Pineoblastoma

Pineoblastoma is a rare, highly malignant brain tumor that arises from the pineal gland, located deep within the center of the brain. It is classified as a WHO Grade IV embryonal tumor, often affecting children and young adolescents, particularly between the ages of 1 to 20 years. Pineoblastomas belong to the pineal parenchymal tumor group and are closely related to medulloblastomas in terms of aggressiveness and histological appearance. Due to its location near the brainstem and cerebrospinal fluid (CSF) pathways, pineoblastoma often presents with early symptoms of raised intracranial pressure and has a high propensity for CSF dissemination, making prompt neurosurgical intervention and multidisciplinary management essential. 🌍 Pineoblastoma in Bangladesh: An Urgent Pediatric Neurosurgical Concern In Bangladesh, awareness about pediatric pineal region tumors remains low, especially in rural and semi-urban areas. Children presenting with morning headaches, vomiting, double vision, or squint are often misdiagnosed with migraines, eye diseases, or gastrointestinal disorders. Lack of timely MRI brain and absence of neuro-oncology teams in many hospitals result in critical delays, during which time the tumor may spread through the CSF. Dr. Md. Nafaur Rahman, a national expert in pediatric neurosurgery, leads the management of complex pineal tumors at the National Institute of Neurosciences & Hospital (NINS) and the Bangladesh Paediatric Neurocare Centre, utilizing advanced surgical tools and multidisciplinary collaboration. 🧬 Causes and Pathogenesis Pineoblastomas originate from primitive neuroectodermal cells of the pineal gland Genetic predispositions such as DICER1 mutations or RB1 gene involvement may be present Sometimes associated with trilateral retinoblastoma in children (involving both eyes and pineal gland) More common in younger children under 5 years, but can affect teenagers too 🧒 Symptoms of Pineoblastoma in Children Early morning headache Nausea and vomiting due to hydrocephalus Parinaud's syndrome – upward gaze palsy, dilated pupils Double vision or squint (due to pressure on midbrain structures) Lethargy, poor appetite, irritability Rapid increase in head size in infants Seizures, imbalance, or weakness in advanced cases In Bangladesh, these signs are often overlooked, delaying referral to pediatric neurosurgery units. 🔍 Diagnosis of Pineoblastoma 🧠 MRI Brain with Contrast Large, solid-cystic tumor in the pineal region Shows heterogeneous enhancement Often associated with obstructive hydrocephalus May show drop metastases in spine on MRI 💉 CSF Analysis Performed after hydrocephalus management Cytology to detect tumor cell dissemination 🧬 Histopathology Confirms diagnosis post-surgery or biopsy Small round blue cells with high mitotic activity May require immunohistochemistry and molecular testing (e.g., DICER1 mutation) 🛠️ Treatment Approach 1. Surgical Management Initial CSF diversion via Endoscopic Third Ventriculostomy (ETV) or VP Shunt for hydrocephalus Followed by tumor biopsy or gross total resection, depending on feasibility Microsurgical removal is critical to reduce tumor burden before chemo/radiotherapy 2. Chemotherapy Multi-agent regimens used, often based on medulloblastoma protocols Helps control microscopic spread and reduce tumor mass Used both before and after radiation in children 3. Radiotherapy Craniospinal irradiation (CSI) is standard due to CSF dissemination risk Boost to pineal region Modified in children under 3 years to reduce cognitive side effects 🔄 Prognosis Prognosis is guarded, especially in young children and cases with CSF spread 5-year survival can reach 60–70% in selected cases with gross total resection and adjuvant therapy Regular MRI follow-up required for recurrence monitoring Long-term effects may include hormonal dysfunction, cognitive deficits, or visual impairment ⚠️ Risks of Delayed Treatment Increased intracranial pressure → herniation → coma or death CSF metastasis to spinal cord, causing paralysis or bladder/bowel issues Permanent neurological disability Treatment becomes less effective in advanced stages 👨‍⚕️ Why Trust Dr. Md. Nafaur Rahman? One of Bangladesh’s top pediatric neurosurgeons for pineal and posterior fossa tumors Expertise in ETV, neuro-navigation guided microsurgery, and tumor biopsy Offers coordinated care with oncologists, endocrinologists, and radiotherapists Based at NINS, Bangladesh’s largest neuroscience hospital Available for consultation at Bangladesh Paediatric Neurocare Centre 📞 Book an Appointment Now Dr. Md. Nafaur Rahman Assistant Professor, Pediatric Neurosurgery, National Institute of Neurosciences & Hospital (NINS) Chief Consultant, Bangladesh Paediatric Neurocare Centre 📞 Serial/Appointment: 01912988182 | 01607033535 🌐 Website: www.neurosurgeonnafaur.com