| Ewing's Sarcoma/Primitive Neuroectodermal Tumor of the Proximal Humeral Epiphysis. | |
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MedLine Citation:
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PMID: 23276342 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of bone is a rare childhood tumor most commonly located in the metadiaphysis. In skeletally immature patients, lesions of the epiphysis are rarely malignant, with the most common diagnosis being chondroblastoma. This article presents a case of ES/PNET of the proximal humeral epiphysis in a 12-year-old boy. To the authors' knowledge, this is the first reported case of epiphyseal ES/PNET confirmed with molecular testing. Radiographs of the patient's painful shoulder showed a well-defined lytic lesion within the humeral epiphysis. Magnetic resonance imaging suggested a chondroid tumor with surrounding edema. Based on the imaging characteristics, the patient's age, and the lesion's location, a preliminary diagnosis of chondroblastoma was made. A trochar biopsy of the lesion demonstrated a small, round, blue cell tumor on frozen section. Subsequently, immunohistochemical staining was uniformly positive in a membrane pattern for CD99, and molecular diagnostic testing demonstrated a EWSR1/FLI1 fusion transcript, confirming the pathologic diagnosis of ES/PNET. Although metadiaphyseal locations for ES/PNET are most common, this case adds to previously reported cases of epiphyseal ES/PNET, suggesting that the diagnosis be considered for pediatric epiphyseal tumors. This case also demonstrates why following rigorous oncologic treatment algorithms by obtaining a limited trochar biopsy, even in the case of a confident radiographic diagnosis, is critically important; the biopsy results can lead to a major change in treatment and avoid contamination of a larger area of soft tissue and bone. |
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Authors:
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Parisa Morris; Paul S Dickman; Matthew J Seidel |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Orthopedics Volume: 36 ISSN: 1938-2367 ISO Abbreviation: Orthopedics Publication Date: 2013 Jan |
Date Detail:
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Created Date: 2013-01-01 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7806107 Medline TA: Orthopedics Country: United States |
Other Details:
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Languages: eng Pagination: e113-6 Citation Subset: IM |
Copyright Information:
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Copyright 2013, SLACK Incorporated. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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