[Medullary cancer of the thyroid].
BACKGROUND
Medullary thyroid carcinoma is a rare disease which originates from the secretion of calcitonin by thyroid parafollicular cells. Sporadic (75%) and inherited (25%) forms of the disease are encountered. Familial forms (termed multiple endocrine neoplasia type IIa, IIb, or familial medullary thyroid carcinoma) may or may not be associated with other endocrinopathies such as pheochromocytoma and/or hyperparathyroidism.


BACKGROUND
Circulating forms of calcitonin, a marker of the disease, are heterogeneous in blood, thus explaining why assays lead to different results according to the method used.


CONCLUSIONS
Family screening is much easier, as germ line mutations of the proto-oncogene RET have recently been identified in inherited forms of the disease. Treatment includes extensive surgery. This, and prophylactic thyroidectomy in gene carriers, is discussed. Prognosis is much better nowadays, but precise follow-up has to be instituted.
Modigliani
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Service d'endocrinologie, hôpital Avicenne, Bobigny, France.
D000310:Adrenal Gland Neoplasms / Q000235:genetics; D000328:Adult; D002116:Calcitonin / Q000097:blood / Q000235:genetics; D018276:Carcinoma, Medullary / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D005820:Genetic Testing; D006801:Humans; D007223:Infant; D018813:Multiple Endocrine Neoplasia Type 2a / Q000235:genetics; D018814:Multiple Endocrine Neoplasia Type 2b / Q000235:genetics; D009154:Mutation; D010673:Pheochromocytoma / Q000235:genetics; D011247:Pregnancy; D011379:Prognosis; D000090063:Proto-Oncogene Mas; D012086:Reoperation; D013964:Thyroid Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D013965:Thyroidectomy; D013997:Time Factors
C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas; D002116:Calcitonin
Le cancer médullaire de la thyroïde.