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Abstract

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 posterior fossa mass. A gross total resection was performed. Pathological examination revealed an AT/RT. Despite the dismal prognosis the patient decided not to undergo an abortion. For this reason postoperative accelerated hyperfractionated 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 died 11 months after diagnosis. Although survival was short, surgery and involved field radiotherapy yielded a progression-free interval of 9 months. This allowed the patient to carry pregnancy to term. Radiosurgery resulted in a complete remission of the first recurrence. Oral chemotherapy was not effective in controlling diffuse tumor spread.

Atypical teratoid rhabdoid tumor in adulthood

“…This contrasts with the published pediatric series in which posterior fossa localization is favored [11,12]. In adults, the cerebral hemispheres were the most frequently affected (23 cases) [1,5,6,7,8,9,10,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33], with frontal lobe being the most common (7 cases). This was followed by the sellar/suprasellar area (6 cases), pineal (5 cases) and thalamus (2 cases).…”

“…The pure AT/RT cases mostly occurred supratentorially (35 cases, 87.5%), with only 3 cases originating infratentorially in the cerebellum [6,7,8], and 2 cases in the spinal cord [9,10]. This contrasts with the published pediatric series in which posterior fossa localization is favored [11,12].…”

Adult-Onset Sellar and Suprasellar Atypical Teratoid Rhabdoid Tumor Treated with a Multimodal Approach: A Case Report

“…Now, detection of the loss of INI1 protein expression is a diagnostic hallmark of ATRT and malignant rhabdoid tumors (MRT) [ 5 ]. ATRT in adult patients have been rarely reported, but their prognosis is quite better than that of a pediatric group [ 3 , 6 , 7 , 8 ]. They are mainly located in the cerebral hemispheres, and ATRT in posterior fossa and spinal cord is not uncommon.…”

Atypical teratoid/rhabdoid tumor in sellar turcica in an adult: A case report and review of the literature

“…[3238] Adult cases are rare and, as far as we are aware, only 42 cases have been reported to date [Table 1]. [2345910111213141516171821232425262729303133343536373941] Furthermore, there are only seven adult cases arising in the sellar turcica described in the literature. [224253034]…”