Juvenile fibromatosis of the periorbital region and eyelid. A clinicopathologic study of six cases.

Six cases of juvenile fibromatosis of the periorbital region and eyelid were studied. The median age of the patients was 8 years (range, 1 to 11 years). A definite propensity for involvement of the infraorbital region and lower eyelid was observed. THREE TUMORS EXHIBITED A PROMINENT LOBULAR PATTERN AND WERE ADHERENT TO THE PERIOSTEUM. The main entities in the differential diagnosis included leiomyoma, neurofibroma, and well-differentiated fibrosarcoma. Two of six tumors recurred locally but did not metastasize. Electron microscopic studies disclosed that the spindle-shaped tumor cells exhibited features of fibroblasts. It appears that conservative treatment (ie, local excision) is adequate.