Cervical intraspinal extradural primitive neuroectodermal tumour in an adult

Abstract

Intraspinal extradural primitive neuroectodermal tumours (PNET) represent a rare entity that almost always carry a poor prognosis. A recent literature research revealed only four cases reported in adults. The tumours have mainly thoracic and lumbar location. We report the only case with cervical location and with the longest survival. Urgent laminectomy was performed resulting in a rapid pain relief for the patient. The patient was followed up with craniospinal irradiation and several cycles of chemotherapy. The patient died due to dissemination of the disease 31 months after the diagnosis. Intraspinal PNET tumours have a rapidly progressive course, and there is no current consensus on the optimal therapeutic approach.

Keywords

• extradural
• primitive neuroectodermal
• tumour
• radiotherapy
• chemotherapy