Disorders of the Mediastinum: Pleura and Chest Wall |

A Rare Primary Pleural Epitheliod Hemangioendothelioma in a Young Male FREE TO VIEW

Arjun Khanna, DM
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All India Institute of Medical Sciences, Delhi, India

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

Chest. 2016;149(4_S):A224. doi:10.1016/j.chest.2016.02.232
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SESSION TITLE: Pleura and Chest Wall

SESSION TYPE: Case Report Poster

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

INTRODUCTION: Epitheloid hemangioendothelioma is a rare form of pleural tumour which tends to follow an intermediate course between hemangioma and conventional angiosarcoma. Here, we describe this tumor in a young male, who was initially diagnosed as solitary fibrous tumor on the basis of radiology, but the final diagnosis was established on histopathology and immunohistochemistry (IHC).

CASE PRESENTATION: A 22 year old non-smoker male was admitted with the complaints of left sided chest pain since two and a half months. Chest radiograph revealed a homogenous pleural based opacity with an incomplete border sign on the left side. Contrast enhanced CT showed a lobulated mass lesion of size 3.4*5.6 cm with contrast enhancement and associated minimal pleural effusion. There was associated scalloping of the 6th left rib. FDG-PET showed no significant FDG uptake. A video-assisted thoracoscopic excisional biopsy of the tumor was done. Histo-pathological examination revealed spindle cells and epithelioid cells in some areas and numerous blood vessels were being formed by the tumor. In the epithelioid part of tumor intracytoplasmic red cells were seen. No significant mitotic figures or area of necrosis was seen. Staining of the tumoral tissue was positive for the anti-vimentin. CD31 was focally positive and CD 34 was strongly positive. CD 99 and bcl-2 were negative ruling out the possibility of a solitary fibrous tumor. Staining with the anti-cytokeratin antibodies was negative, which excludes the diagnosis of a metastatic carcinoma or malignant mesothelioma. Final diagnosis of epitheloid hemangioendothelioma was made. The patient is being followed up closely.

DISCUSSION: Epitheloid hemangioendothelioma is a rare form of pleural tumour which tends to follow an intermediate course between hemangioma and conventional angiosarcoma. This tumour is characterized by an epithelioid or histiocytoid endothelial cell proliferation. It can be found in any organ but is most often found in the liver and lung. EHE mainly affects females during the sixth decade1. Usually, its evolution is slow, but aggressive forms have been described. The diagnosis is suspected on histological features and confirmed by immunohistochemistry (IHC) 2. The closest differential is the primary pleural angiosarcoma.

CONCLUSIONS: Rare pleural tumors such as primary pleural epitheliod hemangioendothelioma are difficult to diagnose and require close liaison between the treating tem, radiologist and pathologist. The diagnosis is best established by IHC staining and close follow up is warranted as clear treatment guidelines are not available.

Reference #1: Del Frate C, Mortele K, Zanardi R, et al. Pseudomesotheliomatous angiosarcoma of the chest wall and pleura. J Thorac Imaging 2003;18:200-3.

Reference #2: Roh MS, Seo JY, Hong SH. Epithelioid angiosarcoma of the pleura: a case report. J Korean Med Sci 2001;16:792-5.

DISCLOSURE: The following authors have nothing to disclose: Arjun Khanna

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