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Epithelioid Hemangioma of Bone and Soft Tissue: A Reappraisal of a Controversial Entity

  • Clinical Research
  • Published:
Clinical Orthopaedics and Related Research®

Abstract

Background

The controversy surrounding diagnosis of an epithelioid hemangioma (EH), particularly when arising in skeletal locations, stems not only from its overlapping features with other malignant vascular neoplasms, but also from its somewhat aggressive clinical characteristics, including multifocal presentation and occasional lymph node involvement. Specifically, the distinction from epithelioid hemangioendothelioma (EHE) has been controversial. The recurrent t(1;3)(p36;q25) chromosomal translocation, resulting in WWTR1-CAMTA1 fusion, recently identified in EHE of various anatomic sites, but not in EH or other epithelioid vascular neoplasms, suggests distinct pathogeneses.

Question/purposes

We investigated the clinicopathologic and radiologic characteristics of bone and soft tissue EHs in patients treated at our institution with available tissue for molecular testing.

Patients and Methods

Seventeen patients were selected after confirming the pathologic diagnosis and fluorescence in situ hybridization analysis for the WWTR1 and/or CAMTA1 rearrangements. Four patients had multifocal presentation. Most patients with EH of bone were treated by intralesional curettage. None of the patients died of disease and only four patients had a local recurrence.

Results

Our results, using molecular testing to support the pathologic diagnosis of EH, reinforce prior data that EH is a benign lesion characterized by an indolent clinical course with an occasional multifocal presentation and rare metastatic potential to locoregional lymph nodes.

Conclusion

These findings highlight the importance of distinguishing EH from other malignant epithelioid vascular tumors as a result of differences in their management and clinical outcome.

Level of Evidence

Level IV, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.

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Acknowledgments

We thank Jesse Galle for assistance with clinical followup and Milagros Soto and Lionel Santibañez for editorial assistance.

Author information

Authors and Affiliations

  1. Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    Costantino Errani MD & John H. Healey MD

  2. Department of Pathology, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY, 10065, USA

    Costantino Errani MD, Lei Zhang MD & Cristina R. Antonescu MD

  3. Department of Radiology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    David M. Panicek MD

Authors
  1. Costantino Errani MD
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  2. Lei Zhang MD
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  3. David M. Panicek MD
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  4. John H. Healey MD
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  5. Cristina R. Antonescu MD
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Corresponding author

Correspondence to Cristina R. Antonescu MD.

Additional information

One or more of the authors received funding from PO1 CA047179-15A2 (CRA), P50 CA 140146-01 (CRA), the Maynard Orthopaedic Research Fund, and Associazione per la Ricerca e la Cura dei Tumori dell’Apparato Locomotore (CE).

All ICMJE Conflict of Interest Forms for authors and Clinical Orthopaedics and Related Research editors and board members are on file with the publication and can be viewed on request.

Each author certifies that his or her institution approved the human protocol for this investigation, that all investigations were conducted in conformity with ethical principles of research, and that informed consent for participation in the study was obtained.

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Errani, C., Zhang, L., Panicek, D.M. et al. Epithelioid Hemangioma of Bone and Soft Tissue: A Reappraisal of a Controversial Entity . Clin Orthop Relat Res 470, 1498–1506 (2012). https://doi.org/10.1007/s11999-011-2070-0

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  • DOI: https://doi.org/10.1007/s11999-011-2070-0

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