Yang Chenlong, Fang Jingyi, Li Guang, Yang Jun, Xu Yulun
Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, No. 6 Tiantan Xili, Dongcheng District, Beijing 100050, China.
Department of Neuro-pathology, Beijing Neurosurgical Institute, Capital Medical University, No. 6 Tiantan Xili, Dongcheng District, Beijing 100050, China.
Spine J. 2016 Aug;16(8):e553-9. doi: 10.1016/j.spinee.2016.03.007. Epub 2016 Mar 9.
Meningeal melanocytoma is an extremely rare pigmented tumor derived from leptomeningeal melanocytes. By and large, it is considered to be a well-differentiated and slow-growing benign lesion. Generally, meningeal melanocytomas are solitary lesions, and the occurrence of the primary multifocal form in the central nervous system is exceedingly rare; it has been previously reported in only six cases.
The present report illustrates a 41-year-old woman with primary multifocal meningeal melanocytoma in the spinal canal. Contrary to earlier reports, the tumors presented with a scattered appearance mimicking neurofibromatosis.
This study is a case report and review of literature.
On admission, the cerebral magnetic resonance images of the patient were normal, whereas the spinal magnetic resonance images showed scattered multifocal nodules mimicking neurofibromatosis. Surgical resection of the responsible lesions was scheduled. In addition to this case presentation, relevant previous reports were reviewed, and the challenging diagnosis, management, and prognosis of meningeal melanocytoma are discussed.
Gross total resection of the two largest lesions was achieved, and histopathological examinations confirmed the diagnosis. Despite the benign histopathological findings, the patient had an aggressive clinical course. On follow-up at 18 months after surgery, she succumbed to the disease.
Clinicians should be alert to a potential aggressive clinical course of meningeal melanocytoma, despite its benign histopathological nature. Of particular note is multifocality and diffuse leptomeningeal hyperpigmentation, which may suggest a poor prognosis. A combined treatment including surgical resection and adjuvant radiotherapy should be considered, and long-term close follow-up is necessary.
脑膜黑素细胞瘤是一种极其罕见的起源于软脑膜黑素细胞的色素性肿瘤。总体而言，它被认为是一种分化良好、生长缓慢的良性病变。通常，脑膜黑素细胞瘤为孤立性病变，中枢神经系统原发性多灶性形式极为罕见；此前仅有6例报道。
本报告描述了一名41岁患有椎管内原发性多灶性脑膜黑素细胞瘤的女性。与早期报告不同，这些肿瘤呈散在分布，类似神经纤维瘤病。
本研究为病例报告及文献复习。
入院时，患者的脑部磁共振成像正常，而脊髓磁共振成像显示散在的多灶性结节，类似神经纤维瘤病。计划对相关病变进行手术切除。除了本病例报告外，还复习了既往相关报告，并讨论了脑膜黑素细胞瘤具有挑战性的诊断、治疗及预后。
成功实现了对两个最大病变的全切，组织病理学检查确诊。尽管组织病理学表现为良性，但患者临床病程呈侵袭性。术后18个月随访时，患者死于该疾病。
临床医生应警惕脑膜黑素细胞瘤潜在的侵袭性临床病程，尽管其组织病理学性质为良性。特别值得注意的是多灶性和软脑膜弥漫性色素沉着，这可能提示预后不良。应考虑手术切除联合辅助放疗的综合治疗，且需要长期密切随访。
