Management of invasive juvenile nasopharyngeal angiofibromas: the role of a multimodality approach.

Objective: Juvenile nasopharyngeal angiofibromas involving the cranial base and intracranial compartment are challenging tumors. We reviewed our experience of these tumors and analyzed the efficacy of a multimodality management.

Methods: Between 1981 and 2000, 15 extensive juvenile nasopharyngeal angiofibromas (Fisch Grade III or IV) were treated at our institution. The mean age of the patients was 14.5 years, and the mean interval between the first symptom and diagnosis was 12.9 months. Initial management included preoperative embolization of the external carotid artery feeders, followed by tumor removal. A maxillofacial procedure was performed in eight cases, a combination of maxillofacial and neurosurgical approach was performed in four cases, and a neurosurgical cranial base approach was performed in three cases.

Results: Total removal after the initial procedure was obtained in eight patients. Subtotal removal justified additional surgery in one case, gamma knife radiosurgery in two cases, and fractionated irradiation in four cases. True recurrences were observed in four cases at a mean interval of 37 months (range, 24-46 mo) and required tailored multimodality management. No cases of perioperative death were observed. One patient underwent hemiparesis after embolization in the early period of our experience. Permanent facial numbness was reported in four cases, moderate cosmetic problems were reported in three cases, and hyposmia was reported in three cases. Except for one patient who was lost to follow-up at 18 months, 12 patients were free of disease and two patients were free of tumor progression. All patients had normal or near-normal daily life at the last check-up, with a median follow-up period of 108 months (mean, 117 mo; range, 91-252 mo).

Conclusion: Extensive juvenile nasopharyngeal angiofibromas are efficiently managed with a multimodal protocol in which preoperative embolization is followed by optimal surgical removal using various transcranial or transfacial approaches. Adjunctive gamma knife radiosurgery is a valuable option for intracavernous residual tumor. Our protocol offers long-term cure with acceptable morbidity.