Primary pleural epithelioid hemangioendothelioma (EHE) – two cases and review of the literature

Lazarus, Angeline A.; G, Fuhrer; Malekiani, Christina; McKay, Sean; Thurber, John

doi:10.1111/j.1752-699x.2010.00221.x

Cited by 25 publications

(33 citation statements)

References 18 publications

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“…Pleural EHE is rare, usually affects males, and is associated with a grim prognosis with disseminated disease [1][2][3]. Pleural involvement in females is even more unusual.…”

Section: Discussionmentioning

confidence: 95%

Pleural Epithelioid Hemangioendothelioma

Bansal

Chawla

Cohen

et al. 2012

Lung

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A 51-year-old female presented to the emergency department complaining of left-sided, nonpleuritic chest pain and a 10-lb. weight loss over the previous 2 months. She was a current smoker with a 20 pack-year history. A PA/lateral chest radiograph and chest computed tomogram (CT) demonstrated a large loculated effusion with pleural thickening (Fig. 1). There were no lung nodules or pathologic mediastinal or hilar lymphadenopathy.A diagnostic thoracentesis was performed, and serosanguineous pleural fluid was obtained. Cytology was negative. For further diagnostic sampling, she underwent a thoracotomy with pleural fluid drainage and pleural biopsy, which demonstrated sheets of atypical epithelioid cells displaying prominent intra-alveolar growth (Fig. 2). The cells stained positive for CD31 and CD34. Stains for pan-cytokeratin, CK5-6, p63, calretinin, S-100, Melan-A, TTF-1, CD68, and EMA were negative. She was given a diagnosis of pleural epithelioid hemangioendothelioma.In the multidisciplinary oncology conference, it was felt she was not a candidate for surgical resection given the extensive nature of the tumor. She was offered chemotherapy with doxorubicin. Less than 4 months after her initial presentation, the patient was admitted to the hospital with a bowel obstruction. Repeat imaging demonstrated further extension of tumor in the left hemithorax with associated volume loss, an enlarging right-sided pleural effusion, evidence of liver and spleen metastasis, and new-onset ascites. Her condition continued to deteriorate with the onset of respiratory failure and she died several days later. DiscussionEpithelioid hemangioendothelioma (EHE) is a malignancy of vascular endothelial origin often involving bone, liver, soft tissues, and lung. Pleural EHE is rare, usually affects males, and is associated with a grim prognosis with disseminated disease [1][2][3]. Pleural involvement in females is even more unusual. In a recent review of 22 patients with pleural EHE, 82% were men and the average survival was only 10 months [1]. Radiographically, pleural EHE is nonspecific and can be easily mistaken for pleural infections or malignant mesothelioma. Chest CT usually demonstrates a pleural effusion with localized or diffuse nodular pleural thickening similar to our case [3][4][5].Diagnosis is confirmed by pathologic examination of specimen obtained by surgical lung biopsy. On immunohistochemistry, EHEs show the typical phenotype of endothelial cells with reactivity to vascular-specific markers, including Factor VIII-related antigen, CD31, CD34, and Fli-1 [5]. The identification of Weibel-Palade bodies and pinocytotic vesicles on electron microscopy can also be useful in the diagnosis of EHE but is

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“…Pleural EHE is rare, usually affects males, and is associated with a grim prognosis with disseminated disease [1][2][3]. Pleural involvement in females is even more unusual.…”

Section: Discussionmentioning

confidence: 95%

Pleural Epithelioid Hemangioendothelioma

Bansal

Chawla

Cohen

et al. 2012

Lung

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“…Most commonly, bevacizumab is used in combination with paclitaxel. Regardless of the chemotherapy bevacizumab is paired with, progression-free survival (PFS) and OS varies from months to years [Grotz et al 2010;Lazarus et al 2011;Gaur et al 2012;Salech et al 2010]. A single-arm, multicenter, phase II trial utilizing single agent bevacizumab for unresectable EHE reported PFS and OS of 39 weeks and 143 weeks, respectively, when bevacizumab was given at 15 mg/kg every 3 weeks [Agulnik et al 2013].…”

Section: Discussionmentioning

confidence: 99%

“…In particular, bevacizumab has been used with carboplatin, paclitaxel, docetaxel and/or etoposide [Grotz et al 2010;O'Grady, 2000;Lazarus et al 2011;Gaur et al 2012]. Most commonly, bevacizumab is used in combination with paclitaxel.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2015

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Hepatic epithelioid hemangioendothelioma (HEHE) is a rare, often misdiagnosed vascular neoplasm with clinical behaviors that range from indolent to highly aggressive. Even when the appropriate diagnosis is achieved, the best treatment for HEHE has not been defined or standardized, further complicating the care of these patients. We present a diagnostically challenging case of HEHE where we utilized capecitabine and bevacizumab as another novel treatment option.

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“…Surgery remains the only curative option while most previously reported cases have failed to show a meaningful clinical response to radiotherapy and/or chemotherapy, suggesting resistance of this tumor to standard anticancer modalities [1,5]. The anti-vascular endothelial growth factor (anti-VEGF) monoclonal antibody bevacizumab has also been used with variable outcomes in a limited number of selected cases [6][7][8][9][10][11]; therefore, the therapeutic benefit of this agent in EH remains controversial. We herein report a case series of four patients with EH, including two cases of localized lung and pleural EH and two cases of liver and bone EH with pulmonary metastases, which were all treated with bevacizumab in the Oncology Unit GPP, "Sotiria" General Hospital, University of Athens, and briefly review the relevant literature (Table 1).…”

Section: Introductionmentioning

confidence: 96%