Rare Tumors (Jun 2021)

A rare giant mixed germ cell tumor of the pineal region with immature elements: Case report and review of the literature

  • Camille K Milton,
  • Panayiotis E Pelargos,
  • Nathaniel D Stetson,
  • Manuel Maldonado-Vital,
  • Kar-Ming A Fung,
  • Ian F Dunn

DOI
https://doi.org/10.1177/20363613211026501
Journal volume & issue
Vol. 13

Abstract

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The diagnosis and management of mixed intracranial germ cell tumors may be complicated by the diversity present within this tumor category. Mixed germ cell tumors demonstrate variable natural histories which may be altered by the inclusion of even the most minute immature histological components. We report the case of an 18-year-old male who presented with a 3-month history of progressive headache and nausea leading to lethargy. Imaging revealed a giant pineal region mass extending superiorly from the roof of the fourth ventricle into the lateral ventricle, with resultant obstructive hydrocephalus. No spinal lesions were noted. Following gross total resection, the patient experienced marked improvement. Pathologic analysis identified an uncommon tumor composition: mature teratoma (96%), immature teratoma (2%), and germinoma (2%). Guided by the immature component, chemotherapy and radiation were added post-operatively to provide this patient with the greatest chance of long-term survival. Intracranial pathology, including germ cell tumors, should be included in the differential for any young patient presenting with new and progressive headache and nausea. This case emphasizes the benefit of a multimodal approach to mixed germ cell tumors of the pineal region and the importance of careful pathologic review of all submitted material.