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Laryngeal embryonal rhabdomyosarcoma in an adult - A case presentation in the eyes of geneticists and clinicians

1. ABSTRACT: BACKGROUND: Rhabdomyosarcoma is a solid tumor, resulting from dysregulation of the skeletal myogenesis program. For rhabdomyosarcomas (RMS) with a predilection for the head and neck, genitourinary tract, extremities, trunk, retroperitoneum, the larynx is still an unusual site. Till now...

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Autores principales: Kukwa, Wojciech, Wojtowicz, Piotr, Jagielska, Beata, Sobczyk, Grzegorz, Kukwa, Andrzej, Czarnecka, Anna M
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3118944/
https://www.ncbi.nlm.nih.gov/pubmed/21569414
http://dx.doi.org/10.1186/1471-2407-11-166
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author Kukwa, Wojciech
Wojtowicz, Piotr
Jagielska, Beata
Sobczyk, Grzegorz
Kukwa, Andrzej
Czarnecka, Anna M
author_facet Kukwa, Wojciech
Wojtowicz, Piotr
Jagielska, Beata
Sobczyk, Grzegorz
Kukwa, Andrzej
Czarnecka, Anna M
author_sort Kukwa, Wojciech
collection PubMed
description 1. ABSTRACT: BACKGROUND: Rhabdomyosarcoma is a solid tumor, resulting from dysregulation of the skeletal myogenesis program. For rhabdomyosarcomas (RMS) with a predilection for the head and neck, genitourinary tract, extremities, trunk, retroperitoneum, the larynx is still an unusual site. Till now only several cases of this laryngeal tumor have been described in world literature in the adult population. The entire spectrum of genetic factors underlying RMS development and progression is unclear until today. Multiple signaling pathways seem to be involved in ERMS development and progression. CASE PRESENTATION: In this paper we report an interesting RMS case in which the disease was located within the glottic region. We report an embryonal rhabdomyosarcoma of the larynx in 33 year-old man. After unsuccessful chemotherapy hemilaryngectomy was performed. In follow up CT no signs of recurrence were found. Recently patient is recurrence free for 62 months. CONCLUSIONS: Considering the histological diagnosis and the highly aggressive nature of the lesion for optimal diagnosis positron electron tomography (PET) and computerized tomography (CT) of the neck and thorax should be performed. At this time surgical treatment with adjuvant radiotherapy seems to be the treatment of choice for this disease. Rhabdomyosarcoma of the larynx has a better prognosis than elsewhere in the body, probably because of its earlier recognition and accessibility to radical surgery.
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spelling pubmed-31189442011-06-22 Laryngeal embryonal rhabdomyosarcoma in an adult - A case presentation in the eyes of geneticists and clinicians Kukwa, Wojciech Wojtowicz, Piotr Jagielska, Beata Sobczyk, Grzegorz Kukwa, Andrzej Czarnecka, Anna M BMC Cancer Case Report 1. ABSTRACT: BACKGROUND: Rhabdomyosarcoma is a solid tumor, resulting from dysregulation of the skeletal myogenesis program. For rhabdomyosarcomas (RMS) with a predilection for the head and neck, genitourinary tract, extremities, trunk, retroperitoneum, the larynx is still an unusual site. Till now only several cases of this laryngeal tumor have been described in world literature in the adult population. The entire spectrum of genetic factors underlying RMS development and progression is unclear until today. Multiple signaling pathways seem to be involved in ERMS development and progression. CASE PRESENTATION: In this paper we report an interesting RMS case in which the disease was located within the glottic region. We report an embryonal rhabdomyosarcoma of the larynx in 33 year-old man. After unsuccessful chemotherapy hemilaryngectomy was performed. In follow up CT no signs of recurrence were found. Recently patient is recurrence free for 62 months. CONCLUSIONS: Considering the histological diagnosis and the highly aggressive nature of the lesion for optimal diagnosis positron electron tomography (PET) and computerized tomography (CT) of the neck and thorax should be performed. At this time surgical treatment with adjuvant radiotherapy seems to be the treatment of choice for this disease. Rhabdomyosarcoma of the larynx has a better prognosis than elsewhere in the body, probably because of its earlier recognition and accessibility to radical surgery. BioMed Central 2011-05-12 /pmc/articles/PMC3118944/ /pubmed/21569414 http://dx.doi.org/10.1186/1471-2407-11-166 Text en Copyright ©2011 Kukwa et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Kukwa, Wojciech
Wojtowicz, Piotr
Jagielska, Beata
Sobczyk, Grzegorz
Kukwa, Andrzej
Czarnecka, Anna M
Laryngeal embryonal rhabdomyosarcoma in an adult - A case presentation in the eyes of geneticists and clinicians
title Laryngeal embryonal rhabdomyosarcoma in an adult - A case presentation in the eyes of geneticists and clinicians
title_full Laryngeal embryonal rhabdomyosarcoma in an adult - A case presentation in the eyes of geneticists and clinicians
title_fullStr Laryngeal embryonal rhabdomyosarcoma in an adult - A case presentation in the eyes of geneticists and clinicians
title_full_unstemmed Laryngeal embryonal rhabdomyosarcoma in an adult - A case presentation in the eyes of geneticists and clinicians
title_short Laryngeal embryonal rhabdomyosarcoma in an adult - A case presentation in the eyes of geneticists and clinicians
title_sort laryngeal embryonal rhabdomyosarcoma in an adult - a case presentation in the eyes of geneticists and clinicians
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3118944/
https://www.ncbi.nlm.nih.gov/pubmed/21569414
http://dx.doi.org/10.1186/1471-2407-11-166
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