Purpose or Case Report: Embryonal tumor with multilayered rosettes (ETMR) is a rare and aggressive type of brain tumor that primarily affects infants and young children. ETMRs are highly malignant tumors with poor prognosis. The VP shunt is used to drain excess cerebrospinal fluid into the abdominal cavity, can inadvertently provide a pathway for tumor cells to travel from the central nervous system to the peritoneum, especially in high grade CNS tumors. Here, we present a young boy diagnosed with ETMR in the posterior fossa with peritoneal dissemination related to VP shunt.
19 month old boy was first noted to have a well defined 3 cm mass in the right pons. It demonstrates diffusion restriction and lack of contrast enhancement. Tissue biopsy was performed first, that came with the diagnosis of ETMR. Then, gross total resection of the mass was performed with VP shunt placement. During therapy, the follow up scans demonstrate residual/recurrent disease along the resection margins. Furthermore, the disease continued to progress and 10 months later from the first surgery, 2nd surgery was performed for tumor debulking from the brainstem. But the diseased continued to progress after the 2nd surgery under therapy with new development of intraspinal leptomeningeal tumor dissemination. 2 months later after the 2nd surgery, multiple peritoneal masses and excess ascitis were noted in the follow-up Spine MRI, in the presence of a VP shunt.
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Conclusions: It is possible that neoplastic cells found in the cerebrospinal fluid may migrate through the shunt and implant in the peritoneal cavity, producing multiple metastases. Shunt-related metastasis should be recognized as a potential risk and an important clinical presentation. This case highlights this rare complication. Understanding this phenomenon is important for developing effective monitoring and treatment strategies.