Yonsei Med J.  2008 Dec;49(6):1036-1040. 10.3349/ymj.2008.49.6.1036.

Pleural Epithelioid Hemangioendothelioma

• Lee YJ
• Chung MJ
• Jeong KC
• Hahn CH
• Hong KP
• Kim YJ
• Kim YT

• KMID: 1782955
• DOI: http://doi.org/10.3349/ymj.2008.49.6.1036

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 (45mg/m2, day 1-3), dacarbazine (300mg/m2, day 1-3) and ifosfamide (2,500mg/m2, day 1-3) with mesna, and she survived for 10 months after the diagnosis.

Keyword

Pleura; epithelioid hemangioendothelioma; immunohistochemistry

MeSH Terms

Adult
Antineoplastic Combined Chemotherapy Protocols/therapeutic use
Diagnosis, Differential
Factor VIII/metabolism
Female
Hemangioendothelioma, Epithelioid/*diagnosis/drug therapy/metabolism
Humans
Immunohistochemistry
Keratins/metabolism
Lung Neoplasms/diagnosis
Pleural Neoplasms/*diagnosis/drug therapy/metabolism
Vimentin/metabolism

Figure

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  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.

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  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.

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  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.

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  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).

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  Fig. 5 (A) Immunohistostaining shows diffuse negative reactivity with cytokeratin (×200). (B) Immunohistostaining shows diffuse positive reactivity with factor VIII (×200).

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