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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| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2021-01-01
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| Series: | Case Reports in Pathology |
| Online Access: | http://dx.doi.org/10.1155/2021/1800854 |
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| 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 |
| collection | DOAJ |
| 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 |
| id | doaj-art-d355d9e78bcf4b318dd78fdcb5189f21 |
| institution | OA Journals |
| issn | 2090-679X |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pathology |
| spelling | doaj-art-d355d9e78bcf4b318dd78fdcb5189f212025-08-20T02:19:51ZengWileyCase Reports in Pathology2090-679X2021-01-01202110.1155/2021/1800854Embryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical CourseManiraj Jeyaraju0Regina Ann Macatangay1Ashley Taylor-King Munchel2Teresa Anne York3Elizabeth A. Montgomery4Michael E. Kallen5University of Maryland School of MedicineDivision of Pediatric Hematology/OncologyDivision of Pediatric Hematology/OncologyDivision of Pediatric Hematology/OncologyDepartment of Pathology and Laboratory MedicineDepartment of PathologyRhabdomyosarcoma 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.http://dx.doi.org/10.1155/2021/1800854 |
| spellingShingle | 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 Case Reports in Pathology |
| title | 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_short | Embryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical Course |
| title_sort | embryonal rhabdomyosarcoma with posttherapy cytodifferentiation and aggressive clinical course |
| url | http://dx.doi.org/10.1155/2021/1800854 |
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