A Young Boy with Recurrent Episodes of Vomiting: The Importance of Early Diagnosis

Introduction: Papillary tumor of the pineal region (PTPR) is a newly described entity, which has been recently included in the World Health Organization classification of central nervous system tumors.

Case-report: A 5-year-old boy presented twice ,in 72 hours, to the emergency department with vomiting with no other symptoms . Was discharged with anti-emetic treatment. He returns on the 4th day of illness with headache, vomiting and diplopia. Radiologic studies revealed triventricular hydrocephalus secondary to a space-occupying lesion in the pineal region. Endoscopic third ventriculostomy was performed to treat hydrocephalus and the lesion was biopsied. Complete resection of the tumor was subsequently performed. The diagnosis of PTPR was established on immunohistopathological examination. Postoperative radiotherapy resulted in complete regression of the tumor with no evidence of tumor recurrence at 12 months.

Conclusion: Papillary tumors of the pineal region constitute a rare entity, with a difficult diagnosis, an uncertain prognosis, and a high risk of local recurrence. They must be treated by a combination of surgery and radiotherapy. This case shows the importance of a detailed clinical history and physical examination to a child presenting with symptoms of intracranial hypertension.