Papillary tumor of the pineal region-report of three cases with literature review

Abstract

Papillary tumor of the pineal region (PTPR) is a rare tumor of the pineal region. Not much is known about the pathogenesis, prognosis, and treatment protocol of this uncommon entity. We present three cases of PTPR with follow-up from 8 months to 98 months. All patients presented with headache and visual disturbance. One patient also had amenorrhea. Radiology revealed an heterogeneously enhancing mass arising from the pineal region with associated hydrocephalus. Histopathologically, all cases showed the papillary architecture, strong pan cytokeratin and cytokeratin 18 positivity, and faint positivity for synaptophysin and neuron-specific enolase. All cases received postoperative radiotherapy. One case showed tumor recurrence after 7 years. Other two cases did not show any recurrence till the last follow-up.