Vaterian cancer in siblings.
The simultaneous occurrence of Vaterian carcinoma in two siblings suggests a genetic influence in their pathogenesis. Their classic clinical presentation of obstructive jaundice and weight loss required pancreaticoduodenectomy for this neoplasm. Pedigree analysis revealed a third sibling who died from an unresectable periampullary malignancy. Neither of the probands exhibited, as late as the seventh decade, evidence compatible with a diagnosis of familial polyposis coli or Gardner's syndrome. Flow cytometry studies revealed an aneuploid distribution in one tumor and tetraploid in the other. The rarity of this neoplasm, in the absence of contributing epidemiologic factors, suggests that this is a pleotrophic manifestation of a cancer-prone genotype.
Austin
J C
JC
Department of Surgery, University of Oklahoma Health Sciences Center, Oklahoma City 73126.
Organ
C H
CH
Williams
G R
GR
Pitha
J V
JV
D000230:Adenocarcinoma / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology; D011125:Adenomatous Polyposis Coli / Q000235:genetics; D000328:Adult; D000368:Aged; D014670:Ampulla of Vater; D003138:Common Bile Duct Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology; D004379:Duodenal Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology; D005260:Female; D005736:Gardner Syndrome / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary; D010375:Pedigree; D011859:Radiography