Pleomorphic xanthoastrocytoma

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Classification according to ICD-10
C71.9 Malignant neoplasm of the brain, unspecified
ICD-10 online (WHO version 2019)

The pleomorphic xanthoastrocytoma (abbreviated PXA, ICD-O code: 9424/3) is a rare glial brain tumor . It is classified as grade II according to the WHO classification of tumors of the central nervous system . Children, adolescents and young adults are predominantly affected. In the vast majority of cases, there is no recognizable connection to a hereditary disease. The occurrence of pleomorphic xanthoastrocytomas in patients with neurofibromatosis type 1 has been described in isolated cases .

Clinical manifestations

Due to their superficial location in the area of ​​the cerebral cortex , often in the area of ​​the temporal lobe, clinical manifestations of pleomorphic xanthoastrocytomas are predominantly the occurrence of new seizure events . In the imaging, pleomorphic xanthoastrocytomas appear as contrast medium-absorbing cortical masses, some cystic masses.

pathology

Histological section (HE staining) of a pleomorphic xanthoastrocytoma

Histologically , this tumor is characterized by the extraordinary pleomorphism , ie the diversity of the glial tumor cells. Mitotic figures do occur, but necrosis (in contrast to glioblastoma ) are usually not observed. The tumor cells can contain lipid deposits (xanthomatous transformation) and are surrounded by reticulin fibers. The immunohistochemical staining for GFAP confirms the glial character of the tumor. Although the diagnostic differentiation from other due to the characteristic histological image brain tumors usually is not difficult, the pronounced Pleomorphie can of the tumor, particularly in the intraoperative frozen section analysis of glioblastoma to misdiagnosis misled.

Pathogenesis

Little is known about the mechanisms involved in the pathogenesis of pleomorphic xanthoastrocytomas. However, these probably differ from the processes involved in the development of other glial tumors. Loss of genetic material in the area of chromosome 9 appears to be frequent. BRAF V600E mutations can be detected in up to 65% of tumors.

therapy

The tumor is usually removed surgically .

forecast

After surgical removal, the prognosis for pleomorphic xanthoastrocytomas is mostly favorable, but malignant progression can occur. The prognostic value of signs of anaplasia (increased mitotic activity, necrosis) in pleomorphic xanthoastrocytomas ("pleomorphic xanthoastrocytoma with anaplastic features") has not been conclusively clarified.

literature

  • Kepes JJ, Louis DN, Giannini C., Paulus W. "Pleomorphic xanthoastrocytoma." In: Kleihues P., Cavenee WK , eds. "World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of the Nervous System". Lyon, France: IARC Press; 2000

Individual evidence

  1. ↑ Alphabetical index for the ICD-10-WHO version 2019, volume 3. German Institute for Medical Documentation and Information (DIMDI), Cologne, 2019, p. 981
  2. ↑ Kepes JJ et al .: Pleomorphic xanthoastrocytoma: a distinctive meningocerebral glioma of young subjects with relatively favorable prognosis. A study of 12 cases. In: Cancer , 1979; 44 (5): 1839-52 PMID 498051 (first description)
  3. ↑ Saikali S. et al .: Multicentric pleomorphic xanthoastrocytoma in a patient with neurofibromatosis type 1. In: J Neurosurg . , 2005; 102 (2): 376-81. PMID 15739569
  4. ^ Giannini C. et al .: "Pleomorphic xanthoastrocytoma: what do we really know about it?" Cancer. 1999; 85 (9): 2033-45. PMID 10223246
  5. ↑ Lipper MH et al .: Pleomorphic xanthoastrocytoma, a distinctive astroglial tumor: neuroradiologic and pathologic features. AJNR Am J Neuroradiol. 1993; 14 (6): 1397-404. PMID 8279337 (Neuroradiology)
  6. ↑ Kepes JJ et al .: Pleomorphic xanthoastrocytoma: a distinctive meningocerebral glioma of young subjects with relatively favorable prognosis. A study of 12 cases. Cancer. 1979; 44 (5): 1839-52 PMID 498051 (first description)
  7. ↑ C. Giannini, D. Hebrink, BW Scheithauer, AP Dei Tos, CD James: Analysis of p53 mutation and expression in pleomorphic xanthoastrocytoma. In: Neurogenetics 3, 2001, pp. 159-162, PMID 11523567 .
  8. ↑ RG Weber, A. Hoischen, M. Ehrler, P. Zipper, K. Kaulich, B. Blaschke, AJ Becker, S. Weber-Mangal, A. Jauch, B. Radlwimmer, J. Schramm, OD Wiestler, P. Lichter, G. Reifenberger: Frequent loss of chromosome 9, homozygous CDKN2A / p14 (ARF) / CDKN2B deletion and low TSC1 mRNA expression in pleomorphic xanthoastrocytomas. In: Oncogene 26, 2007, pp. 1088-1097, PMID 16909113 .
  9. ↑ Genevieve Schindler, David Capper, Jochen Meyer, Wibke Janzarik, Heymut Omran, Christel Herold-Mende, Kirsten Schmieder, Pieter Wesseling, Christian Mawrin, Martin Hasselblatt, David N. Louis, Andrey Korshunov, Stefan Pfister, Christian Hartmann, Werner Paulus, Guido Reifenberger, Andreas Deimling: Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma. In: Acta Neuropathologica. , S., doi: 10.1007 / s00401-011-0802-6 .
  10. ^ Giannini C. et al .: Pleomorphic xanthoastrocytoma: what do we really know about it? In: Cancer . 1999; 85 (9): 2033-45. PMID 10223246
  11. ↑ Tonn JC Pleomorphic xanthoastrocytoma: report of six cases with special consideration of diagnostic and therapeutic pitfalls. In: Surgical Neurology . 1997; 47 (2): 162-9. PMID 9040821

Web links

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