Q:

A familial form of medullary thyroid carcinoma (MTC) should be suspected whenever:

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A familial form of medullary thyroid carcinoma (MTC) should be suspected whenever:


  1. The tumor is multifocal.
  2. The tumor is bilateral (foci of tumor are present in both thyroid lobes).
  3. Pathologic examination of the resected thyroid gland reveals the presence of C-cell hyperplasia in areas of the gland adjacent to foci of MTC.
  4. All of the above.

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D. All of the above.

DISCUSSION: Sporadic MTC is unilateral in at least 80% of cases. However, in patients with MTC occurring as a component of the multiple endocrine neoplasia (MEN) type 2A or type 2B syndromes, the tumor is virtually always multifocal and bilateral. Typically, in this setting the MTC appears as multiple whitish-tan tumor nodules in the middle and upper thirds of each thyroid lobe.

A diffuse premalignant proliferation of the C cells of the thyroid is thought to precede the development of MTC in patients with familial MTC. This proliferation, known as C-cell hyperplasia (CCH), consists of parafollicular clusters of increased numbers of C cells. The finding of CCH in areas of the thyroid adjacent to gross foci of MTC is strong evidence for familial MTC.

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