What Is The Follow
Periodic follow-up examinations are essential for all patients with MTC because the thyroid cancer can return, sometimes many years after successful initial treatment. These follow-up visits include a careful history and physical examination, with particular attention to the neck area. Neck ultrasound is also a very important tool to visualize the neck and look for nodules, lumps or enlarged lymph nodes that might indicate that the cancer has recurred.
Blood tests are also important in the follow-up of MTC patients. All patients who have had their thyroid glands removed require thyroid hormone replacement with levothyroxine. Thyroid stimulating hormone should be checked periodically, and the dose of levothyroxine adjusted to keep TSH in the normal range. There is no need to keep TSH suppressed in patients with MTC.
Measurement of calcitonin and CEA are a necessary routine part of the follow-up of patients with MTC. Following thyroidectomy, it is hoped that calcitonin levels will be essentially undetectable for life. A detectable or rising calcitonin level should raise suspicion for possible cancer recurrence. Detectable calcitonin levels may require additional tests.
Signs And Symptoms Of Medullary Thyroid Cancer
Many cases of MTC can initially be asymptomatic. This means that the condition may not cause any noticeable symptoms at first, so those with MTC may not know that they have it.
However, if symptoms do occur, they may include:
- a lump in the throat
- a hoarse voice or vocal cord dysfunction
- difficulty breathing or a blocked windpipe
- dysphagia, or difficulty swallowing
An older 2011 review of MTC outlines that symptoms of more advanced MTC can occur from the complications it may cause. For example, MTC leading to high levels of calcitonin may cause diarrhea. Dysfunctional hormone production due to a diseased thyroid can also cause facial flushing.
Seek prompt medical care for any symptoms related to cancer, and seek immediate emergency care for any severe symptoms relating to difficulty breathing.
Systemic Therapy: Cytotoxic Agents And Tyrosine Kinase Inhibitors
Progressive or symptomatic metastatic MTC that cannot be treated with surgery, local management, or radiotherapy should be considered as a candidate for systemic therapy. In general, traditional cytotoxic agents should not be considered as the first-line therapy for patients with persistent or recurrent MTC because of their limited benefit and toxicity . Activating germline mutations of the RET proto-oncogene, a tyrosine kinase receptor, are found in almost all patients with MEN2A and MEN2B. Somatic RET mutations have been documented in approximately 50% to 60% of sporadic MTC cases. Somatic RAS mutations have also been found in MTC patients without somatic RET mutations . In addition, vascular endothelial growth factor receptors are often overexpressed in MTC tumor cells and vascular endothelium . Because angiogenesis plays a crucial role in growth and metastasis of tumors, increased VEGF expression promotes thyroid cancer cell growth, LN metastasis, local invasion, and enhanced distant metastasis . Thus, the most successful agents target both the VEGFRs and tyrosine kinases. Although several TKIs have been evaluated in clinical trials, both vandetanib and cabozantinib were recently approved for treatment of advanced, metastatic or progressive MTC on the basis of phase 3 clinical trials .
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Medullary Thyroid Cancer Surgery
Medullary thyroid cancer surgery is introduced here. The correct operation depends upon the medullary thyroid cancer extent of disease, patient evaluation, and surgeon’s expertise. What is most important, is that all of the medullary thyroid cancer is removed from the neck in the initial surgery!!! That surgery does not necessarily have to be a large surgery but it needs to be a comprehensive surgery which removes all of the thyroid gland and all of the affected and high at risk lymph nodes of the neck. It can, however, also be a relatively small incision about 1.5 inches in length in the lower front of the neck.
Again, what the most critical issue is that all of the medullary thyroid cancer within the neck must be effectively removed at the initial surgery. An expert thyroid cancer surgeon is necessary to accomplish the correct surgery addressing all of the neck disease and maintaining all critical structures to maintain the patient’s appearance, function, and quality of life. The wrong choice in surgeon increases the risk of medullary thyroid cancer persistence, recurrence, complications, and even ultimately survival of medullary thyroid cancer. Make sure you have a true medullary thyroid cancer surgery expert!!!
