Juvenile fibrosarcoma of the orbit and eyelid. A study of five cases.

Five patients had juvenile fibrosarcoma of the orbit and eyelid. At initial surgical intervention, the patients ranged in age from newborn to 8 years (mean, 3 years 9 months). Three patients were male and two were female. Proptosis and/or painless swelling of the eyelid were the most common signs and symptoms. Four tumors grew within a few weeks or months, and all five were poorly circumscribed. They were composed of immature, spindle-shaped fibroblastic cells in a classic herringbone pattern or in interlacing fascicles. Hypercellularity and mitotic activity were present in all lesions. The differential diagnosis included rhabdomyosarcoma, fibromatosis, and fibrous histiocytoma. Electron microscopy confirmed the fibroblastic nature of the tumor cells in three cases. Follow-up ranged from one to 32 years (median, seven years). Two of five tumors recurred locally but none metastasized. Apparently surgical excision is the treatment of choice and the tumor has a favorable prognosis.