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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\u00a019-year period and summarizes the current body of literature on SNMM.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This retrospective analysis included 12\u00a0patients with SNMM treated from 2001 to 2019\u202fat an academic center. Additionally, a\u00a0literature review of the last 29\u00a0years on treatment and survival data of SNMM was conducted.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Main initial symptoms were epistaxis and nasal obstruction. Of the patients 9 underwent endoscopic surgery, 6 received adjuvant therapy. 3\u00a0patients who did not undergo surgery, received chemoradiotherapy, radiotherapy alone, and chemotherapy alone, respectively. At the time of diagnosis 2\u00a0patients had distant metastases and 4\u00a0patients developed distant metastases during the course of the disease. Mean overall survival (OS) was 30.6\u00a0months, 3\u2011year and 5\u2011year OS were 25%, and 18.2%, respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s00508-021-01847-6","type":"journal-article","created":{"date-parts":[[2021,4,12]],"date-time":"2021-04-12T12:03:12Z","timestamp":1618228992000},"page":"1137-1147","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Sinonasal mucosal melanoma: treatment strategies and survival rates for a\u00a0rare disease entity"],"prefix":"10.1007","volume":"133","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2530-9931","authenticated-orcid":false,"given":"Alexandros","family":"Andrianakis","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Kiss","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Pomberger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Axel","family":"Wolf","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dietmar","family":"Thurnher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter Valentin","family":"Tomazic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,4,12]]},"reference":[{"issue":"8","key":"1847_CR1","doi-asserted-by":"crossref","first-page":"1664","DOI":"10.1002\/(SICI)1097-0142(19981015)83:8<1664::AID-CNCR23>3.0.CO;2-G","volume":"83","author":"AE Chang","year":"1998","unstructured":"Chang\u00a0AE, Karnell\u00a0LH, Menck\u00a0HR. 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