Paediatria Croatica, Vol. 68 No. 1, 2024.
Stručni rad
https://doi.org/10.13112/PC.2024.5
Ventriculoperitoneal shunt as a risk factor for extraneural dissemination of atypical teratoid/rhabdoid tumor in children
Filip Murn
; Klinika za dječje bolesti Zagreb
Sandro Gašpar
Tonći Grmoja
Filip Jadrijević-Cvrlje
Ana Tripalo Batoš
Rhea Mužar
Goran Roić
Sažetak
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.
Ključne riječi
rhabdoid tumor; central nervous sytem NEOPLASMS; abdominal cavity; ventriculo-peritoneal shunt; magnetic resonance imagE
Hrčak ID:
317037
URI
Datum izdavanja:
31.3.2024.
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