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. 1988 Jun;207(6):655–661. doi: 10.1097/00000658-198806000-00003

Vaterian cancer in siblings.

J C Austin 1, C H Organ Jr 1, G R Williams 1, J V Pitha 1
PMCID: PMC1493544  PMID: 2839122

Abstract

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.

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Selected References

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