Document Detail

Ewing's Sarcoma/Primitive Neuroectodermal Tumor of the Proximal Humeral Epiphysis.
MedLine Citation:
PMID:  23276342     Owner:  NLM     Status:  In-Data-Review    
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.
Parisa Morris; Paul S Dickman; Matthew J Seidel
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Orthopedics     Volume:  36     ISSN:  1938-2367     ISO Abbreviation:  Orthopedics     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-01-01     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7806107     Medline TA:  Orthopedics     Country:  United States    
Other Details:
Languages:  eng     Pagination:  e113-6     Citation Subset:  IM    
Copyright Information:
Copyright 2013, SLACK Incorporated.
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