[Hemothorax disclosing hemorrhagic necrosis of a pheochromocytoma: circumstance for detection of multiple endocrine neoplasia type IIa. Detection of 5 familial cases].
Whilst investigating 26 members of the same family, we discovered 5 cases of multiple endocrine neoplasia type II a. The present report demonstrates the diagnostic value of basal plasma thyrocalcitonin (TCT) assays, before and after stimulation with pentagastrin, and of plasma carcinoembryonic antigen (CEA) assays. Some of the clinical features encountered were novel--e.g. in one patient the phaeochromocytoma was revealed by a haemothorax with cardiovascular collapse--and others were peculiar; thus, in 4 cases the medullary thyroid carcinoma (MTC) was less than 2 cm in diameter and without lymph node or visceral metastases, even in patients aged 87, 59 and 56. More classically, MTC, always multifocal, was clinically silent, as were the two cases of phaeochromocytoma and hyperparathyroidism. Phaeochromocytomas were difficult to diagnose. Ultrasonic tomography did not prove very helpful and the disease was transmitted as an autosomal dominant trait. Finally, MTC secreted a variety of substances (TCT, CEA, beta-endorphin, somatostatin), and HLA A2-B15 antigens were found in 3 patients.
Dupond
J L
JL
Calmettes
C
C
Viennet
G
G
Mallet
H
H
Devalland
C
C
Mantion
G
G
Etiévent
J
J
D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000473:pathology; D000328:Adult; D000368:Aged; D005260:Female; D005602:France; D006470:Hemorrhage / Q000150:complications; D006491:Hemothorax / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000175:diagnosis* / Q000235:genetics; D009336:Necrosis / Q000150:complications; D010673:Pheochromocytoma / Q000150:complications* / Q000473:pathology
Hémothorax révélateur d'une nécrose hémorragique d'un phéochromocytome: circonstance de découverte d'une néoplasie endocrine multiple de type II a. Dépistage de cinq cas familiaux.