Pulmonary epithelioid hemangioendothelioma: Report of three cases

Abstract

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of low-grade malignancy, the prognosis of which remains unpredictable. Surgical and/or chemotherapeutic options have to be evaluated depending on intrathoracic tumor spread and systemic metastases. Three cases of PEH with both clinical and pathological findings are herein summarized and the relevant current literature discussed.

Pulmonary epithelioid hemangioendothelioma

“…The disease in the present two cases was discovered incidentally from chest radiography and CT. Roentgenologicals fi ndings such as multiple bilateral nodules have been noted in previous reports of PEH but were often mistaken for metastases or granulomatous disease. 6 Initially, we also suspected these lesions to be multiple lung metastases from an unknown primary. 18 F-FDG-PET/CT was recently reported by Watanabe et al as a useful diagnostic tool for indicating the suitability of resection for PEH.…”

Multiple organ metastases of pulmonary epithelioid haemangioendothelioma and a review of the literature

“…The rarity of this condition, the lack of clear standards for treatment, and the partial-to-complete spontaneous regression of EHE seen in some patients up to 15 years from initial detection makes it difficult to decide on the most appropriate treatment. This is a rare disease, with approximately 100 cases described in the English literature [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] (Table 1) and approximately 10 cases reported in China [22,23].…”

Combination capecitabine and bevacizumab in the treatment of metastatic hepatic epithelioid hemangioendothelioma

“…Other therapies reported include combination or single-agent regimens of the following: interferon-α, sunitinib, sorafenib, thalidomide, lenalidomide, vinorelbine, dacarbazine, cisplatin, ifosfamide, etoposide, docetaxel, paclitaxel, nab-paclitaxel, liposomal doxorubicin, gemcitabine, pamidronate, cyclophosphamide and nonsteroidal anti-inflammatory drugs [Cardinal et al 2009;Gaur et al 2012;Sangro et al 2012;Raphael et al 2010;Sumrall et al 2010;Schilling et al 2009;Sharif et al 2004;Marquez-Medina et al 2004;Belmont et al 2008;Cronin and Arenberg, 2004;Schattenberg et al 2008;Grenader et al 2011;Kelly and O'Neil, 2005;Pintoffl et al 2009;Coppo et al 2004;Lakkis et al 2013](see Table 1). …”