P14.89 Papillary tumor of the pineal region: three cases from a single institution

Abstract

\n \n \n Papillary tumors of the pineal region are highly unusual lesions of unknown incidence. These are neuroepithelial tumors with papillary and solid areas. There is no standardized treatment because of the difficulty to make randomized prospective trials due to its rarity. We report three patients with papillary tumor of the pineal region.\n \n \n \n Retrospective review of the clinical records of patients with papillary tumor of the pineal region at a tertiary neurological center in Buenos Aires, Argentina was done between 2/2012-04/2021. Clinical characteristics, imaging, pathology, treatment and follow-up data were obtained.\n \n \n \n Two patients were male and one female. All three patients were adults, two in the fourth decade of life and one in the seventh. Clinical presentation included headache along with either visual symptoms, gait disturbance, or both. Imaging workup included brain and spinal magnetic resonance imaging, finding expanding lesions in the pineal region, hyperintense in FLAIR/T2 sequences, with gadolinium enhancement in T1 with contrast sequence and variable degrees of hydrocephalus. Spinal lesions were not found in these patients. Cerebrospinal fluid cytology was negative in all cases. Tumor biopsy was the initial approach in all cases. Two patients underwent an initial third ventriculostomy procedure but all were submitted to a supracerebellar infratentorial approach for major resection. Posterior treatment included chemoradiotherapy with temozolomide. One patient had a distant recurrence after seven years with and remained with stable disease for two more years, and the other two patients are under control with no recurrence after 2 and 2.5 years respectively.\n \n \n \n Papillary tumors in the pineal regions are aggressively treated as recurrence is very frequent regarding previous reports (> 70%). Total resection is the only predictive factor associated to better outcome, however, more information is needed in order to achieve treatment consensus.\n