Three cases of Pulmonary Epithelioid Hemangioendothelioma

폐 유상피 혈관내피종 3예

A pulmonary epithelioid hemangioendothelioma (PEH) is a rare tumor with a vascular origin. A PEH can arise in many organ systems, such as the lung, liver, bone and soft tissues. It is a borderline malignancy but the clinical course is usually benign. In this report, we describe three cases of PEH. Case 1, a 61-year-old man, had nonspecific chest discomfort and the chest X-ray showed a solitary lung nodule. This nodule was diagnosed by an open lung biopsy. The pathologic findings including abundant necrosis, mitosis and hyperchromatic and pleomorphic nuclei, indicated a malignancy. The electron microscopic study showed Weibel-Palade bodies and the immunohistochemical stain for CD31 showed a positive reaction in the tumor cells, and linear staining along the vascular lumina. Case 2, a 34-year-old man, was admitted for an evaluation of multiple small nodules, incidentally detected a screening chest X-ray. The nodules were diagnosed by a immunohistochemical stain for the factor VIII-related antigen. Case 3, a 34-year-old woman, complained of left pleuritic chest pain. A simple chest film and the chest CT scan revealed multiple bilateral nodules and a left pleural effusion. An immunohistochemical stain for the factor VIII-related antigen was used to diagnose the nodules. Forth-one months after the diagnosis, she died of pulmonary insufficiency.

국내에서 폐 유상피 혈관내피종이 악성으로 진행하여 사망한 예는 1예만 보고되어 있다. 단일 폐결절이 있는 환자에서 개흉 폐절제술후 병리조직학적으로 종양세포의 유사분열과 괴사를 동반하여 악성 유상피 혈관내피종으로 진단하고 예후가 불량할 것으로 예상되는 1예와 단순 흉부 촬영상 양측 다발성 폐결절이 있는 무증상 환자에서 비디오 흉강경 폐생검으로 폐 유상피 혈관내피종을 진단 후 조직학적 및 임상적으로 악성 소견 없이 경과를 관찰 중인 1예 및 양측 폐의 다발성 결절성 병변과 늑막액을 동반하여 개흉 폐생검으로 폐 유상피 혈관내피종을 진단 후 호흡부전으로 사망한 1예의 폐 유상피 혈관내피종 3예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.