Aggressive papillary tumor of the temporal bone: delayed extensive recurrence following radiation therapy

Endolymphatic sac tumors have been characterized as aggressive papillary tumors of the temporal lobe, exhibiting extensive bony destruction and intracranial extension. This report describes the delayed recurrence of an aggressive endolymphatic sac tumor following full course radiotherapy many years previously. The discussion reviews the current literature on these aggressive lesions with emphasis on clinical presentation, diagnosis, radiographic and histologic appearance and management. This case highlights the need for total surgical excision in the management of these lesions. Radiotherapy as primary management may slow tumor growth but does not appear to be an appropriate therapeutic modality for cure.