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口鼻区域原发性无色素性黏膜黑色素瘤:两例新病例报告及文献综述

Primary amelanotic mucosal melanoma of the oronasal region: report of two new cases and literature review.

作者信息

Paulo Luiz Fernando Barbosa de, Servato João Paulo Silva, Rosa Roberta Rezende, Oliveira Maiolino Thomaz Fonseca, Faria Paulo Rogério de, Silva Sindeval José da, Cardoso Sergio Vitorino, Loyola Adriano Mota

机构信息

Oral Pathology Area, School of Dentistry, Federal University of Uberlândia, Uberlândia, Brazil.

Oral and Maxillofacial Surgery Department, Clinical Hospital, Federal University of Uberlândia, Uberlândia, Brazil.

出版信息

Oral Maxillofac Surg. 2015 Dec;19(4):333-9. doi: 10.1007/s10006-015-0501-x. Epub 2015 May 3.

Abstract

Primary amelanotic mucosal melanoma is a rare entity with challenging histopathological features. Because these tumors are thought to be biologically more aggressive, they have a poorer prognosis than that of pigmented melanomas. In this work, we present a literature review about the clinical, histopathological, and immunohistochemical features of primary amelanotic mucosal melanoma of the oronasal region and report two new cases. Amelanotic mucosal melanoma commonly affects men in the seventh decade of life and tend to have a poor prognosis, as seen by the high incidence of metastasis, recurrences, and, ultimately, death. There is a similar pattern in the clinic-pathological predilections (such as age, gender, primary site, and metastatic potential) of amelanotic mucosal melanoma when comparing with data reported for pigmented lesions. This work reinforces knowledge about amelanotic mucosal melanomas and epidemiologic predilections. The optimal management of this lesion remains controversial.

摘要

原发性无色素性黏膜黑色素瘤是一种罕见的疾病,具有具有挑战性的组织病理学特征。由于这些肿瘤被认为在生物学上更具侵袭性,它们的预后比色素性黑色素瘤更差。在这项工作中,我们对口腔颌面部原发性无色素性黏膜黑色素瘤的临床、组织病理学和免疫组化特征进行了文献综述,并报告了两例新病例。无色素性黏膜黑色素瘤通常影响70岁左右的男性,预后往往较差,转移、复发以及最终死亡的发生率都很高。与色素性病变报告的数据相比,无色素性黏膜黑色素瘤在临床病理倾向(如年龄、性别、原发部位和转移潜能)方面有相似的模式。这项工作加强了对无色素性黏膜黑色素瘤及其流行病学倾向的认识。这种病变的最佳治疗方法仍存在争议。

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