Adult Atypical Teratoid/Rhabdoid Tumor in the Pineal Region: Case Report and Literature Review.
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. 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. This case highlights the importance of immunohistochemistry in the diagnosis of the disease and the importance of an aggressive treatment.