Desmoid fibromatosis of the sinonasal tract and nasopharynx. A clinicopathologic study of 25 cases

Abstract

Background: Desmoid fibromatoses are a group of nonmetastasizing, well differentiated, unencapsulated fibrous tissue proliferations that have a tendency for local invasion and recurrence. Biologically, they fall in an intermediate category between benign fibrous lesions and fibrosarcoma. Because of the rarity of this lesion in the upper respiratory tract and inadequate characterization of its biologic potential in the literature, this study was undertaken.

Methods: The files of the Armed Forces Institute of Pathology were reviewed for cases of fibromatosis involving the sinonasal and nasopharyngeal areas that occurred between 1885 and 1985. For each case, histologic materials were reviewed, clinical data tabulated, and follow-up obtained.

Results: Twenty-five cases were identified. Sixteen patients were male and 9 female, ranging in age from 8 months to 62 years (mean, 29 years and 11 months). A single site was involved in 18 patients and multiple contiguous adjacent sites in 7. The maxillary sinus was the site most frequently involved (22 patients), followed by the nasal cavity (5 patients), the ethmoid sinus (4 patients), orbit (4 patients), sphenoid and frontal sinuses (2 patients each), and the nasopharynx (1 patient). Twenty-four patients were followed for periods ranging from 1 year to 20 years and 7 months (median, 6 years and 9 months; mean, 8 years and 2 months). At last follow-up, 18 patients were alive and well with no evidence of disease, 2 patients were alive with unknown disease histories, and 3 patients were alive with recurrent or residual disease. One patient died without evidence of disease. Five patients (21%) (4 adults and 1 child) developed recurrences; 3 patients had 1 recurrence at 6, 16, and 34 months, respectively, 1 patient had 2 recurrences at 3.5 and 5.5 months, and 1 patient was alive with recurrent disease at 6.5 years. One patient was lost to follow-up.

Conclusions: Twenty-five cases of desmoid fibromatosis involving the sinonasal tract and nasopharynx were described. These lesions appear to have lower recurrence rates and morbidity than desmoid fibromatoses arising in many other areas of the body.