Anaplastic pleomorphic xanthoastrocytoma in a 12-year-old with early leptomeningeal dissemination: a rare pediatric case report from Central India

Shyamji Rawat; Navya S. Boppadapu; Anjanette Nabiyal; Abhishek Badgoliya; Karthick Raju V. A.; Dhirendra K. Panwar

doi:10.18203/2349-3259.ijct20262510

Authors

Shyamji Rawat Department of Radiation Oncology, Netaji Subash Chandra Bose Medical College, Jabalpur, Madhya Pradesh, India https://orcid.org/0000-0002-3757-5401
Navya S. Boppadapu Department of Radiation Oncology, Netaji Subash Chandra Bose Medical College, Jabalpur, Madhya Pradesh, India
Anjanette Nabiyal Department of Radiation Oncology, Netaji Subash Chandra Bose Medical College, Jabalpur, Madhya Pradesh, India https://orcid.org/0009-0002-4232-1235
Abhishek Badgoliya Department of Radiation Oncology, Netaji Subash Chandra Bose Medical College, Jabalpur, Madhya Pradesh, India https://orcid.org/0009-0003-6170-1310
Karthick Raju V. A. Department of Radiation Oncology, Netaji Subash Chandra Bose Medical College, Jabalpur, Madhya Pradesh, India https://orcid.org/0009-0002-2137-8822
Dhirendra K. Panwar Department of Radiation Oncology, Netaji Subash Chandra Bose Medical College, Jabalpur, Madhya Pradesh, India https://orcid.org/0009-0001-6999-7465

DOI:

https://doi.org/10.18203/2349-3259.ijct20262510

Keywords:

Anaplastic pleomorphic xanthoastrocytoma, Pediatric glioma, Leptomeningeal dissemination, WHO CNS grade 3, Drop metastasis

Abstract

Pleomorphic xanthoastrocytoma (PXA) is a rare glioma subtype, making up less than 1% of astrocytoma, mainly affecting young adults. It typically appears in solid, cystic, or mixed forms in the supratentorial region, especially the temporal lobe, with a peak onset around age 20. Reported cases describe cerebrospinal fluid dissemination, often occurring months after diagnosis. We report a case of 12-year-old boy presenting with headache and vomiting for over a month, with no significant medical or family history. Magnetic resonance imaging brain showed lesion in the right fronto-parietal region. He underwent surgery to excise the lesion, which was diagnosed as a PXA, classified as World Health Organization (WHO) grade 3. Immunohistochemical analysis indicated positivity for ATRX, H3K27me3, and p53 protein suggesting high-grade glial tumor. After surgery, he had recurrence of headache within a month. Follow-up magnetic resonance imaging revealed residual tumor, leading to second surgery for tumor re-exploration. He received adjuvant radiation therapy and chemotherapy with Temozolomide. Despite treatment, he re-developed symptoms, and further imaging showed metastasis in the left medial temporal lobe and lumbar spine. Unfortunately, he did not respond to chemotherapy and passed away within a month. This case highlights the complex behavior of PXA, which complicates disease management. It denotes surgical excision alone is inadequate and should be complemented by spine staging scans and radiation therapy. Poor prognosis indicators include necrosis, high mitotic activity and incomplete resection underscoring the need for comprehensive treatment approach.

References

Kepes JJ, Rubinstein LJ, Eng LF. Pleomorphic xanthoastrocytoma: a distinctive meningocerebral glioma of young subjects with relatively favorable prognosis. A study of 12 cases. Cancer. 1979;44(5):1839-52.

Perry A, Brat DJ. Practical surgical neuropathology: a diagnostic approach. 1st Edition. Churchill Livingstone. 2010.

Mahajan S, Dandapath I, Garg A, Sharma MC, Suri V, Sarkar C. The evolution of pleomorphic xanthoastrocytoma: from genesis to molecular alterations and mimics. Lab Invest. 2022;102(7):670-81.

Giannini C, Scheithauer BW, Burger PC, Brat DJ, Wollan PC, Lach B, et al. Pleomorphic xanthoastrocytoma: what do we really know about it? Cancer. 1999;85(9):2033-45.

Hirose T, Ishizawa K, Sugiyama K, Kageji T, Ueki K, Kannuki S. Pleomorphic xanthoastrocytoma: a comparative pathological study between conventional and anaplastic types. Histopathology. 2008;52(2):183-93.

Choudry UK, Khan SA, Qureshi A, Bari E. Primary anaplastic pleomorphic xanthoastrocytoma in adults. Case report and review of literature. Int J Surg Case Rep. 2016;27:183-8.

Whittle IR, Gordon A, Misra BK, Shaw JF, Steers AJ. Pleomorphic xanthoastrocytoma. Report of four cases. J Neurosurg. 1989;70(3):463-8.

Koga H, Zhang S, Kumanishi T, Washiyama K, Ichikawa T, Tanaka R, et al. Analysis of p53 gene mutations in low- and high-grade astrocytomas by polymerase chain reaction-assisted single-strand conformation polymorphism and immunohistochemistry. Acta Neuropathol. 1994;87(3):225-32.

