Primary intraspinal primitive neuroectodermal tumor: report of two cases and review of the literature.
BACKGROUND
Primary intraspinal primitive neuroectodermal tumor (PNET) is a very rare tumor entity. The optimal therapeutic approach is not known yet. We report on two women with primary intraspinal PNETs and review the literature. We describe the typical course of the disease, compare our patients to the other 17 cases reported until today, and discuss therapeutic options.


METHODS
Case A: In a 49-year-old woman with an intraspinal PNET at L2, laminectomy and a gross tumor removal was accomplished. Postoperative radiation was performed from T12 to L3 to a dose of 50.4 Gy. Subsequently she was treated with chemotherapy containing vincristine, cisplatinum and lomustine. Case B: A 29-year-old woman presented with intramedullary PNET lesions at T1-3 and T10-11. Due to the multifocal location, she received a primary craniospinal axis irradiation to a dose of 35.2 Gy plus a boost to the tumor region to a total dose of 53.2 Gy.


RESULTS
Both patients developed multilocular intraspinal relapses with meningeosis neoplastica 17 and 6 months from radiation therapy and underwent palliative chemotherapy. Case A died 23 months, case B 17 months after primary diagnosis.


CONCLUSIONS
Despite modern treatment with microsurgery, irradiation and chemotherapy in primary intraspinal PNETs, local relapse or dissemination in most cases lead to death within a few months. An improvement of treatment outcome can only be achieved by intensification through multidisciplinary treatment.
Albrecht
Clemens F
CF
Department of Radiotherapy, University of Goettingen, Goettingen, Germany. calbrech@med.uni-goettingen.de
Weiss
Elisabeth
E
Schulz-Schaeffer
Walter J
WJ
Albrecht
Tanja
T
Fauser
Susanne
S
Wickboldt
Jürgen
J
Hess
Clemens F
CF
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008526:Medulla Oblongata / Q000473:pathology* / Q000601:surgery; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000473:pathology* / Q000628:therapy; D013120:Spinal Cord Neoplasms / Q000473:pathology* / Q000628:therapy; D014057:Tomography, X-Ray Computed