Primary pleomorphic xanthoastrocytoma of the spinal cord: A case report and review of literature

Abstract

Primary pleomorphic xanthoastrocytoma (PXA) of the spinal cord is a rare slow growing tumor. To our knowledge, only five such cases have been reported in the literature till date. We report the clinical, radiological, and histopathological features of a spinal PXA in a 23-year-old female previously operated 5 years back for a spinal tumor, presented with weakness in lower limbs, sphincter incontinence and low back pain. Magnetic resonance imaging scan with contrast reveals an intramedullary lesion in the spinal cord from D8-D10 level. The patient was operated with reexploration of the previous incision, and gross total excision was achieved. Histopathology confirmed the diagnosis of PXA. Clinical and radiological follow-up is required to detect early recurrence. Adjunct radiotherapy or and chemotherapy should be considered only when there is postoperative residual or recurrence, however there are no definite guidelines in view of the rarity of this condition.

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

“…The latter has been described as those which show necrosis, cellular anaplasia which is severe, proliferating microvasculature, brisk mitosis (≥5 mitosis/10 high power fields) and increased Ki-67 labelling index 3 , 5 . As shown by the present study and those by Tonn et al, Sharma et al, and Ng et al, these patients typically present at a young age, though exceptional cases of elderly patients do occur 4 , 6 , 14 . Contrary to the typical clinical presentation of PXAs with seizures and cortical lesions, the cases in this series of anaplastic PXAs highlight a gamut of varied presentation, ranging from space occupying lesions presenting with headache and weakness to paraplegia and incontinence with an intramedullary mass; the latter being exceedingly rare 15 .…”

“…This holds true particularly for those with the features of anaplasia 1 . It is known to be a tumour of the children, though exceptions are known 2 , 4 , 6 .…”

Intradural extramedullary pleomorphic xanthoastrocytoma: A case report

“…[1] se neoplastic spindle cells may be arranged in fascicles, often accompanied by hypercellular reticulin staining. [3,13,17] Focal collections of perivascular lymphocytes are also noted. Most are classified as the WHO Grade II; however, 15-20% demonstrate anaplastic features and are classified as the WHO Grade III.…”

“…[4,13,16] BRAF mutations are found in approximately 75% of Grade II PXAs, with a lower frequency in anaplastic PXAs. [9,11,17,18] 9 Prior cases reported of PXA in the spine Only nine prior cases of primary spinal PXA have been reported; of these, only one was in an intradural, extramedullary location, the remainder being intramedullary lesions. [3,4,[6][7][8][9]13,17,18] Classically, PXAs present as cortically based tumors with cystic and solid components, usually isointense on T1-weighted and hyperintense on T2-weighted images, with marked postcontrast enhancement.…”

“…[3,7] Primary spinal PXAs are extremely rare, with only nine prior cases reported in the literature. [3,4,[6][7][8][9]13,17,18] Notably, the majority of these are intramedullary, with only one prior reported case of a primary spinal intradural, extramedullary PXA. [3]…”