Department of Otorhinolaryngology, Head and Neck Surgery, Medical University of Graz, Auenbruggerplatz 26, 8036, Graz, Austria.
Wien Klin Wochenschr. 2021 Nov;133(21-22):1137-1147. doi: 10.1007/s00508-021-01847-6. Epub 2021 Apr 12.
Sinonasal mucosal melanoma (SNMM) is a rare disease entity comprising 0.4-1.3% of all melanomas. Surgery with free margins has been the primary treatment over decades. Neither the addition of radiotherapy nor chemotherapy could significantly improve outcome rates of this devastating malignancy. This study presents our clinical experience with SNMM over a 19-year period and summarizes the current body of literature on SNMM.
This retrospective analysis included 12 patients with SNMM treated from 2001 to 2019 at an academic center. Additionally, a literature review of the last 29 years on treatment and survival data of SNMM was conducted.
Main initial symptoms were epistaxis and nasal obstruction. Of the patients 9 underwent endoscopic surgery, 6 received adjuvant therapy. 3 patients who did not undergo surgery, received chemoradiotherapy, radiotherapy alone, and chemotherapy alone, respectively. At the time of diagnosis 2 patients had distant metastases and 4 patients developed distant metastases during the course of the disease. Mean overall survival (OS) was 30.6 months, 3‑year and 5‑year OS were 25%, and 18.2%, respectively.
Unspecific symptoms and hidden anatomic locations lead to delayed diagnosis and increased rates of metastatic dissemination. Distant metastasis is the main treatment failure in SNMM. Surgery with free margins remains the primary treatment for SNMM. Adjuvant radiotherapy might improve local control in individual cases but efficient systemic therapy is needed to improve outcome rates. To evaluate and define more effective targeted treatment options and improve outcome rates, homogeneous data and prospective multicentric analysis are needed.
鼻腔鼻窦黑色素瘤(sinonasal mucosal melanoma,SNMM)是一种罕见疾病,占所有黑色素瘤的 0.4%-1.3%。数十年来,广泛切除且切缘无肿瘤残留一直是主要的治疗方法。放疗和化疗的加入均不能显著提高这种破坏性肿瘤的生存率。本研究总结了 19 年来我们在 SNMM 方面的临床经验,并对 SNMM 的现有文献进行了总结。
本回顾性分析纳入了 2001 年至 2019 年在学术中心治疗的 12 例 SNMM 患者。此外,还对过去 29 年关于 SNMM 的治疗和生存数据的文献进行了综述。
主要的初始症状是鼻出血和鼻塞。9 例患者接受了内镜手术,6 例患者接受了辅助治疗。3 例未手术的患者分别接受了放化疗、单纯放疗和单纯化疗。诊断时,2 例患者有远处转移,4 例患者在病程中发生远处转移。总的中位生存时间(overall survival,OS)为 30.6 个月,3 年和 5 年的 OS 分别为 25%和 18.2%。
不典型的症状和隐匿的解剖位置导致诊断延迟,转移扩散的发生率增加。远处转移是 SNMM 治疗失败的主要原因。广泛切除且切缘无肿瘤残留仍然是 SNMM 的主要治疗方法。辅助放疗可能会提高局部控制率,但需要有效的全身治疗来提高生存率。为了评估和确定更有效的靶向治疗方案并提高生存率,需要进行同质数据和前瞻性多中心分析。