Faculty Collaboration Database

Malignant gliomas with primitive neuroectodermal tumor-like components: a clinicopathologic and genetic study of 53 cases. Brain Pathol 2009 Jan;19(1):81-90

Date

05/03/2008

Pubmed ID

18452568

Pubmed Central ID

PMC8094809

DOI

10.1111/j.1750-3639.2008.00167.x

Scopus ID

2-s2.0-57449090950 (requires institutional sign-in at Scopus site)   147 Citations

Abstract

Central nervous system neoplasms with combined features of malignant glioma and primitive neuroectodermal tumor (MG-PNET) are rare, poorly characterized, and pose diagnostic as well as treatment dilemmas. We studied 53 MG-PNETs in patients from 12 to 80 years of age (median = 54 years). The PNET-like component consisted of sharply demarcated hypercellular nodules with evidence of neuronal differentiation. Anaplasia, as seen in medulloblastomas, was noted in 70%. Within the primitive element, N-myc or c-myc gene amplifications were seen in 43%. In contrast, glioma-associated alterations involved both components, 10q loss (50%) being most common. Therapy included radiation (78%), temozolomide (63%) and platinum-based chemotherapy (31%). Cerebrospinal fluid (CSF) dissemination developed in eight patients, with response to PNET-like therapy occurring in at least three. At last follow-up, 27 patients died, their median survival being 9.1 months. We conclude that the primitive component of the MG-PNET: (i) arises within a pre-existing MG, most often a secondary glioblastoma; (ii) may represent a metaplastic process or expansion of a tumor stem/progenitor cell clone; (iii) often shows histologic anaplasia and N-myc (or c-myc) amplification; (iv) has the capacity to seed the CSF; and (v) may respond to platinum-based chemotherapy regimens.

Author List

Perry A, Miller CR, Gujrati M, Scheithauer BW, Zambrano SC, Jost SC, Raghavan R, Qian J, Cochran EJ, Huse JT, Holland EC, Burger PC, Rosenblum MK

Author

Elizabeth J. Cochran MD Adjunct Professor in the Pathology department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Adult
Aged
Aged, 80 and over
Antineoplastic Agents, Alkylating
Brain Neoplasms
Combined Modality Therapy
Dacarbazine
Female
Follow-Up Studies
Genes, myc
Glioma
Humans
In Situ Hybridization, Fluorescence
Medulloblastoma
Neoplasm Metastasis
Neoplasms, Multiple Primary
Neuroectodermal Tumors, Primitive
Prognosis
Proto-Oncogene Proteins
Radiotherapy
Treatment Outcome
Young Adult