Subtotal resection and adjuvant radiotherapy for a fourth ventricle rosette-forming glioneuronal tumor in a child: a case report and review of management strategies

Introduction and importance: Rosette-forming glioneuronal tumor (RGNT) is a rare World Health Organization grade I tumor of the central nervous system, most commonly found in the fourth ventricle. Preoperative diagnosis is challenging, and optimal management for incompletely resected tumors is not well-established. Case presentation: An 8-year-old boy presented with headaches and declining vision due to obstructive hydrocephalus caused by a large fourth ventricle tumor with extension into the cerebellopontine angle. After an endoscopic third ventriculostomy, the patient underwent maximal safe subtotal resection. Histopathological examination confirmed RGNT. Given the residual tumor, adjuvant radiotherapy was administered. Following treatment, the patient’s symptoms resolved, and follow-up imaging demonstrated stable residual disease. Clinical discussion: Although RGNTs are indolent tumors, their location in the fourth ventricle often precludes complete resection. Subtotal resection followed by adjuvant radiotherapy may represent a valid option in selected cases where complete resection is not feasible, with the aim of balancing tumor control and neurological preservation. This case adds to the limited pediatric experience and emphasizes the importance of a personalized, multidisciplinary approach with careful long-term surveillance. Conclusion: This case highlights the importance of individualized, multidisciplinary decision-making in the management of pediatric RGNTs of the fourth ventricle when complete resection is not feasible.