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Surgery

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Thyroid & Adrenal

Question
42 out of 99
 

A patient with hyperparathyroidism with pheochromocytoma develops a thyroid swelling. This is most likely due to which of the following



A Follicular adenoma
B Papillary carcinoma

C Medullary carcinoma
D Follicular carcinoma

Ans. C Medullary carcinoma (REF. SABISTON SURGERY 18TH EDITION PG 1268 CHP 40)

Table 40-1 -- Clinical Features of Sporadic MTC, MEN 2A, MEN 2B, and FMTC

CLINICAL SETTING

FEATURES OF MTC

INHERITANCE PATTERN

ASSOCIATED ABNORMALITIES

GENETIC DEFECT

Sporadic MTC

Unifocal

None

None

Somatic RET mutations in >20% of tumors

MEN 2A

Multifocal, bilateral

Autosomal dominant

Pheochromocytomas, hyperparathyroidism

Germline missense mutations in extracellular cysteine codons of RET

MEN 2B

Multifocal, bilateral

Autosomal dominant

Pheochromocytomas, mucosal neuromas, megacolon, skeletal abnormalities

Germline missense mutation in tyrosine kinase domain of RET

FMTC

Multifocal, bilateral

Autosomal dominant

None

Germline missense mutations in extracellular or intracellular cysteine codons of RET

a. Twenty-five percent of all MTC cases are hereditary and occur in the setting of MEN 2A, MEN 2B, or FMTC.

b. In MEN 2A, patients develop multifocal, bilateral MTC, associated with C-cell hyperplasia.

c. About 42% of affected patients develop pheochromocytomas, which may also be multifocal and bilateral and are associated with adrenal medullary hyperplasia.

d. Hyperparathyroidism develops in 10% to 35% of patients and is due to hyperplasia, which may be asymmetrical, with one or more glands becoming enlarged.

e. Cutaneous lichen amyloidosis has been described in some patients with MEN 2A. In this entity, macular amyloidosis presents as brownish plaques of multiple tiny papules, usually in the interscapular area.

f. Lastly, Hirschsprung's disease is infrequently associated with MEN 2A.

g. This disease is characterized by absence of autonomic ganglion cells within the distal colonic parasympathetic plexus, resulting in obstruction and megacolon.

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