Mesenteric fibromatosis. A follow-up study.
Eighty-two fibromatoses of the mesentery and other peritoneal sites were studied, with emphasis on features associated with recurrence. Twelve patients had Gardner's syndrome. Seventy-two tumors were completely excised at the time of surgery; 56 patients had no evidence of tumor recurrence (mean, 91 months), and in 16 patients tumors recurred (mean, 117 months of follow-up). Patients with Gardner's syndrome had a much higher risk of recurrence and death related to fibromatosis or surgery for fibromatosis than sporadic cases. There was a weaker association between recurrence and multiplicity. A history of trauma or estrogen exposure, size, mitotic activity, gross circumscription, and histologic features did not correlate with recurrence. Biopsies or partial resections were performed on 10 tumors (8 in patients without Gardner's syndrome and 2 in patients with Gardner's syndrome). The patients without Gardner's syndrome did well: 7 are alive without evidence of disease (mean, 72 months) and 1 died of lung carcinoma at 108 months. The 2 patients with Gardner's syndrome have persistent fibromatosis: 1 patient had recurrence in the abdominal wall at 103 months and 1 is alive at 72 months with intra-abdominal masses. We conclude that mesenteric fibromatosis is much more aggressive in patients with Gardner's syndrome than in patients without Gardner's syndrome, that pathologic features are not good predictors of aggressive behavior, and that complete surgical excision may not always be necessary.
Burke
A P
AP
Department of Gastrointestinal Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000.
Sobin
L H
LH
Shekitka
K M
KM
D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D005350:Fibroma / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D005500:Follow-Up Studies; D005736:Gardner Syndrome / Q000401:mortality / Q000473:pathology / Q000601:surgery; D006801:Humans; D008297:Male; D008643:Mesentery / Q000473:pathology*; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D010534:Peritoneal Neoplasms / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D011379:Prognosis; D012008:Recurrence