Cargando…

Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity: A single center experience and review of literature

BACKGROUND: 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 mali...

Descripción completa

Detalles Bibliográficos
Autores principales: Andrianakis, Alexandros, Kiss, Peter, Pomberger, Markus, Wolf, Axel, Thurnher, Dietmar, Tomazic, Peter Valentin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Vienna 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8599389/
https://www.ncbi.nlm.nih.gov/pubmed/33844113
http://dx.doi.org/10.1007/s00508-021-01847-6
_version_ 1784600941124648960
author Andrianakis, Alexandros
Kiss, Peter
Pomberger, Markus
Wolf, Axel
Thurnher, Dietmar
Tomazic, Peter Valentin
author_facet Andrianakis, Alexandros
Kiss, Peter
Pomberger, Markus
Wolf, Axel
Thurnher, Dietmar
Tomazic, Peter Valentin
author_sort Andrianakis, Alexandros
collection PubMed
description BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
format Online
Article
Text
id pubmed-8599389
institution National Center for Biotechnology Information
language English
publishDate 2021
publisher Springer Vienna
record_format MEDLINE/PubMed
spelling pubmed-85993892021-11-24 Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity: A single center experience and review of literature Andrianakis, Alexandros Kiss, Peter Pomberger, Markus Wolf, Axel Thurnher, Dietmar Tomazic, Peter Valentin Wien Klin Wochenschr Original Article BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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. Springer Vienna 2021-04-12 2021 /pmc/articles/PMC8599389/ /pubmed/33844113 http://dx.doi.org/10.1007/s00508-021-01847-6 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Original Article
Andrianakis, Alexandros
Kiss, Peter
Pomberger, Markus
Wolf, Axel
Thurnher, Dietmar
Tomazic, Peter Valentin
Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity: A single center experience and review of literature
title Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity: A single center experience and review of literature
title_full Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity: A single center experience and review of literature
title_fullStr Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity: A single center experience and review of literature
title_full_unstemmed Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity: A single center experience and review of literature
title_short Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity: A single center experience and review of literature
title_sort sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity: a single center experience and review of literature
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8599389/
https://www.ncbi.nlm.nih.gov/pubmed/33844113
http://dx.doi.org/10.1007/s00508-021-01847-6
work_keys_str_mv AT andrianakisalexandros sinonasalmucosalmelanomatreatmentstrategiesandsurvivalratesforararediseaseentityasinglecenterexperienceandreviewofliterature
AT kisspeter sinonasalmucosalmelanomatreatmentstrategiesandsurvivalratesforararediseaseentityasinglecenterexperienceandreviewofliterature
AT pombergermarkus sinonasalmucosalmelanomatreatmentstrategiesandsurvivalratesforararediseaseentityasinglecenterexperienceandreviewofliterature
AT wolfaxel sinonasalmucosalmelanomatreatmentstrategiesandsurvivalratesforararediseaseentityasinglecenterexperienceandreviewofliterature
AT thurnherdietmar sinonasalmucosalmelanomatreatmentstrategiesandsurvivalratesforararediseaseentityasinglecenterexperienceandreviewofliterature
AT tomazicpetervalentin sinonasalmucosalmelanomatreatmentstrategiesandsurvivalratesforararediseaseentityasinglecenterexperienceandreviewofliterature