a pheochromocytoma. Additionally, it may show secondary tumors or evidence of metastatic disease.
Once the diagnosis is made, tests to locate the tumor are performed. We use MRI, I-131 MIBG scan, and CT scans to locate the tumor, check for evidence of multiple tumors and to find evidence of metastatic disease. 90% of pheos are in the adrenal glands. 10% of cases show tumors on both adrenal glands. 10% of tumors may be found outside the adrenal glands. 10% are malignant. Additionally, other screening test are done to ensure that the patient doesn’t have an inherited syndrome which may cause tumors in other endocrine organs( MEN type 2 ).
After the diagnosis is confirmed, the tumor is located and extent of disease determined, an operative approach is organized. These tumors secrete hormones that may cause dangerously high blood pressure during surgery. The effect of these hormones must be blocked to allow the tumor to be removed as safely as possible. This is discussed on the pre-op care for adrenalectomy page.
Once the patient is adequately prepared the tumor is removed laparoscopically. The details of this are discussed on the laparoscopic adrenalectomy page. After surgery, the patient is observed in the intensive care unit for 24 to 48 hours. Variations in blood pressure may occur and careful monitoring after surgery is important. Once the patient is stable off medication for high blood pressure they are discharged. Patients are seen about 10 days post-op and then follow-up usually with an endocrinologist is arranged. About 10% of pheos are malignant. It can be difficult to be certain about this even with careful examination of the tumor by a pathologist. Generally, the larger the tumor the more likely it is to be malignant. Because of this uncertainty, patients who have had pheos removed should be monitored regularly for recurrent disease. View a video of a laparoscopic adrenalectomy for a pheochromocytoma.
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From a surgical stand point APA is the most important cause of hyperaldosteronism and is commonly called Conn’s snydrome . It accounts for about 30% of hyperaldosteronism. Typically with Conn’s syndrome the degree of hyperaldosteronism is greater than in IHA. Conn’s sydrome is caused by a discrete benign tumor of the adrenal gland (APA). Why an adenoma develops isn’t clearly understood. As with other causes of hyperaldosteronism, it is suspected when a patient has significant high blood pressure and low potassium levels. Further screening with blood test that show an elevated aldosterone level and suppressed renin activity suggests the diagnosis. It is confirmed with a 24 hour collection of urine to measure the amount of aldosterone secreted. These tests can be influenced by a variety of medications and dietary issues and requires an experienced endocrinologist for the best accuracy.
Once the diagnosis is made, then efforts to discern the cause of the over secretion are undertaken. A CT scan or MRI is performed. If the patient is under 40 years old and a clear tumor is seen in one gland with a clearly normal gland on the other side then, generally, it is appropriate to consider surgical removal of the adrenal gland with the adenoma. Most of these tumors are small (2 cm) and laparoscopic removal is the standard of care. We will discuss the surgical techniques later.
Overall the accuracy of these imaging studies is very good (about 80%). However, as patients age, small benign non-functioning tumors can appear in the adrenal gland. Over the age of 40 2-3% of the population may have these incidentally noted tumors. These are called incidentalomas and we will consider them on a subsequent page. Because of this, in patients over 40 years of age, it can not be assumed that a tumor found on imaging is the cause of the hyperaldosteronism. As an example , a patient could have an incidentaloma seen on CTscan in one gland, but the source of the hyperaldosteronism could be a tiny adenoma or primary adrenal hyperplasia (PAH) in the other gland. Therefore in patients over 40 we often recommend adrenal vein sampling (AVS). This is an invasive technique where a catheter is inserted into a large vein in the groin and samples of blood are taken from the vein of each adrenal gland and aldosterone levels are measured. If the aldosterone secretion is significantly elevated from one gland then this is the source of the problem regardless of what the imaging studies show. Some physicians think AVS should always be done in cases of primary hyperaldosteronism. It is a matter of on going debate. AVS is not without risk. It requires significant experience by the radiologist for the best accuracy and it is not available in all communities. There is significant expense involved. There is a small risk of bleeding or damage to the adrenal gland.
Once the diagnosis is made and the source of the problem is clearly located to one gland. Then laparoscopic adrenalectomy is performed. In appropriately selected patients, surgery controls high blood pressure in more than 70% of patients at one year after surgery. Over the next 5 years some patients may have their high blood pressure return, however, it is usually easy to control. Issues with low potassium are generally corrected with resection of the adenoma. We will review the risks and benefits of surgery on a separate page.
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Cushing’s syndrome (CS) is caused by over-production of a type of hormone called glucocorticoids. The most important one is called cortisol. These hormones are made by the adrenal cortex. Glucocorticoids regulate how the body handles protein and glucose. They affect immunology and the inflammatory response. They influence blood pressure and the bodies’ response to stress.
Secretion of these hormones are stimulated by a hormone from the brain called adrenocotical tropic hormone (ACTH). Measurement of this hormone helps us determine the cause of excess cortisol secretion. CS can develop in several ways and is a complicated subject. For our discussion, we will only consider CS that occurs due to excess cortisol production from a tumor of the adrenal gland. This accounts for about 20% of all cases of CS.
Patients with CS suffer from obesity, especially in the central body and face. The arms and legs are relatively thin. Fat deposits may develop on the upper back, adjacent the neck (buffalo hump). High blood pressure occurs in most patients. Muscle weakness and muscle wasting is common. The skin becomes thin and easily damaged. Red and blue stretch marks may appear on the abdomen.
