Aggressive fibromatosis (fibrosarcoma) of the facial nerve.
Aggressive fibromatosis of the facial nerve is a very rare tumor. Three cases have been previously reported. The tumor is locally recurrent and often has a fatal outcome. This report is of a ten-year-old boy whose tumor originally developed in the parotid area with subsequent spread to the base of the skull, the neck and the cerebellopontine angle. Treatment was by wide surgical excision, radiation therapy and chemotherapy. Despite treatment, the patient died. The clinical features of this case will be described. Only wide surgical excision early in the course of the disease may offer a chance for cure.
Pulec
J L
JL
Pulec Ear Clinic, Los Angeles, California 90017.
D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D001932:Brain Neoplasms / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000556:secondary; D002528:Cerebellar Neoplasms / Q000473:pathology / Q000556:secondary; D002530:Cerebellopontine Angle / Q000473:pathology; D002648:Child; D005153:Facial Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D005154:Facial Nerve / Q000473:pathology* / Q000601:surgery / Q000648:ultrastructure; D005354:Fibrosarcoma / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008334:Mandible / Q000473:pathology / Q000601:surgery; D008339:Mandibular Neoplasms / Q000556:secondary / Q000601:surgery; D009361:Neoplasm Invasiveness; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local; D010306:Parotid Gland / Q000473:pathology / Q000601:surgery; D010307:Parotid Neoplasms / Q000473:pathology / Q000556:secondary / Q000601:surgery; D011859:Radiography; D011878:Radiotherapy; D012469:Salivary Glands / Q000648:ultrastructure