An unusual case of aggressive malignant spread of epithelioid hemangioendothelioma

Ashley D. Hickman, Evandro D. Bezerra, Anja C. Roden, Matthew T. Houdek, Jonathan D. Barlow, Steven I. Robinson, Andrea E. Wahner Hendrickson

Research output: Contribution to journalArticlepeer-review


Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm which typically originates from liver, lung, or bone. Due to the low incidence of disease, the most effective treatment is not easily studied and much of the information known about EHE has been learned through case reports and case series. In this case, we will present an uncommon form of primary soft tissue EHE with local recurrence, bone metastasis, and lymphangitic spread to the lungs leading to respiratory failure. Imaging of the chest was atypical for EHE with intraseptal thickening and hilar lymphadenopathy. Respiratory failure was progressive despite aggressive multimodal treatment. This case highlights an unusually aggressive recurrence and metastasis of primary soft tissue EHE with atypical pulmonary imaging findings.

Original languageEnglish (US)
JournalRare Tumors
StatePublished - 2021


  • EHE
  • Sarcoma
  • epithelioid hemangioendothelioma
  • lymphangitic spread
  • pulmonary epithelioid hemangioendothelioma
  • soft-tissue EHE
  • vascular neoplasm

ASJC Scopus subject areas

  • Histology
  • Oncology


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