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Familial multicentric fibromatosis—Desmoids. A report of three cases in a Jordanian family
Author(s) -
Zayid Ismail,
Dihmis Carlos
Publication year - 1969
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(196910)24:4<786::aid-cncr2820240420>3.0.co;2-0
Subject(s) - medicine , etiology , fibromatosis , gardner syndrome , gardner's syndrome , familial adenomatous polyposis , daughter , family history , incidence (geometry) , abdominal wall , pediatrics , dermatology , pathology , surgery , cancer , colorectal cancer , physics , evolutionary biology , optics , biology
Desmoid tumors, in and outside the abdominal wall, are reported in 3 adult members of one family—a mother, son, and daughter. No evidence of familial polyposis or any other feature of Gardner's syndrome were noted. Review of the literature revealed no similar familial incidence of desmoids except in Gardner's syndrome. The authors believe that the findings in this exceptional family support the theory that a heritable factor, in the form of a dominant autosomal gene, plays a role in the etiology of desmoids. It is, incidentally, noted that 2 of the tumors encountered in this family are outside the abdominal wall—one being intraabdominal—and are recorded as examples of these rather rare tumors. The literature on the etiology of desmoids is reviewed and discussed.