Primary Intraspinal Primitive Neuroectodermal Tumor: Report of Two Cases and Review of the Literature
- 1 January 2003
- journal article
- case report
- Published by Springer Science and Business Media LLC in Journal of Neuro-Oncology
- Vol. 61 (2), 113-120
- https://doi.org/10.1023/a:1022118317876
Abstract
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. Patients and method. 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 50.4Gy. 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 35.2Gy plus a boost to the tumor region to a total dose of 53.2Gy. 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. Conclusion. 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.Keywords
This publication has 28 references indexed in Scilit:
- Primary primitive neuroectodermal tumor of the cauda equinaHuman Pathology, 2000
- Intraspinal Primitive Neuroectodermal Tumour: Report of Two Cases and Review of the LiteratureActa Neurochirurgica, 1999
- Hyperfractionated craniospinal radiation therapy for primitive neuroectodermal tumors: results of a phase II studyInternational Journal of Radiation Oncology*Biology*Physics, 1999
- Primary Intramedullary Primitive Neuroectodermal Tumor of the Spinal Cord: Case Report and Review of the LiteratureNeurosurgery, 1997
- Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: a Childrens Cancer Group randomized trial.Journal of Clinical Oncology, 1995
- Spinal cord radiation “tolerance”: Doctrine versus dataInternational Journal of Radiation Oncology*Biology*Physics, 1990
- The treatment of medulloblastomaJournal of Neurosurgery, 1990
- Primary Primitive Neuroectodermal Tumor of the Spinal Cord Associated with Neural Tube DefectPediatric Neurosurgery, 1989
- Intraspinal primitive neuroectodermal tumor arising from the sacral spinal nerve rootJournal of Computed Tomography, 1987
- Primitive neuroectodermal tumors of the central nervous system in childrenJournal of Neurosurgery, 1978