Intramedullary and Retroperitoneal Melanocytic Tumor Associated With Congenital Blue Nevus and Nevus Flammeus: An Uncommon Combination of Neurocutaneous Melanosis and Phacomatosis Pigmentovascularis—Case Report
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- KUROKAWA Ryu
- Department of Neurologic Surgery, Dokkyo Medical University
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- KIM Phyo
- Department of Neurologic Surgery, Dokkyo Medical University
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- KAWAMOTO Toshiki
- Department of Neurologic Surgery, Dokkyo Medical University
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- MATSUDA Hadzki
- Department of Anatomic and Diagnostic Pathology, Dokkyo Medical University
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- HAYASHI Shujiro
- Department of Dermatology, Dokkyo Medical University
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- YAMAZAKI Soji
- Department of Dermatology, Dokkyo Medical University
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- HATAMOCHI Atsushi
- Department of Dermatology, Dokkyo Medical University
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- MORI Shozo
- Second Department of Surgery, Dokkyo Medical University
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- SHIMODA Mitsugi
- Second Department of Surgery, Dokkyo Medical University
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- KUBOTA Keiichi
- Second Department of Surgery, Dokkyo Medical University
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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.
収録刊行物
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- Neurologia medico-chirurgica
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Neurologia medico-chirurgica 53 (10), 730-734, 2013
一般社団法人 日本脳神経外科学会
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詳細情報 詳細情報について
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- CRID
- 1390001205055969536
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- NII論文ID
- 10031191647
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- NII書誌ID
- AN00358613
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- COI
- 1:STN:280:DC%2BC2c%2FitFShtA%3D%3D
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- ISSN
- 13498029
- 04708105
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- PubMed
- 24077274
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- 本文言語コード
- en
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- データソース種別
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- JaLC
- Crossref
- PubMed
- CiNii Articles
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- 抄録ライセンスフラグ
- 使用不可