Author of

• 25929

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  P1.09 - Pathology (Not CME Accredited Session) (ID 941)

  • Event: WCLC 2018
  • Type: Poster Viewing in the Exhibit Hall
  • Track:
  • Presentations: 1
  • Moderators:
  • Coordinates: 9/24/2018, 16:45 - 18:00, Exhibit Hall

  • 26542

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    P1.09-38 - Pulmonary Epithelioid Hemangioendothelioma (EHE) with Von Recklinghausen Disease (ID 11764)

    16:45 - 18:00  |  Author(s): Motohisa Kuwahara
    • Abstract

    Background
    

    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.

    a9ded1e5ce5d75814730bb4caaf49419 Method
    

    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.

    4c3880bb027f159e801041b1021e88e8 Result
    

    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.

    8eea62084ca7e541d918e823422bd82e Conclusion
    

    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.

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