Atypical teratoid/rhabdoid tumors in adult patients: case report and review of the literature

Atypical teratoid/rhabdoid tumors (AT/RT) of the central nervous system are rare and extremely aggressive malignancies of early childhood. We report a case of AT/RT in an adult patient. A 30-year-old woman presented with headache, vomiting and ataxia during the second trimester of pregnancy. Magnetic resonance imaging revealed a poste rior fossa mass. A gross total resection was performed. Pathological examin ation revealed an AT/RT. Despite the dismal prognosis the patient decided n ot to undergo an abortion. For this reason postoperative accelerated hyperf ractionated radiotherapy was limited to the tumor region. Six months later the woman delivered a healthy baby. One week postpartum, a central nervous system recurrence localized apart from the primary lesion was treated with radiosurgery. Two months later a diffuse progression was noted. Despite a 6 week course of oral temozolomide, the tumor progressed and the patient die d 11 months after diagnosis. Although survival was short, surgery and involved field radiotherapy yielde d a progression-free interval of 9 months. This allowed the patient to carr y pregnancy to term. Radiosurgery resulted in a complete remission of the f irst recurrence. Oral chemotherapy was not effective in controlling diffuse tumor spread.