Medullary Thyroid Carcinoma

Cancer of the thyroid medulla is an uncommon kind of thyroid cancer. The term medullary indicates the sort of thyroid gland cells that are damaged by malignancy. The majority of medullary thyroid cancer patients (about 80 percent) have no family history of the problem; the remaining 20 percent of patients acquire the condition from a parent (therefore the disease called ‘familial’). Familial medullary thyroid cancer is dominantly inherited, so a patient with the gene mutation for medullary thyroid cancer has a 50% risk of passing it on to their offspring.

The most typical symptom is a solitary nodule on the front of the neck. It is frequently detected during a standard physical examination. Thyroid and neck lumps are often benign, but if you find an unusual bulge in your neck, you should see your doctor immediately.

• Neck pain: Frontal neck pain may be associated with the development of a thyroid tumor. This discomfort can also reach the ears.

• Hoarseness: The nerve that controls your vocal cords passes down the trachea close to the thyroid gland. If the disease has gone to the vocal cord, the quality of your voice may be affected.

Sometimes, thyroid cancer might result in a chronic cough. If you have a cough that is unrelated to a cold or one that persists, you should contact a doctor.

• Difficulty swallowing (dysphagia): If a thyroid tumor becomes large enough, it can impinge on the esophagus and cause difficulty swallowing.

• Breathing difficulties (dyspnea): Similar to difficulty swallowing, if a thyroid tumor is large enough, it can press against the windpipe and impede breathing.

Medullary thyroid carcinoma histology

When to see your doctor?

Anyone who sees a persistent lump or swelling right below Adam’s apple should consult a physician, particularly if the symptoms correlate with any of the following:

  • Inexplicable hoarseness that persists for an extended period of time
  • Problems swallowing
  • A persistently painful throat
  • Trouble in the neck

However, not every neck lump is malignant. Many other less severe disorders, such as lymph node enlargement, can create a lump in the neck.

If a physician feels that the lump may be cancer, he or she would typically refer the patient to a specialist for additional testing.

Diagnosis

Typically, thyroid cancer is diagnosed through a fine needle aspiration (FNA) biopsy of a thyroid nodule or after surgical removal of the nodule. Patients in whom the results of a fine-needle aspiration (FNA) biopsy (or histopathology) are suggestive or diagnostic of MTC should undergo further evaluation by measuring the blood levels of the proteins calcitonin and carcinoembryonic antigen (CEA), which are typically elevated in patients with MTC. In addition to serving as tumor markers during long-term follow-up to detect any remaining disease or recurrence of the cancer, these tests are useful to confirm the diagnosis of MTC, which can help the surgeon plan the correct surgery, and to confirm the absence of any remaining disease or recurrence of the cancer.

Staging

Your health care team utilizes the results of your tests and procedures to evaluate the cancer’s extent and stage. Your cancer’s stage informs your care team of your prognosis and helps them choose the most effective treatment.

The stage of cancer is indicated by a number between 1 and 4. A lower figure indicates that the cancer is more likely to react to treatment and frequently indicates that the malignancy is limited to the thyroid. A higher score indicates a more serious diagnosis, and cancer may have progressed beyond the thyroid to other organs.

Each form of thyroid cancer has a unique progression of phases. For example, medullary and anaplastic thyroid tumors have distinct phases. Different kinds of differentiated thyroid cancer, including papillary, follicular, Hurthle cell, and poorly differentiated, follow the same progression of phases. Depending on your age, the stage of differentiated thyroid cancers may vary.

Associations

MEN2A

  • Medullary thyroid carcinoma
  • Pheochromocytoma (an adrenal gland tumor that can produce excess hormones and can drastically increase blood pressure and heart rate)
  • Hyperparathyroidism primary (a parathyroid tumor that causes elevated blood calcium)
  • Hirschsprung’s disease and cutaneous lichen amyloidosis may be present.

MEN2B

  • Medullary thyroid carcinoma
  • Pheochromocytoma
  • Intestinal neuromas (benign nerve lumps in the intestines)
  • Mucosal ganglioneuromas (benign nerve lumps predominantly occurring around the lips, tongues, and eyelids)

Medullary thyroid cancer in the family

  • Medullary thyroid cancer alone.

Treatment

  • The cornerstone of MTC treatment is the total excision of local and regional disease
  • Adjuvant radiation therapy: Use of external beam radiation when substantial soft tissue invasion or extracapsular lymph node metastasis is observed
  • Chemotherapy and inhibitors of tyrosine kinase
  • Radiation therapy (I-131 MIBG, Y-90 Dotatoc)

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