Neurologia medico-chirurgica
Online ISSN : 1349-8029
Print ISSN : 0470-8105
ISSN-L : 0470-8105
Case Reports
Intramedullary and Retroperitoneal Melanocytic Tumor Associated With Congenital Blue Nevus and Nevus Flammeus: An Uncommon Combination of Neurocutaneous Melanosis and Phacomatosis Pigmentovascularis—Case Report
Ryu KUROKAWAPhyo KIMToshiki KAWAMOTOHadzki MATSUDAShujiro HAYASHISoji YAMAZAKIAtsushi HATAMOCHIShozo MORIMitsugi SHIMODAKeiichi KUBOTA
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JOURNAL OPEN ACCESS

2013 Volume 53 Issue 10 Pages 730-734

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Abstract

Neurocutaneous melanosis (NCM) is a rare condition characterized by central nervous system melanocytic tumors associated with congenital melanocytic nevi. Phacomatosis pigmentovascularis (PPV) is an association of vascular nevus with pigmentary nevus. Aberrant maturation of neural crest-derived cells is considered to be related to pathogenesis in both conditions. However, association of NCM and PPV has not been reported to the best of our knowledge. Melanocytoma, which usually involves the leptomeninges or spinal cord, is extremely rare in the retroperitoneum. We present here a case of a patient with NCM, PPV, and melanocytic tumors in the spinal cord and retroperitoneum, which were treated surgically. A 40-year-old woman had a 2-year history of dysesthesia and weakness in the left leg. History included congenital giant blue nevus-like lesion in the trunk, a port-wine stain in the sacral area, and Caesarean section performed 8 years before, when diffuse pigmentation in the peritoneum was noted. Magnetic resonance (MR) imaging of the spine revealed an intramedullary tumor at T10 level with paramagnetic signal characteristics. The spinal cord tumor was totally removed, and the histological diagnosis was melanocytoma. Three months later, a left retroperitoneal mass with histological features of melanocytic tumor was removed. Neither tumors recurred and the patient stays ambulatory 4 years after the surgery. Multiple subtypes of melanocytic tumors with distinctive features of NCM and PPV can develop simultaneously, mimicking malignant melanoma. Gross total resection of each tumor, when indicated, is beneficial.

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© 2013 by The Japan Neurosurgical Society

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