Pleomorphic primitive neuroectodermal tumor with glial and neuronal differentiation: clinical, pathological, cultural, and chromosomal analysis of a case

This paper examines a case of pleomorphic primitive neuroectodermal tumor (PNET) with glial and neuronal differentiation in a 7-year-old girl who was clinicopathologically reported with immunohistochemical and chromosomal analysis. Clinically, a highly aggressive behavior leading to early recurrence...

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Bibliographic Details
Published in:Journal of neuro-oncology 2002-08, Vol.59 (1), p.71-79
Main Authors: UEMATSU, Yuji, TAKEHARA, Rie, SHIMIZU, Mina, TANAKA, Yoshiyuki, ITAKURA, Toru, KOMAI, Norihiko
Format: Article
Language:English
Subjects:
Biological and medical sciences
Brain Neoplasms - diagnosis
Brain Neoplasms - genetics
Brain Neoplasms - metabolism
Brain Neoplasms - pathology
Cell Differentiation
Child
Chromosome Mapping
Diploidy
Female
Humans
Immunohistochemistry
Karyotyping
Magnetic Resonance Imaging
Medical sciences
Neoplasm Recurrence, Local - genetics
Neoplasm Recurrence, Local - metabolism
Neoplasm Recurrence, Local - pathology
Neuroectodermal Tumors, Primitive, Peripheral - diagnosis
Neuroectodermal Tumors, Primitive, Peripheral - genetics
Neuroectodermal Tumors, Primitive, Peripheral - metabolism
Neuroectodermal Tumors, Primitive, Peripheral - pathology
Neuroglia - pathology
Neurology
Neurons - pathology
Tumors of the nervous system. Phacomatoses
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Description
Summary:This paper examines a case of pleomorphic primitive neuroectodermal tumor (PNET) with glial and neuronal differentiation in a 7-year-old girl who was clinicopathologically reported with immunohistochemical and chromosomal analysis. Clinically, a highly aggressive behavior leading to early recurrence with cerebrospinal fluid seedings was characteristic despite total removal and chemoradiation therapy. Pathologically, severe pleomorphism was noted and large ganglioid cells were predominant. Immunohistochemically, the expression of low-molecular neurofilament was recognized in the surgical specimens and increased in the recurrence. Coexpression of vimentin and neurofilament/GFAP was recognized in the culture. Chromosomal analysis showed near-diploidy, but different karyotype from that PNETs previously reported. These findings suggested that PNETs with pleomorphism and differentiation into both glial and neuronal lineages may show aggressiveness and require more aggressive therapy.
ISSN:0167-594X
1573-7373
DOI:10.1023/A:1016321030590