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Spontaneous mesenteric fibromatosis in Gardner's syndrome

โœ Scribed by Ralph C. Richards; Scott W. Rogers; Eldon J. Gardner


Publisher
John Wiley and Sons
Year
1981
Tongue
English
Weight
658 KB
Volume
47
Category
Article
ISSN
0008-543X

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โœฆ Synopsis


Mesenteric fibromatosis has been reported in frequent association with familial polyposis and with

Gardner's syndrome. This vague "benign" process has been characterized as a postsurgical phenomenon with low morbidity. Two cases of spontaneous mesenteric fibromatosis, noted at the time of original laparotomy for colectomy in Gardner's syndrome patients, are reported. No history of abdominal trauma was present, and both have well-documented Gardner's findings. A 32% incidence of desmoid reaction is reported among affected members of the original Gardner's syndrome Kindred 109; five or 55% of these patients had the mesenteric form of the process. The potential fatal course of the mesenteric disease is emphasized.

Cancer 47597-601, 1981.

ESENTERIC FIBROMATOSIS, in frequent associa-M tion with familial polyposis with or without other Gardner's syndrome traits, has been reported in at least 50 cases to date.4 In all but one of these early reports in which records are complete,' the process has arisen in the postsurgical abdomen. The majority of the surgical procedures were done for intestinal problems. Simpson'" and Karakousis4 have suggested that surgical trauma plays an etiologic role in the initiation of the process much as it is thought to do in most other desmoid tumors. Two recent cases of mesenteric fibrosis (desmoid), diagnosed at the time of original abdominal exploration for colectomy in Gardner's syndrome, have raised serious questions about the role of surgical trauma in the etiology of this disease process in Gardner's syndrome. These two cases form the basis for this report.

Case Reports

Case I

A 25-year-old male was in the fifth generation of Kindred 109, the original Gardner's syndrome family. He was born shortly after his 22-year-old father died from massive mesenteric fibromatosis six years following colectomy. The patient had a postauricular fibroma at age 21h years and


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