Diffuse Leptomeningeal Glioneuronal Tumor With Peritoneal Seeding After Ventriculoperitoneal Shunt in an Adult Patient: A Case Report.
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Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare central nervous system neoplasm characterized by diffuse leptomeningeal dissemination and variable molecular alterations. Although predominantly reported in pediatric patients, adult cases have increasingly been described. We report a 28-year-old woman who presented with progressive headache and hydrocephalus accompanied by diffuse leptomeningeal enhancement on MRI. She was initially misdiagnosed with tuberculous meningitis and underwent ventriculoperitoneal (VP) shunt placement. Progressive neurological deterioration led to open biopsy, which revealed oligodendroglial-like tumor cells with positive immunoreactivity for Olig2 and synaptophysin. Molecular analysis identified H3K28M, FGFR1, and ATRX mutations with 1p deletion. The patient received craniospinal irradiation followed by temozolomide chemotherapy, achieving temporary disease stabilization. Fifteen months after diagnosis, she developed massive ascites, and peritoneal biopsy confirmed metastatic dissemination consistent with DLGNT. Despite treatment, she died 18 months after initial diagnosis. This case highlights the diagnostic challenge of adult DLGNT, the molecular heterogeneity of the disease, and the potential risk of peritoneal dissemination following VP shunt placement. Awareness of possible extraneural spread is essential in managing this rare tumor.