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CC BY 4.0 · Indian J Med Paediatr Oncol 2025; 46(02): 219-225
DOI: 10.1055/s-0043-1774775
Case Report with Review of Literature

A Case Report and Review of Literature: Epithelioid Hemangioendothelioma—An Uncommon Challenging Case

Authors

  • Sweta Soni

    1   Department of Radiation Oncology, All India Institute of Medical Sciences, Rajkot, Rajkot, Gujarat, India

  • Bharti Devnani

    2   Department of Radiation Oncology, All India Institute of Medical Sciences, Jodhpur, Jodhpur, Rajasthan, India

  • Poonam Elhence

    3   Department of Pathology, All India Institute of Medical Sciences, Jodhpur, Jodhpur, Rajasthan, India

  • Kapil Soni

    4   Department of ENT, All India Institute of Medical Sciences, Jodhpur, Jodhpur, Rajasthan, India

  • Deepak Vedant

    3   Department of Pathology, All India Institute of Medical Sciences, Jodhpur, Jodhpur, Rajasthan, India

  • Palak Gupta

    4   Department of ENT, All India Institute of Medical Sciences, Jodhpur, Jodhpur, Rajasthan, India

  • Puneet Pareek

    2   Department of Radiation Oncology, All India Institute of Medical Sciences, Jodhpur, Jodhpur, Rajasthan, India

  • Rakesh Kumar Vyas

    2   Department of Radiation Oncology, All India Institute of Medical Sciences, Jodhpur, Jodhpur, Rajasthan, India

Funding None.
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Abstract

Introduction Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor of soft tissue and bone that may uncommonly occur in the liver, lung, and head and neck region. EHEs have a higher predilection for recurrence at the local site as well as distant metastasis. Surgical excision is important and is the treatment in localized diseases. A decision to give adjuvant radiotherapy should be subjective and may differ on case-to-case basis. Limited studies are available exploring the role of targeted or systemic therapy.

Case Presentation A 56-year-old lady represented with right-sided submandibular region EHE with bilateral lung metastasis. The patient underwent surgery and radiotherapy followed by targeted therapy tab pazopanib for systemic control. At 2 years of follow-up, positron emission tomography-computed tomography showed local regional control and stable systemic diseases.

Conclusion The uncertainty in choosing the most suitable treatment of EHE patients is high and may result in dissatisfactory outcomes among several patients. The present case study identified a treatment dilemma making management more challenging for rare EHE with mandibular involvement.

Keywords

hemangioendothelioma - surgery - radiotherapy - pazopanib - outcomes - case report

Ethics Approval and Consent to Participate

For submission of a case report, clearance from the Institute Ethics Committee is waived at All India Institute of Medical Sciences, Jodhpur. It is notable that the patient was not subjected to any experimental investigation or treatment at any point of time.


Patient's Consent

Written informed consent was obtained from the patient's guardian for publication of this case report and accompanying images.


Data Availability Statement

All data generated or analyzed during this study are included in this published article.


Authors' Contributions

S.S., B.D.: Conception and design of this study, acquisition of data, analysis and interpretation of data, and drafting. All authors read and approved the final manuscript




Publication History

Article published online:
22 September 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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