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Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease

BACKGROUND: Leiomyosarcoma of the head and neck is a rare cancer with high local aggressiveness. Radical surgery and adjuvant treatment offer the best chance for cure, nonetheless 5-years recurrence rate remains high. Despite international guidelines are available for soft tissue sarcomas, no recomm...

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Autores principales: Pinotti, Enrico, Sandini, Marta, Famularo, Simone, Jaconi, Marta, Romano, Fabrizio, Nespoli, Luca, Gianotti, Luca
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5461669/
https://www.ncbi.nlm.nih.gov/pubmed/28593036
http://dx.doi.org/10.1186/s13569-017-0078-2
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author Pinotti, Enrico
Sandini, Marta
Famularo, Simone
Jaconi, Marta
Romano, Fabrizio
Nespoli, Luca
Gianotti, Luca
author_facet Pinotti, Enrico
Sandini, Marta
Famularo, Simone
Jaconi, Marta
Romano, Fabrizio
Nespoli, Luca
Gianotti, Luca
author_sort Pinotti, Enrico
collection PubMed
description BACKGROUND: Leiomyosarcoma of the head and neck is a rare cancer with high local aggressiveness. Radical surgery and adjuvant treatment offer the best chance for cure, nonetheless 5-years recurrence rate remains high. Despite international guidelines are available for soft tissue sarcomas, no recommendations are specifically endorsed for leiomyosarcoma of the head and neck, due to the rarity of its presentation and consequently scarcity of data on long-term outcome. CASE PRESENTATION: A 50-year old woman, operated 10 years before for leiomyosarcoma of the nasal pit and with negative 5-years follow-up, was admitted to our ward for impairment of the hepatic function. Total-body CT scan detected multiple localizations at lungs, kidneys, pancreas, bones, muscles, lymph nodes and thyroid. The pathologic report after lung biopsy confirmed the diagnosis of metastasis from leiomyosarcoma and the patients was scheduled for first line chemo-radiotherapy. CONCLUSIONS: Despite adequate primary treatment, distant and disseminated metastatic disease may be not excluded in leiomyosarcoma of the head and neck.
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spelling pubmed-54616692017-06-07 Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease Pinotti, Enrico Sandini, Marta Famularo, Simone Jaconi, Marta Romano, Fabrizio Nespoli, Luca Gianotti, Luca Clin Sarcoma Res Case Report BACKGROUND: Leiomyosarcoma of the head and neck is a rare cancer with high local aggressiveness. Radical surgery and adjuvant treatment offer the best chance for cure, nonetheless 5-years recurrence rate remains high. Despite international guidelines are available for soft tissue sarcomas, no recommendations are specifically endorsed for leiomyosarcoma of the head and neck, due to the rarity of its presentation and consequently scarcity of data on long-term outcome. CASE PRESENTATION: A 50-year old woman, operated 10 years before for leiomyosarcoma of the nasal pit and with negative 5-years follow-up, was admitted to our ward for impairment of the hepatic function. Total-body CT scan detected multiple localizations at lungs, kidneys, pancreas, bones, muscles, lymph nodes and thyroid. The pathologic report after lung biopsy confirmed the diagnosis of metastasis from leiomyosarcoma and the patients was scheduled for first line chemo-radiotherapy. CONCLUSIONS: Despite adequate primary treatment, distant and disseminated metastatic disease may be not excluded in leiomyosarcoma of the head and neck. BioMed Central 2017-06-06 /pmc/articles/PMC5461669/ /pubmed/28593036 http://dx.doi.org/10.1186/s13569-017-0078-2 Text en © The Author(s) 2017 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Pinotti, Enrico
Sandini, Marta
Famularo, Simone
Jaconi, Marta
Romano, Fabrizio
Nespoli, Luca
Gianotti, Luca
Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease
title Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease
title_full Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease
title_fullStr Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease
title_full_unstemmed Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease
title_short Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease
title_sort disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. case report of a singular presentation of a rare disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5461669/
https://www.ncbi.nlm.nih.gov/pubmed/28593036
http://dx.doi.org/10.1186/s13569-017-0078-2
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