Şule Çalışkan KAMIŞ, Begül YAĞCI, Ayşe Selcan KOÇ
The Journal of Pediatric Academy - 2026;7(3):138-143
Pineoblastomas (PBs) are rare, aggressive tumors of the pineal region. They usually present with signs of increased intracranial pressure, whereas psychiatric manifestations are exceedingly uncommon. Despite multimodal treatment, the prognosis remains poor. We describe a 9-year-old girl who presented in 2016 with headache and diplopia. Brain computed tomography revealed a 16x16 mm mass in the posterior third ventricle associated with obstructive hydrocephalus. She underwent endoscopic third ventriculostomy, followed by gross total resection via an infratentorial supracerebellar approach. Histopathology confirmed pineoblastoma (World Health Organization Central Nervous System 2021 grade 4), which showed synaptophysin and cluster of differentiation 56 positivity with a Ki-67 index of 70-80%. Postoperatively, she received cranial radiotherapy and chemotherapy, the latter later modified due to hypersensitivity reactions. Recurrences and metastases were treated with bevacizumab, irinotecan, and temozolomide. In 2019, oncological treatment was discontinued at the family's request. The patient subsequently developed psychiatric manifestations, including mutism, agitation, and self-injurious behavior. Electroencephalography demonstrated frontal delta paroxysms. Serial magnetic resonance images revealed stable, non-enhancing nodules and periventricular gliosis without progression. Advanced imaging modalities and molecular subgrouping were not available; therefore, the differentiation between viable tumor and treatment-related changes remains uncertain. Remarkably, she has survived for more than seven years since diagnosis, with persistent radiological quiescence without ongoing oncological therapy. This case illustrates an exceptional clinical course of pediatric pineoblastoma, combining long-term survival and psychiatric symptoms that may reflect complex structural and treatment-related neurotoxicity rather than a confirmed paraneoplastic process. It underscores the importance of multidisciplinary management and highlights the diagnostic challenges in distinguishing tumor recurrence from post-treatment effects in long-term survivors.
