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The Case for Primary Salivary Rhabdomyosarcoma

Rhabdomyosarcomas of the parotid and submandibular glands have the histological appearance of a skeletal muscle tumor yet can be found in tissue with no striated muscular elements. We examine the potential cell-of-origin for rhabdomyosarcoma and whether salivary tumors represent primary malignancy o...

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Autores principales: Geltzeiler, Mathew, Li, Guangheng, Abraham, Jinu, Keller, Charles
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4381625/
https://www.ncbi.nlm.nih.gov/pubmed/25883905
http://dx.doi.org/10.3389/fonc.2015.00074
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author Geltzeiler, Mathew
Li, Guangheng
Abraham, Jinu
Keller, Charles
author_facet Geltzeiler, Mathew
Li, Guangheng
Abraham, Jinu
Keller, Charles
author_sort Geltzeiler, Mathew
collection PubMed
description Rhabdomyosarcomas of the parotid and submandibular glands have the histological appearance of a skeletal muscle tumor yet can be found in tissue with no striated muscular elements. We examine the potential cell-of-origin for rhabdomyosarcoma and whether salivary tumors represent primary malignancy or metastasis. We have previously established genetically engineered mouse models of rhabdomyosarcoma. In these mice, rhabdomyosarcoma is only induced when a Pax3:Foxo1 fusion oncogene is activated with concurrent loss of p53 function (for alveolar rhabdomyosarcoma) or loss of p53 function alone (for embryonal rhabdomyosarcoma) using Cre-lox technology. These mutations are only activated under the control of promoters specific for selected cell lineages, previously thought to be myogenesis-restricted. RT-PCR and immunohistochemistry for lineage-specific promoter gene products reveal these promoters are active in wild-type mouse salivary gland. Given that mouse rhabdomyosarcoma frequently originates in the salivary glands and these myogenic-related promoters are normally expressed in salivary tissue, a high likelihood exists that the salivary gland contains a cell-of-origin of this muscle-related cancer.
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spelling pubmed-43816252015-04-16 The Case for Primary Salivary Rhabdomyosarcoma Geltzeiler, Mathew Li, Guangheng Abraham, Jinu Keller, Charles Front Oncol Oncology Rhabdomyosarcomas of the parotid and submandibular glands have the histological appearance of a skeletal muscle tumor yet can be found in tissue with no striated muscular elements. We examine the potential cell-of-origin for rhabdomyosarcoma and whether salivary tumors represent primary malignancy or metastasis. We have previously established genetically engineered mouse models of rhabdomyosarcoma. In these mice, rhabdomyosarcoma is only induced when a Pax3:Foxo1 fusion oncogene is activated with concurrent loss of p53 function (for alveolar rhabdomyosarcoma) or loss of p53 function alone (for embryonal rhabdomyosarcoma) using Cre-lox technology. These mutations are only activated under the control of promoters specific for selected cell lineages, previously thought to be myogenesis-restricted. RT-PCR and immunohistochemistry for lineage-specific promoter gene products reveal these promoters are active in wild-type mouse salivary gland. Given that mouse rhabdomyosarcoma frequently originates in the salivary glands and these myogenic-related promoters are normally expressed in salivary tissue, a high likelihood exists that the salivary gland contains a cell-of-origin of this muscle-related cancer. Frontiers Media S.A. 2015-04-01 /pmc/articles/PMC4381625/ /pubmed/25883905 http://dx.doi.org/10.3389/fonc.2015.00074 Text en Copyright © 2015 Geltzeiler, Li, Abraham and Keller. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Oncology
Geltzeiler, Mathew
Li, Guangheng
Abraham, Jinu
Keller, Charles
The Case for Primary Salivary Rhabdomyosarcoma
title The Case for Primary Salivary Rhabdomyosarcoma
title_full The Case for Primary Salivary Rhabdomyosarcoma
title_fullStr The Case for Primary Salivary Rhabdomyosarcoma
title_full_unstemmed The Case for Primary Salivary Rhabdomyosarcoma
title_short The Case for Primary Salivary Rhabdomyosarcoma
title_sort case for primary salivary rhabdomyosarcoma
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4381625/
https://www.ncbi.nlm.nih.gov/pubmed/25883905
http://dx.doi.org/10.3389/fonc.2015.00074
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