[Multiple endocrine neoplasia of the first type (Wermer's syndrome)].
11 patients with Wermer's syndrome (WS) aged 24-67 years were treated. They had a total of 30 tumors: 4(13.3%) hypophyseal, 11(36.7%) pancreatic, 9(30%) parathyroid, 4(13.3%) adrenal and 2(6.7%) duodenal. Each patient had two tumors minimum and 8 tumors maximum. In 8 cases WS presented clinically as hypoglycemia, in 3 cases as Zollinger-Ellison syndrome. The diagnosis was made using ultrasonography, CT, MRT and angiography. Cytogenetic examination was made in 2 cases. All the patients were operated. Six of them were operated two times, one--four times. The following surgery was made: 4 removals of parathyroid adenomas, 4 adrenalectomies, 1 removal of the hypophysis, 4 enucleations of pancreatic insulinomas, 5 distal hemipancreatectomies, 1 distal subtotal resection of the pancreas, 1 gastropancreatoduodenal resection. Clinically symptomatic tumors should be operated first.
Kuzin
N M
NM
Egorov
A V
AV
Kuznetsov
N S
NS
Lotov
A N
AN
Lakreeva
M G
MG
D000328:Adult; D000368:Aged; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000628:therapy*
Mnozhestvennaia éndokrinnaia neoplaziia 1-go tipa (sindrom Vermera).