Abstract

Pilomyxoid astrocytoma (PMA) is one of the rare primary CNS tumors of the pediatric age group which is a histological variant of pilocytic astrocytoma (PA) with poor prognosis. We present a case of 18 months old child presented with sudden onset of unconsciousness for a few hours. On computed tomography (CT) and magnetic resonance imaging (MRI) there was a large infiltrating mass with central haemorrhage seen in the suprasellar region with epicenter at hypothalamus-optic chiasma. There was one satellite nodule seen in the left anterior temporal lobe in a perisylvian location with leptomeningeal enhancement in left Sylvian fissure suggesting cerebrospinal fluid (CSF) dissemination. Based on these distinctive features, the presumed diagnosis was given as PMA also confirmed on histopathological examination after surgery. A palliative shunting to relieve hydrocephalous followed by debulking surgery was performed which is one of novel kind of treatment plans in such rare cases. In this way, few characteristic radiological features along with acute clinical presentation and combination treatment plan in toddler make our case of PMA unique which has not been reported in the literature yet.

Keywords: Pilomyxoid astrocytoma; Haemorrhage; Hypothalamusoptic chiasma; CSF dissemination