P1.09-38 Pulmonary Epithelioid Hemangioendothelioma (EHE) with Von Recklinghausen Disease

Abstract

Malignant thoracic epithelioid tumors are an uncommon. The heterogenous group of tumors that include the low to intermediate grade epithelioid hemangioendothelioma(EHE) and epithelioid angiosarcoma under examination of the morphologic and immunohistochemical features. We here reported a rare case of multiple pulmonary epithelioid hemangioendothelioma with von Recklinghausen disease. Case A 34–year–old male was admitted to our hospital with an abnormal shadow in the bilateral lung field. He has von Recklinghausen disease, and non-smoker. The laboratory data and physical examination are normal. A chest computed tomography scan showed multiple nodal lesions of 2 mm to 12 mm in diameter in the bilateral lung fields. Thoracoscopic partial resection of the right middle lobe was performed. The tumor was well-circumscribed lesion. In the pathological findings, sections showed a proliferation of epithelioid tumor cells having oval-shaped and vesicular nuclei, prominent nucleoli and pale eosinophilic cytoplasm arranged in small nested or cord patterns. Intracytoplasmic vacuoles some of which contain erythrocytes were recognized. Mitotic figures were rarely seen. In the immunohistochemical examination, the tumor cells were positive for CD31 and CD34, but negative for D2-40, CAM5.2, Ber-EP4, alpha-SMA, desmin, S-100 protein, NSE, TTF-1, calretinin, claudin-4, CD36 and c-kit. RT-PCR analysis showed CAMTA1-WWTR1 fusions was negative. Those features were consistent with pulmonary epithelioid hemangioendothelioma. Pulmonary epithelioid hemangioendothelioma(EHE) is rare tumor and there is no report in the literature of EHE with von Recklinghausen disease. The behavior of this neoplasm is uncertain, so the methods of diagnosis and treatment will demand to do careful observation and further examination.