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Skeletal Radiology
Published in: Skeletal Radiology 7/2013

01-07-2013 | Scientific Article

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma: characterization of five cases

Authors: M. Fernanda Amary, Paul O’Donnell, Fitim Berisha, Roberto Tirabosco, Tim Briggs, Rob Pollock, Adrienne M. Flanagan

Published in: Skeletal Radiology | Issue 7/2013

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Abstract

Objective

To describe the imaging and histopathology of pseudomyogenic hemangioendothelioma.

Materials and methods

Five cases of pseudomyogenic hemangioendothelioma, which presented over the last 5 years, were retrieved from the files of the Royal National Orthopaedic Hospital. The imaging and histopathology were reviewed in all cases. Magnetic resonance imaging, which was available from all cases, was assessed for the following features: the number of lesions, location in soft tissue (superficial and or deep/subfascial) and bone, and the signal characteristics and morphology of individual lesions. Immunohistochemistry was performed in all cases to characterize the lesions.

Results

Four of the five patients had multiple lesions involving a single limb. Bone was involved in 3 of the 5 individuals. All tumors diffusely expressed ERG and cytokeratins AE1/3, but not MNF116. CD31 was weakly positive in 4 cases. INI-1 expression was retained in all cases. Imaging features included ill-defined, infiltrative lesions in subcutaneous fat with extension to the adjacent skin, poor- to well-defined intramuscular nodules and predominantly intracortical focal bone lesions with rare medullary involvement.

Conclusion

Pseudomyogenic hemangioendothelioma represents a distinct recently characterized tumor type presenting in young adults, with a tendency towards multicentric bone and soft tissue involvement.
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Metadata
Title
Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma: characterization of five cases
Authors
M. Fernanda Amary
Paul O’Donnell
Fitim Berisha
Roberto Tirabosco
Tim Briggs
Rob Pollock
Adrienne M. Flanagan
Publication date
01-07-2013
Publisher
Springer-Verlag
Published in
Skeletal Radiology / Issue 7/2013
Print ISSN: 0364-2348
Electronic ISSN: 1432-2161
DOI
https://doi.org/10.1007/s00256-013-1577-8

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