P05.66 Metastatic atypical teratoid rhabdoid tumor (ATRT) of the central nervous system in the adult: A case report

Abstract

Background

Atypical teratoid rhabdoid tumor (ATRT) is a rare, highly malignant tumor of the central nervous system, ussually diagnosed in children less than 5 years of age. ATRT is rarely found in adults with average survival of these patients less than two years. Treatment regimens are often extrapolated from the pediatric experience and modified according to adults characteristics.

Case report

Here we present a case of a 35-year-old right-handed woman with metastatic ATRT of the brain first seen at our institution in march 2016. She complained on three months lasting loose of hearing and face paresis of the left side, with slight equilibrium disturbances. MRI of the head revealed four lesions intracranially, with homogeneous enhancement after contrast aplication. These lesions were located in both pontocerebellar angles, one in the roof of the forth ventricle and one deeply in the hypothalamic and campal region of the left brain. She was operated the largest tumor, supposed to be nevrinoma of the left acustic nerve. Histological examination confirmed ATRT. Before starting with pooperative radiation therapy (RT), she revealed spastic paraparesis with the subsequent MRI finding of spinal cord compression caused by an intradural, extramedullar tumor at C7 level. There were also found many homogeneously enhanced deposits along the spine, namely meningeal carcinomatoses. Following successful surgical resection of the metastases at C7 level, she started with craniospinal RT. Later on the disease progressed intracranially with signs of epileptic seazures and right hemiparesis. She was treated with additional RT of the head. From January 2017 till august 2017 she was treated with chemotherapy, exchanging VACA/ICE sheme, achieving a partial remission. In february 2018 disease progressed in the posterior fossa, she received palliative irradiation.

Conclusion

Presented case report is our first and the only adult patient of rare ATRT of the CNS. Although the prognosis is dismal in pediatric population, long term survival is possible in adult ATRT cases after combination of surgery, RT and chemotherapy. As the disease of our patient was metastatic at the time of diagnoses more two years ago, the patient is still alive with a relatively good quality of life for the most part of the time.