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.
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