Malignant gliomas with primitive neuroectodermal tumor-like components: A clinicopathologic and genetic study of 53 cases Journal Article
| Authors: | Perry, A.; Miller, C. R.; Gujrati, M.; Scheithauer, B. W.; Zambrano, S. C.; Jost, S. C.; Raghavan, R.; Qian, J.; Cochran, E. J.; Huse, J. T.; Holland, E. C.; Burger, P. C.; Rosenblum, M. K. |
| Article Title: | Malignant gliomas with primitive neuroectodermal tumor-like components: A clinicopathologic and genetic study of 53 cases |
| 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. © 2008 The Authors. |
| Keywords: | survival; human tissue; treatment outcome; treatment response; major clinical study; genetics; proto-oncogene proteins; combined modality therapy; temozolomide; follow up; glioma; brain neoplasms; follow-up studies; in situ hybridization, fluorescence; dacarbazine; gene amplification; radiotherapy; carmustine; stem cell; neuroblastoma; glioblastoma; genes, myc; medulloblastoma; cancer stem cell; antineoplastic agents, alkylating; neoplasm metastasis; platinum; oligodendroglioma; cell clone; metastases; drug substitution; oncogene c myc; cerebrospinal fluid analysis; nerve cell differentiation; oncogene c myb; gliosarcoma; neoplasms, multiple primary; neuroectoderm tumor; anaplastic carcinoma; neuropathology; neuroectodermal tumors, primitive; myc; primitive neuroectodermal tumor; small cell; chromosome 10q |
| Journal Title: | Brain Pathology |
| Volume: | 19 |
| Issue: | 1 |
| ISSN: | 1015-6305 |
| Publisher: | Blackwell Publishing |
| Date Published: | 2009-01-01 |
| Start Page: | 81 |
| End Page: | 90 |
| Language: | English |
| DOI: | 10.1111/j.1750-3639.2008.00167.x |
| PUBMED: | 18452568 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | --- - "Cited By (since 1996): 11" - "Export Date: 30 November 2010" - "CODEN: BRPAE" - "Source: Scopus" |
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