DR. SUBHODEEP THAKUR

Dr. BIJNYA BIRAJITA PANDA, Dr.Kshirabdhi Tanaya Sahoo, DR. SUBHRA SARITA BEHERA

Abstract

Pineoblastoma is a rare type of malignant supratentorial Primitive Neuroectodermal Tumor (sPNET) mainly affecting children, but can also occur in adults. Parinaud syndrome or Dorsal midbrain syndrome is a supranuclear brainstem syndrome involving the vertical gaze centre.

A 13 year old girl presented with headache,vomiting and altered sensorium. Pupil was hyporeflexive with superior gaze palsy and progressed to develop ‘setting sun sign’ with eyes fixed in down gaze. There was convergence- retraction nystagmus and loss of light near dissociation. MRI brain revealed hypointense lesion involving the Quadrigeminal plate cistern and Obstructive Hydrocephalus.

Diagnosis of Pinealoblastoma with Parinaud Syndrome was made and therapeutic VP shunt was placed to ease the hydrocephalus.Patient started on 4 cycles of chemotherapy (Cisplatin+Etoposide) followed by Radiotherapy.This case is unique due to the rarity of the disease and the nature of its ocular presentation.