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Chirurgia 2000 April;13(2):81-96

Copyright © 2000 EDIZIONI MINERVA MEDICA

language: Italian

Updating on aggressive fibromatosis (known as Desmoid Tumor) with particular reference to surgical treatment. Review of the literature and case reports

Borri A., Bencini L., Bontà M., Pernice L. M.

Aggressive fibromatosis, commonly known as Desmoid Tumor, is a rare neoplasm arising from the musculoaponeurotic structures and rarely from coelomic cavities. These lesions are well documented in Gardner's syndrome. The biological behaviour is not constant: there are lesions without any risk for the patient and others with aggressive growth and many recurrences. They could be considered as stromal cells tumors with a significant tendency for recurrence after surgical resection but lacking of metastatic potential. A review of the Literature concerning the surgical treatment is made and personal observations are presented: three were extraabdominal desmoid tumors, three of the abdominal wall and one was in the mesentery. All the patients underwent wide surgical resection; in one case the whole muscle involved in the lesion (''compartimental'' excision) was resected. During the follow-up, only one extrabdominal recurrence 18 months later was observed. A second wide excision followed by adjuvant brachytherapy using 192Ir was attempted. The necessity of a follow-up with modern radiology, expecially during the first three years after excision is underlined. There is no consensus concerning the therapy, but primary treatment seems to be wide local excison, where feasible. Surgery and adjuvant radiotherapy are indicated in the treatment of patients with recurrence or when the margins are positive following excision. Any personal experience with farmacologic therapies has been made, but recent data seem to be unsatisfying.