Dermatopathology Service, Harvard Medical School, Massachusetts General Hospital, WRN825 Mass General Hospital, 55 Fruit Street, Boston, MA 02114, USA.
Dermatopathology Service, Harvard Medical School, Massachusetts General Hospital, WRN825 Mass General Hospital, 55 Fruit Street, Boston, MA 02114, USA.
Surg Pathol Clin. 2024 Dec;17(4):667-682. doi: 10.1016/j.path.2024.07.006. Epub 2024 Aug 17.
Sinonasal mucosal melanoma (SNMM) is a rare aggressive tumor often diagnosed at advanced stage. SNMM is associated with a greater than 50% rate of recurrence and a disease-specific 5 year survival rate of less than 25%. Sinonasal anatomy may contribute to difficulty in early detection; SNMM is usually asymptomatic until bulky and polypoidal. Presenting clinical symptoms are often nonspecific. The histopathologic features of SNMM are quite variable and immunohistochemical analysis is usually necessary for diagnosis. Mucosal melanomas lack ultraviolet signature, have low somatic mutational burden, and are reported to have more genomic instability manifested as structural variants, deletions, and amplifications.
鼻腔鼻窦黏膜黑色素瘤(SNMM)是一种罕见的侵袭性肿瘤,常在晚期诊断。SNMM 复发率大于 50%,疾病特异性 5 年生存率小于 25%。鼻腔鼻窦解剖结构可能导致早期检测困难;SNMM 通常无症状,直到肿块和息肉样。临床表现通常是非特异性的。SNMM 的组织病理学特征差异很大,通常需要免疫组织化学分析来诊断。黏膜黑色素瘤缺乏紫外线特征,体细胞突变负担低,据报道具有更多的基因组不稳定性,表现为结构变异、缺失和扩增。