Why the study?
Due to the low incidence of epithelioid hemangioendothelioma, the most effective treatment is not easily studied and much of the information has been learned through case reports and series.
This case highlights an unusually aggressive recurrence and metastasis of primary soft tissue EHE with atypical pulmonary imaging findings.
Fatal treatment-refractory lymphangitic spread may follow late soft-tissue EHE recurrence; leaves open surveillance and therapy as Level 5 evidence.
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.
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Hickman et al. (2021) studied this question.
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