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Treatment may be repeated after 6 to 12 months to ablate any remaining thyroid tissue. TSH-suppressive doses of l -thyroxine levothyroxine are given after treatment, and serum thyroglobulin levels are measured to help detect recurrent or persistent disease.

Neck ultrasonography will detect recurrence in lymph nodes. Thyroid hormone in thyroid-stimulating hormone—suppressive doses is given to minimize chances of regrowth and cause regression of any microscopic remnants of papillary carcinoma. Nikiforov YE, Seethala RR, Tallini G, et al : Nomenclature revision for encapsulated follicular variant of papillary thyroid carcinoma: A paradigm shift to reduce overtreatment of indolent tumors. It is more common among older patients and in regions of iodine deficiency.

It is more malignant than papillary carcinoma , spreading hematogenously with distant metastases. Treatment requires near-total thyroidectomy with postoperative radioiodine ablation of residual thyroid tissue as in treatment for papillary carcinoma. Metastases are more responsive to radioiodine therapy than are those of papillary carcinoma. Thyroid-stimulating hormone-suppressive doses of l -thyroxine are given after treatment. Serum thyroglobulin measurements and neck ultrasongraphy should be done periodically to detect recurrent or persistent disease.

It may be sporadic usually unilateral ; however, it is often familial, caused by a mutation of the ret proto-oncogene. Although calcitonin can lower serum calcium and phosphate levels, serum calcium is normal because the high level of calcitonin ultimately down-regulates its receptors. Characteristic amyloid deposits that stain with Congo red are also present. Metastases spread via the lymphatic system to cervical and mediastinal nodes and sometimes to liver, lungs, and bone.

Patients typically present with an asymptomatic thyroid nodule, although many cases are now diagnosed during routine screening of affected kindreds with MEN 2A or MEN 2B before a palpable tumor develops. Medullary carcinoma may have a dramatic biochemical presentation when associated with ectopic production of other hormones or peptides eg, adrenocorticotropic hormone [ACTH], vasoactive intestinal polypeptide, prostaglandins, kallikreins, serotonin. The best test is measurement of serum calcitonin , which is greatly elevated. All patients with medullary thyroid carcinoma should have genetic testing; relatives of those with mutations should have genetic testing and measurement of basal and stimulated calcitonin levels.

Total thyroidectomy is indicated even if bilateral involvement is not obvious. Lymph nodes are also dissected.


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If hyperparathyroidism is present, removal of hyperplastic or adenomatous parathyroids is required. Pheochromocytoma , if present, is usually bilateral.

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Pheochromocytomas should be identified and removed before thyroidectomy because of the danger of provoking hypertensive crisis during the operation. Long-term survival is common in patients with medullary carcinoma and MEN 2A; more than two thirds of affected patients are alive at 10 years. Medullary carcinoma of the sporadic type and in MEN 2B has a worse prognosis. Relatives with an elevated calcitonin level without a palpable thyroid abnormality should undergo thyroidectomy because there is a greater chance of cure at this stage.

Some experts recommend surgery in relatives who have normal basal and stimulated serum calcitonin levels but who have the ret proto-oncogene mutation. It occurs mostly in older patients and slightly more often in women. The tumor is characterized by rapid, painful enlargement. Rapid enlargement of the thyroid may also suggest thyroid lymphoma, particularly if found in association with Hashimoto thyroiditis. No effective therapy exists, and the disease is generally fatal. In a few patients with smaller tumors, thyroidectomy followed by external beam radiation therapy has been curative.

Chemotherapy is mainly experimental. Thyroid tumors develop in people whose thyroid is exposed to large amounts of environmental radiation, as occurs as a result of atomic bomb blasts, nuclear reactor accidents, or incidental thyroid irradiation due to radiation therapy. Tumors may be detected 10 years after exposure, but risk remains increased for 30 to 40 years.

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