Multiple Primary Primitive Neuroectodermal Tumours within the Spinal Epidural Space with Non-concurrent Onset

Abstract

A case of multiple primary primitive neuroectodermal tumours (PNETs), which occurred at different levels of the spinal epidural space successively over a period of 8 months, is reported. A 24-year-old male, presenting with rapidly progressive paralysis, hyperthesia and a posterior epidural mass extending from T8 to T10 revealed by magnetic resonance imaging (MRI), exhibited a good recovery after initial emergency surgery. Lower back pain, chest pain and paralysis were subsequently reported. Spinal MRI in month 7 revealed a mass extending from T12 to L1 and another mass extending from T4 to T5 was detected epidurally in month 8. Additional operations were performed and radiotherapy was given. Pathological findings were consistent with PNETs and symptoms improved with treatment, particularly following each surgical excision.

Keywords

Spinal epidural space Intraspinal tumour Primitive neuroectodermal tumour (PNET)Multiple primary tumours

References

Dehner

: Peripheral and central primitive neuroectodermal tumors: A nosologic concept seeking a consensus. Arch Pathol Lab Med 1986; 110: 997–1005.

Liu

Yang

Garcia

: Intraspinal primitive neuroectodermal tumor arising from the sacral spinal nerve root. J Comput Tomogr 1987; 11: 350–354.

Akai

Iizuka

Kadoya

: Primitive neuroectodermal tumor in the spinal epidural space—case report. Neurol Med Chir (Tokyo) 1998; 38: 508–511.

Dorfmuller

Wurtz

Umschaden

: Intraspinal primitive neuroectodermal tumour: Report of two cases and review of the literature. Acta Neurochir (Wien) 1999; 141: 1169–1175.

Virani

Jain

: Primary intraspinal primitive neuroectodermal tumor (PNET): A rare occurrence. Neurol India 2002; 50: 75–80.

Aydin

Sen

Ozel

: Primary primitive neuroectodermal tumor within the spinal epidural space: Report of a case and review of the literature. Neurol Res 2004; 26: 774–777.

Hart

Earle

: Primitive neuroectodermal tumors of the brain in children. Cancer 1973; 32: 890–897.

Kleihues

Burger

Scheithauer

: The new WHO classification of brain tumours. Brain Pathol 1993; 3: 255–268.

Rorke

Hart

McLendon

: Supratentorial primitive neuroectodermal tumor (PNET). In: Pathology and Genetics of Tumors of the Central Nervous System ( Kleihues

Cavenee

, eds). 2000; pp 141–144.

10.

Louis

Ohgaki

Wiestler

: The 2007 WHO Classification of Tumours of the Central Nervous System. Acta Neuropathol (Berl) 2007; 114: 97–109.

11.

Albrecht

Weiss

Schaeffer

WJ Schulz

: Primary intraspinal primitive neuroectodermal tumor: Report of two cases and review of the literature. J Neurooncol 2003; 61: 113–120.

12.

De Tommasi

Occhiogrosso

: Primary intramedullary primitive neuroectodermal tumor (PNET)–case report and review of the literature. Eur J Neurol 2006; 13: 240–243.

13.

Luna

Martinez

Leal

: Is the ancillary chemotherapy approach of any value in the treatment of infratentorial primitive neuroectodermal tumors with surgery and radiotherapy? Childs Nerv Syst 1998; 14: 109–112; discussion 113.

14.

Nutman

Postovsky

Zaidman

: Primary intraspinal primitive neuroectodermal tumor treated with autologous stem cell transplantation: Case report and review of the literature. Pediatr Hematol Oncol 2007; 24: 53–61.

15.

George

Loudon

Moser

: In vitro cytolysis of primitive neuroectodermal tumors of the posterior fossa (medulloblastoma) by lymphokine-activated killer cells J Neurosurg 1988; 69: 403–409.