DESMOID FIBROMATOSIS OF THE SINONASAL TRACT AND NASOPHARYNX - A CLINICOPATHOLOGICAL STUDY OF 25 CASES

BACKGROUND. Desmoid fibromatoses are a group of nonmetastasizing, well differentiated, unencapsulated fibrous tissue proliferations that hav e a tendency for local invasion and recurrence. Biologically, they fal l in an intermediate category between benign fibrous lesions and fibro sarcoma. Because of the rarity of this lesion in the upper respiratory tract and inadequate characterization of its biologic potential in th e literature, this study was undertaken. METHODS. The files of the Arm ed Forces Institute of Pathology were reviewed for cases of fibromatos is involving the sinonasal and nasopharyngeal areas that occurred betw een 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, rangin g in age from 8 months to 62 years (mean, 29 years and 11 months). A s ingle site was involved in 18 patients and multiple contiguous adjacen t sites in 7. The maxillary sinus was the site most frequently involve d (22 patients), followed by the nasal cavity (5 patients), the ethmoi d sinus (4 patients), orbit (4 patients), sphenoid and frontal sinuses (2 patients each), and the nasopharynx (1 patient). Twenty-four patie nts were followed for periods ranging from 1 year to 20 years and 7 mo nths (median, 6 years and 9 months; mean, 8 years and 2 months). At la st follow-up, 18 patients were alive and well with no evidence of dise ase, 2 patients were alive with unknown disease histories, and 3 patie nts were alive with recurrent or residual disease. One patient died wi thout evidence of disease. Five patients (21%) (4 adults and 1 child) developed recurrences; 3 patients had 1 recurrence at 6, 16, and 34 mo nths, 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 pati ent was lost to follow-up. CONCLUSIONS. Twenty-five cases of desmoid f ibromatosis involving the sinonasal tract and nasopharynx were describ ed. These lesions appear to have lower recurrence rates and morbidity than desmoid fibromatoses arising in many other areas of the body. (C) 1996 American Cancer Society.