OBJECTIVE AND IMPORTANCE: Neurocytomas are typically located within the supratentorial ventricular system. Extraventricular neurocytomas are very rare, and this is only the second reported case of a pontine neurocytoma. We discuss the clinical presentation, histology, and treatment of these rare tumors. CLINICAL PRESENTATION: A 58-year-old man presented with a 4-month history of headache and unilateral facial and distal extremity paresthesia. Magnetic resonance imaging (MRI) scans demonstrated a 2.6 x 2.2-cm ring-enhancing cystic mass in the right pons. INTERVENTION: MRI-guided stereotactic biopsy yielded a diagnosis of atypical neurocytoma. Because of the location and malignant histological features of the tumor, the patient was initially treated with external beam radiation therapy. Several months later, MRI scans demonstrated tumor progression. The patient then underwent three rounds of temozolomide chemotherapy, during and after which his symptoms worsened. Aggressive subtotal resection of the tumor was achieved via a right suboccipital craniectomy. CONCLUSION: Twenty-eight months postoperatively, the patient is symptom free, and MRI scans demonstrate no evidence of residual or recurrent tumor.
OBJECTIVE AND IMPORTANCE: Neurocytomas are typically located within the supratentorial ventricular system. Extraventricular neurocytomas are very rare, and this is only the second reported case of a pontine neurocytoma. We discuss the clinical presentation, histology, and treatment of these rare tumors. CLINICAL PRESENTATION: A 58-year-old man presented with a 4-month history of headache and unilateral facial and distal extremity paresthesia. Magnetic resonance imaging (MRI) scans demonstrated a 2.6 x 2.2-cm ring-enhancing cystic mass in the right pons. INTERVENTION: MRI-guided stereotactic biopsy yielded a diagnosis of atypical neurocytoma. Because of the location and malignant histological features of the tumor, the patient was initially treated with external beam radiation therapy. Several months later, MRI scans demonstrated tumor progression. The patient then underwent three rounds of temozolomide chemotherapy, during and after which his symptoms worsened. Aggressive subtotal resection of the tumor was achieved via a right suboccipital craniectomy. CONCLUSION: Twenty-eight months postoperatively, the patient is symptom free, and MRI scans demonstrate no evidence of residual or recurrent tumor.
Authors: Marta Navas; Rafael G Sola; Cristina V Torres; Sophia F Shakur; Rafael Manzanares; Carlos Gordillo; Jose A Jimenez Journal: Childs Nerv Syst Date: 2014-05 Impact factor: 1.475