Primary Leptomeningeal Melanocytic Tumors of the Spine: Report of Two Cases and Review of the Literature

Abstract

Background: Primary leptomeningeal melanocytic tumors of the central nervous system are rare and, especially in the spine, less frequent compared with other entities. There is no consensus regarding the best care of these tumors.

Case description: We report 2 cases of primary leptomeningeal melanocytic tumors, 1 primary leptomeningeal melanoma (PLM) and 1 primary leptomeningeal melanocytoma (PLMC) of the upper cervical spine, and emphasize different surgical findings and clinical courses of these patients. A review of the literature according to primary leptomeningeal melanocytic tumors of the spine was done, especially to compare different treatment modalities in the younger history.

Conclusions: Primary melanocytic tumors of the spine are exceedingly rare. Before surgery it is difficult to make a correct diagnosis. Usually an unexpected intraoperative finding with consecutive histopathologic analyses leads to the final diagnosis. An accurate search for melanocytic tumors outside the central nervous system as a primary source is mandatory. PLMC has a better prognosis than PLM. There is no consensus regarding the adjuvant therapy, but patients with PLM should be given radiotherapy, chemotherapy, and immunotherapeutic approaches as immune checkpoint blockade after surgery. Communicating hydrocephalus is highly associated with PLM, but may occur in PLMC as well.

Keywords: Case report; Primary leptomeningeal melanocytoma; Primary leptomeningeal melanoma; Rare CNS diseases.