Medullary thyroid cancer surgery must be considered in several different lights when you think about the neck. Surgery of the thyroid gland itself and surgery for the lymph nodes of the neck. The basic concept is however very simple, remove all of the cancer.
Medullary Thyroid Cancer Symptoms
Medullary carcinoma is characterized by a long latency period. The first manifestation of the disease is often an increase in regional lymph nodes. With further progression of medullary thyroid cancer, accompanied by compression and germination of the nearest organs, hoarseness of voice, difficulty swallowing, shortness of breath, dry superficial cough and difficulty breathing occur. During palpation, lymph nodes are enlarged, immobile or inactive, and may form conglomerates with surrounding tissues.
In 10-60% of patients with medullary thyroid cancer with MEN-2 syndromes, symptoms of pheochromocytoma are detected. The disease is manifested by headaches, sweating and mental instability. With prolonged existence, there is an increase in blood pressure. In 10-25% of patients with medullary thyroid cancer with MEN-2A syndrome, hyperparathyroidism occurs, which is usually manifested by asymptomatic hypercalcemia or urolithiasis. In addition, with MEN-2A, Hirschsprungs disease and papular lesions of the upper back can be detected.
All forms of medullary thyroid cancer can produce lymphogenic and hematogenic metastases. With distant metastasis, the lungs suffer more often . Secondary foci are usually located in the lower parts of the lungs, have a multiple character and are displayed on radiographs in the form of small focal shadows resembling dissemination in tuberculosis. Possible metastasis to the bones, liver, pleura, kidneys and brain.
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Peptide Receptor Radionuclide Therapy
Somatostatin receptor is present on a majority of cases of MTC and can be detected with somatostatin receptor-based PET modalities . While no randomized controlled trials of Peptide Receptor Radionuclide Therapy in MTC have been done to date the use of radiolabeled somatostatin analogs for the treatment of MTC has been reported in a number of smaller studies. Patients treated with 90Y-DOTATOC had a response rate of 29% in a phase II study . In a single-institution retrospective analysis of patients with MTC treated with 177Lu-DOTATATE, a 62% response rate based on RECIST 1.1 criteria was reported . Recent reviews have summarized the published experience of PRRT in the treatment of patients with medullary thyroid cancer. Overall cumulative objective response rates to PRRT in patients with MTC of 5.1 and 10.6% were reported . While these studies do show promise, the lack of controlled studies of PRRT in MTC as well as the potential for significant side-effects including hematologic and nephrotoxicity point to the need for randomized controlled trials in this area.
How We Vetted This Article:
- Esfandiari, N., Hughes, D., Yin, H., Banerjee, M., & Haymart, M. R. . The effect of extent of surgery and number of lymph node metastases on overall survival in patients with medullary thyroid cancer. Journal of Clinical Endocrinology and Metabolism,99, 448-454
- Hong, W. K. & . Hait, W. N. . Cancer Medicine, Vol. 8. Shelton, CT.: PMPH-USA, Limited.
- How is thyroid cancer staged?
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Treatment Of Medullary Thyroid Cancer
The usual treatment approach for MTC is surgery to remove the thyroid. This takes place during a procedure called thyroidectomy. Surgeons may also remove the lymph nodes in the same region.
Thyroidectomy may also be an option for those at high risk of MTC in order to prevent it. For example, this may be the case for people with RET gene changes.
Sometimes, further care is necessary to continue to rid MTC or further cancer or examine how well treatment is working. Further treatment may include:
How Is Medullary Thyroid Cancer Treated
MTC is usually treated by removing the thyroid. This surgery is called a thyroidectomy. In certain people with a high risk for MTC, such as people carrying certain gene changes, a thyroidectomy may be performed to prevent cancer.
Besides surgery, sometimes other treatments are also required, including radiation therapy or chemotherapy. Also, targeted therapies are available that act on changes in DNA found in some cases of MTC.
After treatment, your doctor will monitor levels of a tumor marker called CEA and the hormones produced by C cells to keep track of how well the treatment is working or if cancer has come back. CEA is a type of tumor marker found in the blood of those with MTC.