Kepes JJ, Rubinstein LJ, Ansbacher L, Schreiber DJ. Histopathological features of recurrent pleomorphic xanthoastrocytomas: further corroboration of the glial nature of this neoplasm. A study of 3 cases. Acta Neuropathol. 1989;78(6):585-93.

Lubansu A, Rorive S, David P, Sariban E, Seligmann R, Brotchi J, et al. Cerebral anaplastic pleomorphic xanthoastrocytoma with meningeal dissemination at first presentation. Childs Nerv Syst. 2004;20(2):119-22.

Fouladi M, Jenkins J, Burger P, Langston J, Merchant T, Heideman R, et al. Pleomorphic xanthoastrocytoma: favorable outcome after complete surgical resection. Neuro Oncol. 2001;3(3):184-92.

Patibandla MR, Nayak M, Purohit AK, Thotakura AK, Uppin M, Challa S. Pleomorphic xanthoastrocytoma with anaplastic features: A rare case report and review of literature with reference to current management. Asian J Neurosurg. 2016;11(3):319.

Kim B, Chung CK, Myung JK, Park SH. Pleomorphic xanthoastrocytoma associated with long-standing Taylor-type IIB-focal cortical dysplasia in an adult. Pathol Res Pract. 2009;205(2):113-7.

Hegi ME, Diserens AC, Gorlia T, Hamou MF, de Tribolet N, Weller M, et al. MGMT gene silencing and benefit from temozolomide in glioblastoma. N Engl J Med. 2005;352(10):997-1003.

Marucci G, Morandi L. Assessment of MGMT promoter methylation status in pleomorphic xanthoastrocytoma. J Neurooncol. 2011;105(2):397-400.

Martínez R, Carmona FJ, Vizoso M, Rohde V, Kirsch M, Schackert G, et al. DNA methylation alterations in grade II- and anaplastic pleomorphic xanthoastrocytoma. BMC Cancer. 2014;14:213.

Dias-Santagata D, Lam Q, Vernovsky K, Vena N, Lennerz JK, Borger DR, et al. BRAF V600E Mutations Are Common in Pleomorphic Xanthoastrocytoma: Diagnostic and Therapeutic Implications. PLoS One. 2011;6(3):e17948.

Jones MC, Drut R, Raglia G. Pleomorphic xanthoastrocytoma: a report of two cases. Pediatr Pathol. 1983;1(4):459-67.

Goldring S, Rich KM, Picker S. Experience with gliomas in patients presenting with a chronic seizure disorder. Clin Neurosurg. 1985;33:15-42.

Grant JW, Gallagher PJ. Pleomorphic xanthoastrocytoma. Immunohistochemical methods for differentiation from fibrous histiocytomas with similar morphology. Am J Surg Pathol. 1986;10(5):336-41.

Iwaki T, Fukui M, Kondo A, Matsushima T, Takeshita I. Pleomorphic xanthoastrocytoma with spinal dissemination: case report. J Comput Assist Tomogr. 1987;11(2):330-3.

Gaskill SJ, Marlin AE, Saldivar V. Glioblastoma multiforme masquerading as a pleomorphic xanthoastrocytoma. Childs Nerv Syst. 1988;4(4):237-40.

Bayindir C, Balak N, Karasu A, Ak H, Kaynar MY, Can M, et al. Anaplastic pleomorphic xanthoastrocytoma. Childs Nerv Syst. 1997;13(1):50-6.

Tonn JC, Paulus W, Warmuth-Metz M, Schachenmayr W, Sörensen N, Roosen K. Pleomorphic xanthoastrocytoma: report of six cases with special consideration of diagnostic and therapeutic pitfalls. Surg Neurol. 1997;47(2):162-9.

Chakraborty A, Bhaisora KS, Behari S, Jaiswal AK, Sardhara J, Mehrotra A, et al. Pleomorphic xanthoastrocytoma with anaplastic features: case reports. Clin Neuropathol. 1999;18(5):247‑52.

Tekkök IH, Suess O, Sav A. Pleomorphic xanthoastrocytoma with anaplastic features: case report and review of the literature. Pediatr Neurosurg. 2004;40(3):138-45.

Chang HT, Shahine B, Chen H. Pleomorphic xanthoastrocytoma of the cerebellum: long-term survival after multimodality therapy. J Neurooncol. 2006;80(1):69-74.

Okazaki T, Kageji T, Matsuzaki K, Hirose T, Watanabe H, Nagahiro S. Pleomorphic xanthoastrocytoma with diffuse spinal nodules: pediatric case report. Brain Tumor Pathol. 2009;26(2):75-80.

Tsutsumi S, Yasumoto Y, Ito M. Pathological laughter caused by frontal glioblastoma: case report. Neurol Med Chir (Tokyo). 2008;48(7):307-10.