Open AccessVaterian Cancer in Siblings
Author(s) -
J C Austin,
Claude H. Organ,
Grant R. Williams,
Josef Pitha
Publication year - 1988
Publication title -
annals of surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.153
H-Index - 309
eISSN - 1528-1140
pISSN - 0003-4932
DOI - 10.1097/00000658-198806000-00003
Subject(s) - medicine , sibling , malignancy , proband , jaundice , pancreaticoduodenectomy , cancer , neoplasm , pathology , genetics , pancreas , mutation , psychology , developmental psychology , biology , gene
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.