Adult Atypical Teratoid/Rhabdoid Tumor in the Pineal Region: Case Report and Literature Review

World Neurosurg. 2020 Feb:134:428-433. doi: 10.1016/j.wneu.2019.11.075. Epub 2019 Nov 20.

Abstract

Background: Atypical teratoid/rhabdoid tumor (AT/RT) is a malignant embryonal central nervous system tumor with a poor prognosis. Its occurrence in the pineal region is extremely rare, with only 6 cases in adults reported so far.

Case description: We report on a puerperal woman with an AT/RT in the pineal region. Total tumor resection was performed. Definitive diagnosis was confirmed by the loss of nuclear INI1/SMARCB1 staining. The patient underwent radiotherapy and chemotherapy with no evidence of recurrence at 31 months after surgery.

Conclusions: This case highlights the importance of immunohistochemistry in the diagnosis of the disease and the importance of an aggressive treatment.

Keywords: Adult; Pineal region; Teratoid rhabdoid tumor.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Brain Neoplasms / pathology*
  • Brain Neoplasms / surgery
  • Female
  • Humans
  • Pineal Gland
  • Rhabdoid Tumor / pathology*
  • Rhabdoid Tumor / surgery
  • Teratoma / pathology*
  • Teratoma / surgery

Supplementary concepts

  • Typical Teratoid Rhabdoid Tumor