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Peripheral primitive neuroectodermal tumour with ganglioneuroma-like areas arising in the cauda equina.
Virchows Arch. 1997 Nov; 431(5):365-9.VA

Abstract

Peripheral primitive neuroectodermal tumour (pPNET or peripheral neuroepithelioma) is one of the malignant small round cell tumours of peripheral nerves, soft tissues and bones, but rarely originates in the spinal canal. We report an example of pPNET arising in the cauda equina of a 14-year-old Japanese boy. At surgery, a well-demarcated tumour measuring 2 x 4 cm in diameter and involving one of the nerve roots of the cauda equina was located within the intradural space with no evidence of extradural extension. Microscopically the tumour was made up of sheets of closely packed small round cells, associated with ganglioneuroma-like islands. Immunohistochemically, the small round tumour cells were intensely positive for neuron-specific enolase (NSE), an MIC2 gene product (O13) and beta 2-microglobulin, whereas the foci with ganglion cell-like cells reacted positively to NSE, synaptophysin and beta 2-microglobulin but were negative for O13. A chimeric transcript of the EWS/FLI-1 fusion gene detected by a nested reverse transcriptase-polymerase chain reaction using formalin-fixed paraffin-embedded tissue justified the diagnosis of pPNET. Only 6 cases of PNET in the cauda equina have been described in the literature, and this is the first case of a pPNET with ganglio-neuroma-like areas. This finding suggests that the primitive tumour cells of pPNET may respond to unknown inductive effects and express a ganglion cell-like morphology.

Authors+Show Affiliations

Department of Pathology and Oncology, University of Occupational and Environmental Health, Kitakyushu, Japan.No affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

9463579

Citation

Hisaoka, M, et al. "Peripheral Primitive Neuroectodermal Tumour With Ganglioneuroma-like Areas Arising in the Cauda Equina." Virchows Archiv : an International Journal of Pathology, vol. 431, no. 5, 1997, pp. 365-9.
Hisaoka M, Hashimoto H, Murao T. Peripheral primitive neuroectodermal tumour with ganglioneuroma-like areas arising in the cauda equina. Virchows Arch. 1997;431(5):365-9.
Hisaoka, M., Hashimoto, H., & Murao, T. (1997). Peripheral primitive neuroectodermal tumour with ganglioneuroma-like areas arising in the cauda equina. Virchows Archiv : an International Journal of Pathology, 431(5), 365-9.
Hisaoka M, Hashimoto H, Murao T. Peripheral Primitive Neuroectodermal Tumour With Ganglioneuroma-like Areas Arising in the Cauda Equina. Virchows Arch. 1997;431(5):365-9. PubMed PMID: 9463579.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Peripheral primitive neuroectodermal tumour with ganglioneuroma-like areas arising in the cauda equina. AU - Hisaoka,M, AU - Hashimoto,H, AU - Murao,T, PY - 1998/2/17/pubmed PY - 1998/2/17/medline PY - 1998/2/17/entrez SP - 365 EP - 9 JF - Virchows Archiv : an international journal of pathology JO - Virchows Arch. VL - 431 IS - 5 N2 - Peripheral primitive neuroectodermal tumour (pPNET or peripheral neuroepithelioma) is one of the malignant small round cell tumours of peripheral nerves, soft tissues and bones, but rarely originates in the spinal canal. We report an example of pPNET arising in the cauda equina of a 14-year-old Japanese boy. At surgery, a well-demarcated tumour measuring 2 x 4 cm in diameter and involving one of the nerve roots of the cauda equina was located within the intradural space with no evidence of extradural extension. Microscopically the tumour was made up of sheets of closely packed small round cells, associated with ganglioneuroma-like islands. Immunohistochemically, the small round tumour cells were intensely positive for neuron-specific enolase (NSE), an MIC2 gene product (O13) and beta 2-microglobulin, whereas the foci with ganglion cell-like cells reacted positively to NSE, synaptophysin and beta 2-microglobulin but were negative for O13. A chimeric transcript of the EWS/FLI-1 fusion gene detected by a nested reverse transcriptase-polymerase chain reaction using formalin-fixed paraffin-embedded tissue justified the diagnosis of pPNET. Only 6 cases of PNET in the cauda equina have been described in the literature, and this is the first case of a pPNET with ganglio-neuroma-like areas. This finding suggests that the primitive tumour cells of pPNET may respond to unknown inductive effects and express a ganglion cell-like morphology. SN - 0945-6317 UR - https://www.unboundmedicine.com/medline/citation/9463579/Peripheral_primitive_neuroectodermal_tumour_with_ganglioneuroma_like_areas_arising_in_the_cauda_equina_ L2 - https://dx.doi.org/10.1007/s004280050112 DB - PRIME DP - Unbound Medicine ER -