Central neurocytomas with MIB-1 labeling index over 10% showing rapid tumor growth and dissemination

SummaryObjective and importance Central neurocytoma is recognized as a indolent intraventricular tumor arising from the ependyma around the foramen of Monro and anterior part of the lateral ventricles, and well demarcated from the brain parenchyma. Surgical removal can be curative without postoperative therapy. However, malignant central neurocytoma refractory to even aggressive treatment is known.Clinical presentation We report two cases of extraventricular central neurocytomas with significant vascular proliferation, mitoses, and MIB-1 labeling index of more than 10%.Intervention Subtotal removal for the one patient and open biopsy for other followed by radiotherapy with chemotherapy were performed. However, the disease progressed and dissemination occurred. Both patients subsequently died 23 and 18 month after the histological diagnosis was established.Conclusion Extraventricular central neurocytoma may present with frequent vascular proliferation and high MIB-1 labeling index. Even if they lack malignant histological findings like frequent mitosis and/or necrosis, the prognosis for such patients is very poor.