Lung Pathology: Chest Tumors |

Pleuropulmonary Epithelioid Hemangioendothelioma: A Case Report and Review of the Literature FREE TO VIEW

Yueqin Hao, PhD; Hua-ping Tang, BA; Jian-you Chen, MA
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Qingdao Municipal Hospital, Qingdao, China

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A337. doi:10.1016/j.chest.2016.02.351
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SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Pleuropulmonary epithelioid hemangioendothelioma is a rare neoplasm tumor of vascular origin with an epithelioid appearance. It is usually associated with an aggressive clinical course leading to early death. With the prognosis being unpredictable, there is still no standard treatment of choice.

CASE PRESENTATION: A 73 year old male presented with right chest pain, and his chest CT scan showed an irregularly mass in the superior lobe of right lung and multiple nodular thickening of the right pleura. Percutaneous lung biopsy guided by CT showed epithelioid hemangioendothelioma. Immunohistochemical stains was positive for CD31, F8, and vimentin and was negative for Calretinin. Following tissue diagnosis, the chemotherapy regimen of pemetrexed combined with vascular endostatin Endostar was administered, but the regiment didn’t benefit the patient. His condition continued to deteriorate and only survived for four months following diagnosis.

DISCUSSION: We searched databases Pubmed, Elsevier, Wanfang, Chinese National Knowledge infrastructure (CNKI) and Chinese sci-tech periodical full-text database using the keyword “pleuropulmonary epithelioid hemangioendothelioma” and only 5 5cases of pleuropulmonary epithelioid hemangioendothelioma were reported. Among all 6 cases including our case, the mean survival time was 6 months after diagnosis. Review of the relevant literature, we found that the prognosis of pleuropulmonary epithelioid hemangioendothelioma was worse compared to the pure lung or pleural epithelioid hemangioendothelioma.

CONCLUSIONS: Pleuropulmonary epithelioid hemangioendothelioma is very rare and its clinical manifestations was nonspecific. Computered tomography usually revealed pleural nodular thickening and lung mass. The pleuropulmonary EPE usually is associated with an aggressive clinical course leading to early death. Definitive diagnosis requires positive immunohistochemical staining for endothelial markers. There are currently no known effective or recommended chemotherapy regimens for pleuropulmonary EHE. Anti-angiogenic drugs also didnit exhibit obvious response. The present study was limited by a small sample size because pleuropulmonary EHE is rarely encountered, so we We need to accumulate more cases to draw definitive conclusion in the future.

Reference #1: Gill R, O’Donnell RJ, Horvai A: Utility of immunohistochemistry for endothelial markers in distinguishing epithelioid hemangioendothelioma from carcinoma metastatic to bone. Arch Pathol Lab Med 2009, 133:967-972.

Reference #2: Zuochun Y, Yuan H, Jing F, et al. A case report of pleuropulmonary epithelioid hemangioendothelioma [J]. Chin J Tuberc Respir Dis, 2006,29(9):643-644.

Reference #3: Enguo C, Wujun H, Chen Z, A case report of pleuropulmonary epithelioid hemangioendothelioma [J]. Chin J Oncol, 2003,25(5):440.

DISCLOSURE: The following authors have nothing to disclose: Yueqin Hao, Hua-ping Tang, Jian-you Chen

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