Ventriculoperitoneal shunt as a risk factor for extraneural dissemination of atypical teratoid/rhabdoid tumor in children

Abstract

Atypical teratoid/rhabdoid tumor (ATRT) is a rare but aggressive malignancy of the pediatric central nervous system (CNS), predominantly affecting children under the age of three. Despite its low frequency, ATRT constitutes a notable proportion of embryonal CNS tumors, particularly within the first year of life. The prognosis for ATRT patients is generally poor, with a significant decline in survival rates when metastasis is detected at diagnosis. Treatment typically involves a multimodal approach including surgery, radiation therapy, and chemotherapy, although outcomes remain suboptimal, especially in cases with younger age at diagnosis and metastases. ATRT exhibits distinct radiological and histopathological characteristics, presenting challenges in diagnosis and treatment planning. Additionally, extraneural metastatic spread of ATRT, although rare, can occur, with ventriculoperitoneal (VP) shunts identified as potential routes for dissemination. We present an extremely rare case of extraneural dissemination into the abdominal cavity along ventriculoperitoneal shunt in a 4-year-old boy. This is a type of extraneural dissemination in children that has only been published once and we are the first to present in Croatia.This case report highlights the diagnostic and therapeutic complexities associated with ATRT, emphasizing the importance of comprehensive staging, genetic evaluation, and vigilant surveillance for potential metastatic spreading via VP shunt. Furthermore, it emphasizes the need for further research to improve treatment outcomes and identify prognostic factors for risk stratification in pediatric patients with ATRT