Pleural Epithelioid Hemangioendothelioma

Abstract

Epithelioid hemangioendothelioma (EHE) is a rare tumor of vascular origin. While it can be found in any tissue, it is most often found in lung and liver and usually has an intermediate behavior. EHEs originating from pleural tissue have been less frequently described than those from other sites. Furthermore, to date, all of the cited pleural EHEs were described as highly aggressive. In the present report, we describe a rare case of pleural EHE extending to lung and bone in a 31-year-old woman. The histological diagnosis was confirmed by both conventional examination and immunohistochemistry. Her disease stabilized during the 4th course of adriamycin (45 mg/m2, day 1-3), dacarbazine (300 mg/m2, day 1-3) and ifosfamide (2,500 mg/m2, day 1-3) with mesna, and she survived for 10 months after the diagnosis.

Clinicopathologic characteristics of primary pleural epithelioid hemangioendothelioma

“…A wide age range at the time of diagnosis was found, and the diagnosis was most often made in the sixth decade of life. Seven patients [3,5,11] acknowledged, and two patients [13,14] denied prior asbestos exposure. Asbestos exposure history was not provided for the remaining 13 cases.…”

“…A complete response resulted that was presumably ongoing 18 months later when the case was reported [8]. The fifth patient received palliative radiation therapy to a painful vertebral metastasis along with systemic doxorubicin [14]. Pulmonary progression was noted after three doses of doxorubicin.…”

“…Seven patients were reported to have developed metastatic disease after the initial diagnosis in a variety of sites including hilar, pulmonary, mediastinal and retroperitoneal lymph nodes, the lung, pleura opposite the primary site, peritoneum, the skin, liver, heart, bone marrow, and abdominal and pelvic serosal surfaces [4,6,8,10,11,14].…”

“…Information available from case reports of primary pleural disease, including pre-existing conditions, disease presentation, therapy, response to therapy, and patient survival, was collected. These characteristics are briefly summarized in Table 1 [3][4][5][6][7][8][9][10][11][12][13][14][15]. Additional details regarding a previously reported case [15] treated at the authors' institution, presented here, were included in the data collection.…”

Beyond a pleural effusion: Primary pleuropulmonary sarcomas and the challenge of diagnosis

“…4 clinical course and a poor prognosis. 5,7 The authors describe two patients with rare causes of pleural effusion, illustrative of the diagnostic difficulty and aggressiveness of these tumors.…”