Hickman Ashley D, Bezerra Evandro D, Roden Anja C, Houdek Matthew T, Barlow Jonathan D, Robinson Steven I, Wahner Hendrickson Andrea E
Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA.
Department of Oncology, Mayo Clinic, Rochester, MN, USA.
Rare Tumors. 2021 Apr 20;13:20363613211010858. doi: 10.1177/20363613211010858. eCollection 2021.
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.
上皮样血管内皮瘤（EHE）是一种罕见的血管肿瘤，通常起源于肝脏、肺或骨骼。由于该疾病发病率低，最有效的治疗方法不易研究，目前已知的许多关于EHE的信息都是通过病例报告和病例系列了解到的。在本病例中，我们将呈现一种罕见的原发性软组织EHE，伴有局部复发、骨转移以及淋巴管扩散至肺部导致呼吸衰竭。胸部影像学表现对于EHE来说不典型，出现了小叶间隔增厚和肺门淋巴结肿大。尽管采取了积极的多模式治疗，呼吸衰竭仍在进展。本病例突出了原发性软组织EHE异常侵袭性的复发和转移，以及非典型的肺部影像学表现。
