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Primary Appendiceal Mucinous Adenocarcinoma in Two First-degree Relatives: Case Report and Review by Adrianne R Racek; Kari G Rabe; Myra J Wick; Apostolos Psychogios; Noralane M Lindor is a Medicine article available to read on EtoBox.

What is Primary Appendiceal Mucinous Adenocarcinoma in Two First-degree Relatives: Case Report and Review about?

Carcinomas of the appendix are exceedingly rare tumors and have an annual age-adjusted incidence of around 0.4 cases per 100,000. Appendiceal adenocarcinoma accounts for < 0.5% of all gastrointestinal neoplasms and, of these, mucinous adenocarcinomas account for the majority. Published accounts of familial instances of primary appendiceal tumors are strikingly rare. We report two siblings who both developed primary mucinous adenocarcinomas. A genetics evaluation was conducted to determine if there was a recognizable underlying single gene disorder; no DNA mismatch repair defect was evident, and no other diagnosis was apparent. A review of appendiceal cancers seen at Mayo Clinic from l997 to the present was conducted to search for additional familial cases. Among 316 cases of primary appendiceal cancer of any histologic type, this sib pair was the only family reporting a second affected family member. The occurrence of appendiceal cancer in siblings may represent a random occurrence. An exceedingly rare predisposition syndrome cannot be ruled out.

Who reads Primary Appendiceal Mucinous Adenocarcinoma in Two First-degree Relatives: Case Report and Review?

Author

Adrianne R Racek; Kari G Rabe; Myra J Wick; Apostolos Psychogios; Noralane M Lindor

Publisher

BioMed Central; Springer (Biomed Central Ltd.); Poznań: "Termedia"; Springer Science and Business Media LLC (ISSN 1897-4287)

Published

2011

Language

EN

Field

Medicine (Health Sciences)