The diagnosis of CS is made when the patient has these clinical features and excess glucocorticoid secretion is proven. Most commonly this is done by measuring the amount of cortisol in the urine over a 24 hour period. Care is taken to ensure that the patient is not taking medications that could elevate cortisol levels falsely. Secretion of these hormones can vary widely and are influenced by many things. Misleading results are not uncommon and other confirmatory tests may be required. These are best done under the supervision of an experienced endocrinologist.
Once the diagnosis of CS has been made the cause (source) of the excess cortisol secretion must be determined. The is done by measuring the amount of ACTH in the blood. If ACTH is elevated then a cause outside the adrenal gland is likely (most commonly a tumor of the gland in the brain that makes ACTH, this is known as Cushing’s disease). If the amount of ACTH in the blood is low them the source of excess cortisol secretion is likely from the adrenal gland. A dexamethasone suppression test may be performed to clarify whether or not CS is do to over-production of ACTH. If the excess cortisol is not due to over production of ACTH then an adrenal source is very likely. About 2/3’s of these cases will be due to benign tumors. About 1/3 of cases will be due to adrenalcortical cancer. Rarely, diffuse over-growth of the cortex of both adrenal glands may be the problem (hyperplasia).
After the diagnosis is made and the source confirmed to the adrenal gland, we perform imaging studies such as a CT scan or MRI. This usually reveals the tumor in one of the adrenal glands and an operative approach can be organized. Our recommended approach is a laparoscopic adrenalectomy. Pre-op and post-op issues are considered on another page. As mentioned above, a malignant tumor may be the cause of CS. Large tumors (> 6 cm) or tumors with evidence of local invasion or metastasis on CT scan are worrisome findings. Measurement of a hormone precursor (DHEA) is frequently elevated when the tumor is malignant. Malignant tumors may be best treated with traditional adrenalectomy.
Non-functional Adrenal Mass
If no hormone is secreted they are considered non-functioning tumors. Benign tumors may be functional or non-functional and malignant tumors may be functional or non-functional. The adrenal gland can be a frequent sight of metastatic disease from cancer that starts in other areas of the body, and on occasion this may be surgically relevant.
Adrenal Carcinoma
The adrenal gland can develop both benign and malignant growths, starting in either the adrenal medulla or the adrenal cortex. If the growths secrets a hormone, they are called functioning tumors. If no hormone is secreted they are considered non-functioning tumors. Benign tumors may be functional or non-functional and malignant tumors may be functional or non-functional The adrenal gland can be a frequent sight of metastatic disease from cancer that starts in other areas of the body, and on occasion this may be surgically relevant. We will discuss the most important benign and malignant tumors of the adrenal glands, any functional syndromes that occur with them,and their evaluation and treatment.
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ACC is rare, the cause is unknown. Smoking may increase the risk of ACC. It occurs more often in women than men. It can happen at any age but the typical patient is about 50 years old. About 50% of these tumors are functional and 50% are non-functional. A variety of hormones can be produced by functioning tumors, most commonly an excess of sex hormones called androgens. The male hormone (testosterone) and the female hormone (estrogen) are common examples of androgens. If too much testosterone is produced in a female with ACC, the patient may notice and increase in body hair,a deepening voice, increase sex drive or thinning hair. In a male these changes may be subtle. If to much estrogen is made in a male with ACC, the patient may notice loss of muscle mass, enlargement of the breast or impotence. Likewise, in a female these symptoms may be subtle. Less commonly, ACC can produce too much cortisol leading to Cushing’s syndrome or too much aldosterone leading to Conn’s syndrome. Blood test are done to detect these changes and then a CAT scan or an MRI can be done to see the tumor. Diagnosis as cancer may be difficult. Size is the best predictor. Tumors larger than 6-8 cm are more likely cancer. Tumors that produce male or female hormones are more likely to be cancer. Surprisingly, a biopsy of the mass does not settle the issue and should not be done because of risk implanting cancer cells along the biopsy tract and because it will not reliably diagnose or exclude cancer.
A functioning ACC usually is detected earlier than non-functioning ACC and may be smaller and therefore more effectively treated. Non-functioning ACC usually presents with local symptoms such as flank discomfort or palpable mass or may be found on an imaging study done for an unrelated reason.
ACC is an aggressive tumor. Surgical resection to a negative margin is the mainstay of treatment. With small tumors ( <5 cm) this may be done with laparoscopic techniques. However, most ACC are relatively locally advanced at the time of diagnosis and larger than 6 cm and open techniques are required to remove it along with a rim of uninvolved tissue. ACC commonly invades other tissues in the area such as the kidney, liver or diaphragm. Portions of these structures may need to be removed to remove all the tumor. Other therapies such as chemotherapy (Mitotane) and radiation therapy may be helpful after the surgery, but removal of the tumor with a margin of normal tissue is the critical aspect of therapy. The aggressiveness of the tumor, it’s relative resistance to chemotherapy and radiation therapy, and the fact that it is commonly diagnosed relatively late, make ACC difficult to cure. Recent studies with antiangiogenesis agents and a class of drugs call tyrosine kinase inhibitors has show promise. Re-growth of the tumor( local recurrence) and metastatic disease commonly to the lungs and liver are problems after initial surgery.
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Because of the vascularity of the adrenal glands, metastasis from cancer starting in other organs is not uncommon. Lung, breast and colon cancer may spread to the adrenal glands. Melanoma and lymphoma are other malignancies that can metastasize to the adrenals. Occasionally, When a isolated metastasis to one adrenal gland is found and on detailed exam and imaging (PET-CT scanning) there is no other evidence of disease, laparoscopic evaluation of that adrenal gland for removal may be appropriate. This resection may provide the patient a survival advantage if complete removal of the metastasis can be done. There is no advantage to partial removal of the metastatic lesion. This approach seems most helpful in isolated lung metastasis or in melanoma metastasis.