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What Is The Initial Management Of A New Diagnosis Of Medullary Thyroid Cancer
If the FNA confirms the diagnosis of medullary thyroid cancer, the following tests are needed:
- Metanephrines and normetanephrines
The cells that make up medullary thyroid cancer usually produce calcitonin and/or CEA. Thus, calcitonin and CEA are tumor markers for medullary thyroid cancer. We use these tests to estimate the extent of disease, and guide how aggressive surgical management should be.
Up to 25% cases of medullary thyroid cancer are familial, meaning it can be passed down by family members. Thus, its important to receive genetic counseling and consider genetic testing. In some cases, medullary thyroid cancer is passed down as part of a genetic syndrome. The other 75% cases are sporadic, which are not inherited or passed down. The genetic test is a blood test that looks for a mutation in the RET gene.
Prognostic Factors For Well
Age appears to be thesingle most important prognostic factor. The prognosis for differentiatedcarcinoma withoutextracapsular extension or vascular invasion is better for patients younger than 40 years.
Patients considered at low risk according to age, metastases, extent, and size risk criteria include women younger than 50 years and men youngerthan 40 years without evidence of distant metastases. The low-risk group also includes older patients with primary papillary tumors smaller than 5cm without evidence of gross extrathyroidalinvasion, and older patients with follicular cancer without major capsular invasion or bloodvessel invasion. Using these criteria, a retrospective study of 1,019patients showed that the 20-year survival rate was 98% for low-risk patients and50% for high-risk patients.
Aretrospective surgical series of 931 previously untreated patients withdifferentiated thyroid cancer found that age older than 45 years, follicular histology, primary tumor larger than 4cm , extrathyroidal extension , and distant metastases were adverse prognostic factors. Favorable prognostic factors included female gender, multifocality, andregional lymph node involvement. Other studies, however, have shown that regional lymph node involvement had noeffect or had an adverse effect on survival.
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Treatment Option Overview For Thyroid Cancer
Standard treatment options for thyroid cancer are described in Table 1.
|Type of Thyroid Cancer||Disease Status||Standard Treatment Options|
|T = primary tumor N = regional lymph node M = distant metastases EBRT = external-beam radiation therapy RAI = radioactive iodine.|
|Papillary and follicular|
|Recurrent papillary and follicular thyroid cancer|
External Beam Radiation Therapy For Medullary Thyroid Cancer
Medullary thyroid cancer treatment with external beam radiation therapy is not commonly required or indicated. The planning and implementation of radiation therapy is beyond the goals for this website. However certain principles must be emphasized. Radiation therapy is not a substitute for incomplete surgery. What is meant by that is all the medullary thyroid cancer in the neck must be completely and effectively removed. Whenever feasible, medullary thyroid cancer patients should be reduced down to microscopic remaining neck disease, at most, also sparing voice box and swallowing tube function. Radiation therapy should not be given as a substitute for incomplete surgery. As a general rule, choosing to treat a medullary thyroid cancer with external beam radiation is a commitment that the surgeon believes that no meaningful re-operation will be feasible in the future and therefore radiation therapy is required to help control the medullary thyroid cancer remaining in the neck. In these circumstances, external beam radiation therapy is quite effective. Medullary thyroid cancer radiation therapy is also associated with significant short term and long term complications and effects that should not be taken lightly.
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Does Medullary Thyroid Cancer Run In Families
Twenty-five percent of MTC cases run in families. MTC may be passed down when families carry a change in the RET gene that causes a condition called multiple endocrine neoplasia type 2, or MEN2. There are two types of MEN2: MEN2A and MEN2B.
MEN2A: If you have MEN2A, you have a high chance of getting MTC. You are also at risk for getting pheochromocytoma, a cancer of the adrenal glands. MEN2A is rare, affecting 1 in 40,000 people. MEN2A may also be called Sipple syndrome or PTC syndrome.
MEN2B: MEN2B can sometimes be passed from parent to child but most of the time, it isnt. If you have MEN2B, you have a 100% chance of getting MTC at a very young age. You also have a 50% chance of getting pheochromocytoma at some point in your life. MEN2B is also called WagenmannFroboese syndrome or MEN3.
