Pleomorphic xanthoastrocytoma with rapid evolution into anaplastic xanthoastrocytoma. Case report and literature review
DOI:
https://doi.org/10.59156/revista.v39i01.654Keywords:
Anaplastic, Astrocytoma, Pleomorphic, XanthoastrocytomaAbstract
Background: pleomorphic xanthoastrocytoma (PXA) is a rare tumor of the CNS, its anaplastic variant (PXA-A) is like PXA in terms of clinical presentation but is more aggressive and has a much poorer prognosis. Five to 20% of patients experience recurrences with malignant transformation.
Objectives: to describe a case of a patient with PXA with rapid progression to PXA-A.
Case description: a 46-year-old patient with histologically confirmed PXA, with rapid progression and malignant transformation to PXA-A, with unfavorable evolution.
Intervention: surgery with subtotal macroscopic resection, then reintervention at the time of recurrence and progression.
Conclusion: PXA is a rare tumor that can vary its histological grade toward anaplasia (PXA-A) and even toward a variety of glioblastoma. Given the limited evidence available, there is no clear protocol for specific oncological treatment.
Downloads
References
Shaikh N, Brahmbhatt N, Kruser T, Kam K, Appin C, Wadhwani N, Chandler J, Kumthekar P, Lukas R. Pleomorphic xanthoastrocytoma: a brief review. CNS Oncology, 2019; 8(3): CNS39.
Noone AM, Howlader N, Krapcho M, Miller D, Brest A, Yu M, Ruhl J, Tatalovich Z, Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2015, National Cancer Institute.
Kepes JJ, Kepes M, Slowik F. Fibrous xanthomas and xanthosarcomas of the meninges and the brain. Acta Neuropathol, 1973; 23(3): 187-99.
Kepes J, Rubinstein L, Ansbacher L, Schreiber D. Histopathological features of recurrent pleomorphic xanthoastrocytomas: further corroboration of the glial nature of this neoplasm. Acta Neuropathol, 1989; 78(6): 585-93.
Fukushima H, Nakano Y, Ishii N, Nozuchi N, Okuno T, Yamasaki K, et al. Histological and genetic analysis of anaplastic pleomorphic xanthoastrocytoma suspected of malignant progression over a 12-year clinical course, Pathol Int, 2019; 69(10): 608-13.
Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary, Acta Neuropathol, 2016; 131(6): 803-20.
Tekkok IH, Sav A. Anaplastic pleomorphic xanthoastrocytomas. Review of the literature with reference to malignancy potential, Pediatr Neurosurg, 2004; 40(4): 171-81.
Vu TM, Liubinas SV, Gonzales M, Drummond KJ. Malignant potential of pleomorphic xanthoastrocytoma. J Clin Neurosci, 2012; 19(1): 12-20.
Okazaki T, Kageji T, Matsuzaki K, Horiguchi H, Hirose T, Watanabe H, Ohnishi T, Nagahiro S. Primary anaplastic pleomorphic xanthoastrocytoma with widespread neuroaxis dissemination at diagnosis-a pediatric case report and review of the literature. J Neurooncol, 2009; 94(3): 431-7.
Gelpi E, Popovic M, Preusser M, Budka H, Hainfellner J. Pleomorphic xanthoastrocytoma with anaplastic features presenting without GFAP immunoreactivity: implications for differential diagnosis. Neuropathology, 2005; 25(3): 241-6.
Gallo P, Cecchi PC, Locatelli F, Rizzo P, Ghimenton C, Gerosa M, Pinna G. Pleomorphic xanthoastrocytoma: long-term results of surgical treatment and analysis of prognostic factors. Br J Neurosurg, 2013; 27(6): 759-64.
Gil-Gouveia R, Cristino N, Farias JP, Trindade A, Ruivo NS, Pimentel J. Pleomorphic xanthoastrocytoma of the cerebellum: illustrated review. Acta Neurochir, 2004; 146(11): 1241-4.
Watanabe N, Ishikawa E, Kohzuki H, Sakamoto N, Zaboronok A, Matsuda M, Shibuya M, Matsumura A. Malignant transformation of pleomorphic xanthoastrocytoma and differential diagnosis: case report. BMC Neurol, 2020; 20(1): 21.
Giannini C, Scheithauer BW, Burger PC, Brat DJ, Wollan PC, Lach B, O’Neill BP. Pleomorphic xanthoastrocytoma: what do we really know about it? Cancer, 1999; 85(9): 2033-45.
Rippe DJ, Boyko OB, Radi M, Worth R, Fuller GN. MRI of temporal lobe pleomorphic xanthoastrocytoma. J Comput Assist Tomogr, 1992; 16(6): 856-9.
Ida CM, Rodriguez FJ, Burger PC, Caron AA, Jenkins SM, Spears GM, Aranguren DL, Lachance DH, Giannini C. Pleomorphic xanthoastrocytoma: natural history and long-term follow-up. Brain Pathol, 2015; 25(5): 575-86.
Schmidt Y, Kleinschmidt-DeMasters BK, Aisner DL, Lillehei KO, Damek D. Anaplastic PXA in adults: case series with clinicopathologic and molecular features. J Neurooncol, 2013; 111(1): 59-69.
Phillips JJ, Gong H, Chen K, Joseph NM, van Ziffle J, Bastian BC, Grenert JP, Kline CN, Mueller S, Banerjee A, Nicolaides T, Gupta N, Berger MS, Lee HS, Pekmezci M, Tihan T, Bollen AW, Perry A, Shieh JTC, Solomon DA. The genetic landscape of anaplastic pleomorphic xanthoastrocytoma. Brain Pathol, 2019; 29(1): 85-96.
Tanaka S, Nakada M, Nobusawa S, Suzuki SO, Sabit H, Miyashita K, Hayashi Y. Epithelioid glioblastoma arising from pleomorphic xanthoastrocytoma with the BRAF V600E mutation. Brain Tumor Pathol, 2014; 31(3): 172-6.
