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Review
. 2008 Dec 31;49(6):1036-40.
doi: 10.3349/ymj.2008.49.6.1036.

Pleural epithelioid hemangioendothelioma

Young Joo Lee  1 , Moon Jae Chung, Ki Cheon Jeong, Chang Hoon Hahn, Ki Pyo Hong, Yee-Jeong Kim, Yong Tai Kim
Affiliations
Review

Pleural epithelioid hemangioendothelioma

Young Joo Lee et al. Yonsei Med J. .

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/m(2), day 1-3), dacarbazine (300 mg/m(2), day 1-3) and ifosfamide (2,500 mg/m(2), day 1-3) with mesna, and she survived for 10 months after the diagnosis.

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Figures

Fig. 1
Fig. 1
On chest CT scan, the largest diameter of the extrapleural tumorous lesion on the apicoposterior segment is (A) 15 mm at initial diagnosis, (B) 30.7 mm after the 3rd adriamycin (45 mg/m2, day 1, every 3 wks), and (C) 30.8 mm after the 2nd MAID regimen, administered every 4 wks as an injection 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.
Fig. 2
Fig. 2
A bone scan at diagnosis shows a focally increased uptake (A) at the level of the 5th thoracic spine suggesting bone metastasis and (B) a linear uptake at the 7th left anterior rib that could not be ruled out as a potential metastasis.
Fig. 3
Fig. 3
Photograph of a specimen of thoracoscopic wedge resection of the right lower lobe shows multiple scattered and variably sized subpleural whitish-tan plaques.
Fig. 4
Fig. 4
Histological photographs of a lung specimen show (A) pleura-based intraparenchymal tumor growth (H & E ×40) and (B) tumor cells surrounded by a hyalinized stroma with intracytoplasimic vacuolization (H & E ×400).
Fig. 5
Fig. 5
(A) Immunohistostaining shows diffuse negative reactivity with cytokeratin (×200). (B) Immunohistostaining shows diffuse positive reactivity with factor VIII (×200).
See this image and copyright information in PMC

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