Management In Persistent Or Recurrent Mtc
Management options for recurrent or residual MTC include close observation for indolent disease, surgical resection of locoregional disease, EBRT, and local therapies, such as radiofrequency ablation, cryoablation, and embolization, or systemic therapies, such as conventional chemotherapy, kinase inhibitors, and immune checkpoint inhibitors for non-resectable diseases. Therefore, treatment approaches in these patients depend on various clinical factors, including presence of the symptoms, possibility of significant structural disease progression, disease localization, disease volume or burden, and location of the metastatic lesion .
Patients with biochemical incomplete responses without structural disease are followed-up with conservative surveillance . Therapeutic interventions based on increased biochemical markers are not recommended. In cases of locoregional disease without distant metastasis, surgical resection is the primary treatment option with or without postoperative EBRT or intensity-modulated radiation therapy . However, active surveillance can be an optimal management modality in many patients with asymptomatic small LN metastases because repeat operations are commonly not curative and can be associated with high surgical complications, such as adjacent nerve injury and permanent hypoparathyroidism .
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Targeted Therapy And/or Chemotherapy
For medullary thyroid cancer patients who have spread of their cancer to several places outside of the neck area or areas of disease within the neck that thyroid cancer surgery experts believe surgery could be more troublesome than the cancer itself, new therapies have been developed and approved by the FDA . Although these FDA approved medullary thyroid cancer targeted medications have been shown to be effective, none of these treatments are curative. Skilled physicians in prescribing these medications are required because of the necessity for close monitoring of symptoms, toxicities, and monitoring of the patient’s medullary thyroid cancer.
The first FDA approved targeted therapy for medullary thyroid cancer was Vandetanib. The second approved targeted therapy for medullary thyroid cancer was Cabozantinib. Neither of these two drugs has been shown, yet, to prolong the lives of medullary thyroid cancer patients however both drugs are approved and have established clinical activity. Complications are common with these targeted therapies including diarrhea, nausea, high blood pressure, weight loss and more. Close clinical observation is required when medullary thyroid cancer patients are managed with systemic targeted therapy. Experts in medical endocrine oncology are those thyroid cancer team specialists that manage these medullary thyroid cancer patients. Again, these medications cannot be taken indefinitely and do not cure the medullary thyroid cancer patients.
Treatments For Medullary Thyroid Cancer
You may be offered the following treatments for medullary thyroid cancer . Your healthcare team will suggest treatments based on your needs and the stage of the cancer. They will work with you to develop a treatment plan.
Localized or regional medullary thyroid cancer has not spread outside of the thyroid. It is most often treated with surgery to remove the thyroid. Hormone therapy is used to replace the hormones that are normally made by the thyroid.
Metastatic medullary thyroid cancer has spread to distant parts of the body. It may be treated with targeted therapy or chemotherapy.
Recurrent medullary thyroid cancer means that the cancer has come back after it has been treated. Treatment will depend on where the cancer recurs. It is treated with surgery, external radiation therapy, targeted therapy or chemotherapy.
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What Kind Of Long
In addition to the usual cancer monitoring, patients should receive an annual chest x-ray as well as blood checks for calcitonin and carcinoembryonic antigen levels. Serum calcitonin is very useful in the follow up of medullary thyroid cancer because no other cells of the body make this hormone. A high serum calcitonin level that had previously been low following total thyroidectomy is indicative of recurrence.
Carcinoembryonic antigen is a protein that is usually found in the blood at a very low level but might rise in certain cancers, such as medullary thyroid cancer. There is no direct relationship between serum calcitonin levels and extent of medullary thyroid cancer. However, trending serum calcitonin and CEA levels can be a useful tool for doctors to consider in determining the pace of change of a patients medullary cancer.
Under the best circumstances, medullary thyroid cancer surgery will remove all of the thyroid and all lymph nodes in the neck that harbor metastatic spread. In this case, post-operative calcitonin levels will go to zero. This is often not the case, and calcitonin levels frequently remain elevated, but less than pre-operatively.
These levels should still be checked every six months, and when they begin to rise, a more diligent examination is in order to find the source.For a general overview of the different types of thyroid cancer, read our article, and you can also visit our to learn about thyroid cancer diagnosis and treatments.