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[黑素细胞瘤与脑膜黑素细胞增多症,相似却不同的病变]

[Melanocytoma and meningeal melanocytosis, similar but different lesions].

作者信息

Padilla-Vázquez Felipe, Escobar-de la Garma Víctor Hugo, Ayala-Arcipreste Arturo, Mendizábal-Guerra Rafael, Cuesta-Mejía Teresa

机构信息

Servicio de Neurocirugía, Hospital Juárez de México, Ciudad de México, México.

Servicio de Neurocirugía, Hospital Juárez de México, Ciudad de México, México.

出版信息

Cir Cir. 2017 May-Jun;85(3):273-278. doi: 10.1016/j.circir.2016.11.006. Epub 2017 Jan 23.

Abstract

BACKGROUND

Meningeal melanomatosis is an extra-axial well-encapsulated malignant tumour with diffuse meningeal growth and dark coloration (due to high melanin contents), while meningeal melanocytoma is the focalized benign variant. Melanocytic lesions may be secondary to melanoma or be histologically benign, however, their diffuse nature makes them impossible to cure. Melanocytosis is a diffuse tumour that can form solitary extra-axial tumours, which invades the parenchyma and presents signs of malignancy with increased mitosis and Ki67, observed in 1 to 6% of immunopathological exams. Melanoma of the leptomeninges, presents signs of malignancy with anaplastic cells, which cluster in fascicles of melanin in the cytoplasm, with more than 3 atypical mitoses per field and Ki67 presenting in more than 6% of the immunopathological fields analysed.

CLINICAL CASE

We present the case of a patient with long-term meningeal melanomatosis, with progressive neurologic deficit and characteristic radiologic features, and another case of meningeal melanocytoma.

CONCLUSIONS

Benign melanocytic neoplasms of the central nervous system must be treated aggressively in the early phases with strict follow-up to avoid progression to advanced phases that do not respond to any treatment method. Unfortunately, the prognosis for malignant melanocytic lesions is very poor irrespective of the method of treatment given.

摘要

背景

脑膜黑素沉着病是一种轴外的、包膜完整的恶性肿瘤,具有弥漫性脑膜生长且颜色较深(由于黑色素含量高),而脑膜黑素细胞瘤是局限性的良性变体。黑素细胞病变可能继发于黑色素瘤,也可能在组织学上为良性,然而,其弥漫性使其无法治愈。黑素细胞增多症是一种弥漫性肿瘤,可形成孤立的轴外肿瘤,侵袭实质并呈现恶性迹象,有丝分裂和Ki67增加,在1%至6%的免疫病理检查中可见。软脑膜黑色素瘤表现为恶性迹象,有间变细胞,这些细胞在细胞质中聚集成黑色素束,每个视野有超过3个非典型有丝分裂,且在分析的免疫病理视野中有超过6%的Ki67表达。

临床病例

我们报告一例长期患有脑膜黑素沉着病的患者,伴有进行性神经功能缺损和特征性影像学表现,以及另一例脑膜黑素细胞瘤病例。

结论

中枢神经系统良性黑素细胞肿瘤在早期必须积极治疗并严格随访,以避免进展到对任何治疗方法均无反应的晚期。不幸的是,无论采用何种治疗方法,恶性黑素细胞病变的预后都非常差。

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