Clinico-Histomorphological and Immunohistochemical Profile of Anaplastic Pleomorphic Xanthoastrocytoma: Report of Five Cases and Review of Literature

Abstract

Background: Pleomorphic xanthoastrocytoma is a rare tumour of children and young adults, particularly for those with features of anaplasia. Materials and Methods : This retrospective study comprises five cases of anaplastic pleomorphic xanthoastrocytomas diagnosed over a period of 4 years in a tertiary care institute. A detailed clinicopathological and immunohistochemical profile of the tumours were noted from the hospital database. Results: Five cases of anaplastic pleomorphic xanthoastrocytomas were evaluated for their clinicoradiological, histomorphological as well as immunohistochemical findings, which included 3 females and 2 males, with age range of 11-40 years and a mean age at presentation of 22 years. Histologically a solid cystic biphasic tumour with moderate to high cellularity, spindled pleomorphic astrocytes, hyperchromatic nuclei showing moderate to marked atypia, intranuclear inclusions, ≥5 mitoses per 10 high power fields, with evidence of necrosis and atypical mitoses was noted. One of the cases showed transformation into glioblastoma with evidence of spinal metastasis on follow-up. The tumours expressed both glial as well as neuronal markers with expression of CD34 with increased Ki 67 ranging between 5-20%. Conclusion: It was concluded that PXA, a low-grade glioneuronal tumour, can show odd site presentation, marked pleomorphism, increased mitosis, atypical mitoses and increased Ki 67 when associated with features of anaplasia. An appropriate panel of immunohistochemical markers in conjunction with a detailed evaluation of histomorphological features and clinicoradiological information are useful for its diagnosis.

Keywords: Anaplasia; Astrocytoma; Immunohistochemistry; Ki 67; Mitosis.

Figure 1

MRI showing an intramedullary mass lesion extending from D8-L1 which was hypointense on T1 (1A) and hyperintense on T2 (1B) with a cyst at lower pole

Figure 2

Histopathology showin g moderately cellular tumour (2A, H & E, 400x) with xanthoma cells and eosinophilic granular bodies (2B, H & E, 400x). Binucleated cells (2C, H & E, 100x) and focal necrosis seen. (2D, H & E, 40x).

Figure 3

Positivity with GFAP (3A, IHC, 100x), S100 (3B, IHC, 100x), NSE (3C, IHC, 400x) and synaptophysin (3D, IHC, 400x)

Figure 4

Ki67 labelling 5% (IHC, 400x)

Figure 5

Histopathology showing cellular tumour with xanthoma cells (5A, H & E, 400x) and multinucleated cells (5B and 5C, H & E, 100x). Perivascular lymphocytes seen (5D, H & E, 100x)

Figure 6

Positivity with GFAP (6A, IHC, 40x), S100 (6B, IHC, 400x), NSE (6C, IHC, 100x) and synaptophysin (6D, IHC, 100x)

Figure 7

Ki67 labelling 18% (IHC, 400x)