[Primary intramedullary melanoma: Case report and literature review]. / Melanoma primario intramedular: reporte de caso y revisión de la literatura.

A dark pigmented intramedullary mass is very rarely encountered in daily practice, and poses a diagnostic challenge. Several entities have to be considered, including melanin-containing tumours (melanotic ependymoma and melanotic schwannoma) and melanocyte-containing tumours (melanocytoma, primary melanoma and melanoma metastases). The case is presented of a 47 year-old male with a pigmented intramedullary tumour located at T7-T8 level. Magnetic resonance images (MRI) revealed a tumour with hyperintensity on T1 and hypointensity on T2. The tumour was resected partially and treated with adjuvant radiotherapy. The diagnosis of primary intramedullary melanoma (PIM) was established based on histology and the absence of other lesions outside of the CNS. A literature review is presented on the other 26 PIM cases reported. PIM are extremely rare tumours, but are the most frequent cause of pigmented intramedullary tumour. Complete surgical resection is the treatment of choice whenever possible, followed by radiotherapy.

Anaplastic ependymoma with holocordal and intracranial meningeal carcinomatosis and holospinal bone metastases.

BACKGROUND AND IMPORTANCE: Ependymomas are the most frequent intramedullary neoplasms in adult patients. Anaplastic histology, extramedullary location, meningeal dissemination at initial diagnosis, and extraneural metastases are rare findings. We describe a case of extramedullary anaplastic ependymoma that presented with holocordal and intracranial leptomeningeal carcinomatosis and bone metastases in all the vertebral bodies and the sternum. Such an aggressive dissemination at initial diagnosis has not been previously reported. CLINICAL PRESENTATION: A 36-year-old woman presented with headache, multiple cranial nerve palsies, visual hallucinations, confusion, hemiparesis, hemihipoestesia, episodes of disconnection, and toxic syndrome. Magnetic resonance imaging and positron emission tomography scan revealed leptomeningeal carcinomatosis in the brainstem, the cerebellum, and along the whole spinal cord. Various nodular, intradural extramedullary lesions were present at multiple dorsal and lumbar levels. Metastatic bone disease affected all the vertebral bodies and various extraspinal bones. An intradural and bone biopsy was performed at L4, providing the diagnosis of anaplastic ependymoma (World Health Organization grade III) with focal neuronal differentiation. Despite chemotherapy, the patient's symptoms quickly progressed, and she died 7 weeks after diagnosis. CONCLUSION: To our knowledge, there are no previous descriptions of ependymomas with this extensive leptomeningeal, spinal, intracranial, and extraneural dissemination at clinical onset. Bone metastases in spinal ependymoma have not been previously reported.