Primary Melanocytic Lesions of the CNS: Report of Five Cases

Abstract

Primary melanocytic tumours of the central nervous system (CNS) form a rare entity which is histologically and clinically distinct from metastatic cutaneous or retinal malignant melanoma. They can be classified into diffuse melanocytosis (diffuse melanosis), malignant melanoma and benign melanocytoma with a small number of intermediate variants. In this paper, 5 cases treated neurosurgically in our department for spinal or cerebral primary CNS malignant melanoma are reported. Primary tumors and further metastases were ruled out. Radiological, histological and clinical features are discussed. Compared to metastatic disease, primary CNS malignant melanoma shows a more benign clinical course with long-term tumour control and a good quality of life. A review of the literature which mainly consists of individual case reports, confirms this assessment. Although therapeutic experience for primary melanocytic lesions of the CNS is based on a small number of published cases, prognosis seems highly dependent on complete tumour resection. Adjuvant radiation seems to be of additional therapeutic benefit. Except for meningeosis melanomatosa chemotherapy must be regarded as experimental. Unfortunately, a standardised therapy concept is still lacking.

Long-Term Survival After Surgical Resection of Primary Spinal Malignant Melanoma -Case Report-

“…Primary CNS malignant melanoma has occurred in the spine, [4][5][6]10,11,19,26) suprasellar region, 3,6,24) cerebral region, 6,7,12,17) pineal region, 18) cerebellopontine angle, 13,25) spinal nerve root, 22,23) and leptomeninges. 16) Forty cases of primary spinal malignant melanoma have been reported, [4][5][6]10,11,19,26) including 14 with dissemination from the primary CNS malignant melanoma.…”

“…16) Forty cases of primary spinal malignant melanoma have been reported, [4][5][6]10,11,19,26) including 14 with dissemination from the primary CNS malignant melanoma. One case had liver and spleen metastases.…”

“…Primary spinal malignant melanoma is unusual, with the first case reported by Hirschberg in 1906, and only 40 cases since then. [4][5][6]10,11,19,26) The prognosis is poor, but primary CNS malignant melanoma including primary spinal malignant melanoma has a better prognosis than cutaneous melanoma. We treated a patient who presented with CNS dissemination 18 years after first treatment for primary spinal malignant melanoma.…”

Primary spinal melanoma of extramedullary origin: a report of three cases and systematic review of the literature

“…2 Clinically, PSM of extramedullary origin may be misdiagnosed as a neurogenic tumor (schwannoma), ependymoma, metastatic tumor, hematoma or meningioma. 3,[5][6][7][9][10][11][17][18][19] Ninety percent of melanomas found in the CNS are metastatic tumors. 8 A thorough physical examination is required to rule out melanoma in other organs outside of the spinal cord, before a diagnosis of PSM can be established.…”

“…Radiotherapy (from 30 to 60 Gy; median dose: 33.5 Gy) is effective for local control and is reserved for palliation of symptoms due to spinal cord compression or as an adjuvant to laminectomy. 5,6,10,11,17,18,21 Melanoma is also refractory to most systemic chemotherapies. Currently, it has not been confirmed whether postoperative radiotherapy or chemotherapy (such as interferon, methotrexate and temozolomide) has a significant role in overall survival time.…”