Melanotic neuroectodermal tumor of the brain recurring 12 years after complete remission: case report

We describe a rare case of melanotic neuroectodermal tumor (MNT) of the brain recurring 12 years after complete remission. An 11-year-old girl initially presented with exotropia and bilateral papilledema. Magnetic resonance (MR) imaging revealed an intracranial extraaxial large tumor at the midfrontal region. T(1)-weighted MR imaging showed the tumor to be well delineated with homogeneous enhancement by gadolinium. The tumor was subtotally removed, and the histological diagnosis was MNT. The residual tumor became enlarged, so gamma knife radiosurgery was performed 5 months after initial surgery. The enhanced lesion disappeared, but another lesion emerged 3 years later. A second gamma knife radiosurgery was performed for this local recurrence. The enhanced lesion disappeared once again. Twelve years after the second gamma knife radiosurgery, another local recurrence was detected. This tumor was subtotally removed. Histological examination confirmed the same diagnosis of MNT. This case suggests that MNTs not completely resected need long-term follow up, even if complete remission was achieved after adjuvant therapy.