Embryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical Course

Rhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents. Embryonal rhabdomyosarcoma (ERMS), its most common subtype, is a malignant soft tissue tumor with morphologic and immunophenotypic features of embryonic skeletal muscle. The histologic findings in ERMS typically inc...

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Autores principales:	Maniraj Jeyaraju, Regina Ann Macatangay, Ashley Taylor-King Munchel, Teresa Anne York, Elizabeth A. Montgomery, Michael E. Kallen
Formato:	article
Lenguaje:	EN
Publicado:	Hindawi Limited 2021
Materias:	Pathology RB1-214
Acceso en línea:	https://doaj.org/article/d355d9e78bcf4b318dd78fdcb5189f21
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spelling	oai:doaj.org-article:d355d9e78bcf4b318dd78fdcb5189f212021-11-22T01:11:21ZEmbryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical Course2090-679X10.1155/2021/1800854https://doaj.org/article/d355d9e78bcf4b318dd78fdcb5189f212021-01-01T00:00:00Zhttp://dx.doi.org/10.1155/2021/1800854https://doaj.org/toc/2090-679XRhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents. Embryonal rhabdomyosarcoma (ERMS), its most common subtype, is a malignant soft tissue tumor with morphologic and immunophenotypic features of embryonic skeletal muscle. The histologic findings in ERMS typically include a range of differentiation in rhabdomyoblasts from primitive to terminally differentiated forms, and the latter become more prominent after chemotherapy-induced cytodifferentiation. Several reports have shown therapy-related cytodifferentiation to portend a good prognosis in ERMS. We discuss the case of a pediatric patient who presented with ERMS of the orbit. Although her tumor showed extensive posttreatment cytodifferentiation and several other good prognostic clinicopathologic factors, it pursued an aggressive course, resulting in early metastasis and death. This case represents an unusual course and may be instructive as to the clinicopathologic features impacting prognostication, and ultimately the biology, of this aggressive family of tumors.Maniraj JeyarajuRegina Ann MacatangayAshley Taylor-King MunchelTeresa Anne YorkElizabeth A. MontgomeryMichael E. KallenHindawi LimitedarticlePathologyRB1-214ENCase Reports in Pathology, Vol 2021 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Pathology RB1-214
spellingShingle	Pathology RB1-214 Maniraj Jeyaraju Regina Ann Macatangay Ashley Taylor-King Munchel Teresa Anne York Elizabeth A. Montgomery Michael E. Kallen Embryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical Course
description	Rhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents. Embryonal rhabdomyosarcoma (ERMS), its most common subtype, is a malignant soft tissue tumor with morphologic and immunophenotypic features of embryonic skeletal muscle. The histologic findings in ERMS typically include a range of differentiation in rhabdomyoblasts from primitive to terminally differentiated forms, and the latter become more prominent after chemotherapy-induced cytodifferentiation. Several reports have shown therapy-related cytodifferentiation to portend a good prognosis in ERMS. We discuss the case of a pediatric patient who presented with ERMS of the orbit. Although her tumor showed extensive posttreatment cytodifferentiation and several other good prognostic clinicopathologic factors, it pursued an aggressive course, resulting in early metastasis and death. This case represents an unusual course and may be instructive as to the clinicopathologic features impacting prognostication, and ultimately the biology, of this aggressive family of tumors.
format	article
author	Maniraj Jeyaraju Regina Ann Macatangay Ashley Taylor-King Munchel Teresa Anne York Elizabeth A. Montgomery Michael E. Kallen
author_facet	Maniraj Jeyaraju Regina Ann Macatangay Ashley Taylor-King Munchel Teresa Anne York Elizabeth A. Montgomery Michael E. Kallen
author_sort	Maniraj Jeyaraju
title	Embryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical Course
title_short	Embryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical Course
title_full	Embryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical Course
title_fullStr	Embryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical Course
title_full_unstemmed	Embryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical Course
title_sort	embryonal rhabdomyosarcoma with posttherapy cytodifferentiation and aggressive clinical course
publisher	Hindawi Limited
publishDate	2021
url	https://doaj.org/article/d355d9e78bcf4b318dd78fdcb5189f21
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Embryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical Course

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