title: [Problems posed by the detection of familial medullary cancer of the thyroid in children].
abstract: The authors give the approach managing for medullary thyroid carcinoma in families afflicted by the multiple endocrine tumor syndromes, as defined by "G. E. T. C." (Groupe d'Etude des Tumeurs à Calcitonine), French Group based in Paris, 1983. Diagnosis of MEN II a usually follows investigations in a family of an adult patient found, at thyroid surgery, to have a MTC. Age related probability of development of hereditary MCT is stated. Investigations of the family must be most careful if the index patient is found to have medullary carcinoma on the both sides of the thyroid gland, and/or pheochromocytoma. Sequential monitoring (by means of blood calcitonin measurements following IV infusion of pentagastrin) of family members at risk between 5-35, allows diagnosis of C-cell hyperplasia and treatment of tumor in his earliest stages--MEN II b is usually recognizable in infancy or early childhood by clinical markers (mucosal neuromata, Marfanlike habitus and abnormalities of myenteric plexus). Report of patients in whom MTC was manifest as early as 18 months of age and metastasized at 2 years, require subjects at risk should be screened by CT immunoassay as soon as feasible and repeated annually until 35 years, and every 5 years after 35. The diagnosis of subjects at risk within a family is made easy by recognition of aforesaid clinical features and some more precocious abnormalities = disturbed intradermal histamine reaction and thickened corneal nerve fibers.
mesh_terms: D000293:Adolescent; D000328:Adult; D002116:Calcitonin / Q000097:blood; D002277:Carcinoma / Q000175:diagnosis / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D003315:Cornea / Q000294:innervation; D006632:Histamine; D006801:Humans; D007428:Intradermal Tests; D009377:Multiple Endocrine Neoplasia / Q000175:diagnosis; D010418:Pentagastrin; D011230:Precancerous Conditions / Q000175:diagnosis; D012306:Risk; D013964:Thyroid Neoplasms / Q000175:diagnosis / Q000235:genetics*; D013997:Time Factors
chemical_list: D006632:Histamine; D002116:Calcitonin; D010418:Pentagastrin
vernacular_title: Problèmes posés par le dépistage des cancers médullaires de la thyroide familiaux chez l'enfant.