Pleural Epithelioid Hemangioendothelioma

Abstract

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

Spontaneous bilateral haemothorax as presentation of primary pleural epithelioid haemangioendothelioma

“…We report a case of pleural EHE, an extremely rare location for this tumour , in a patient with the highest age of onset recorded thus far [together with Márquez‐Medina ] (Table ) and debuting as an SBH, the second documented case of pleural EHE with this characteristic. Pleural EHE is a rare tumour that originates in the vascular endothelium with an intermediate degree of malignancy between haemangioma and angiosarcoma.…”

Epithelioid hemangioendothelioma of the sternum

“…EHE in the chest is extremely rare. The literature review (Table ) reveals that the most common sites in the chest are lungs, mediastinum, and pleura . Rare cases occurring in the brachiocephalic vein and the superior vena cava have also been reported .…”

Primary pleural epithelioid hemangioendothelioma

“…11 The main complaints of patients in this analysis are non specific symptoms such as dyspnea, cough, chest pain and weight loss. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Only two cases have been discovered incidentally on routine chest radiography, 5,9 Imaging shows in all cases either effusion or thickening of the pleura associated in some cases to lung nodules. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] As EHE is a very rare cause of pleural disease, more common etiology need to be ruled out firstly.…”

“…[3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Only two cases have been discovered incidentally on routine chest radiography, 5,9 Imaging shows in all cases either effusion or thickening of the pleura associated in some cases to lung nodules. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] As EHE is a very rare cause of pleural disease, more common etiology need to be ruled out firstly. In our case, the pleural effusion was thought to be resulting from metastasis of digestive cancer and so the patient underwent useless investigations leading to a delay in the proper diagnosis.…”

“…1 Primary pleural location is extremely rare with less than 30 cases reported in the literature as analyzed in Table 1. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] The aim of this report is to present the case of a 79-year-old man who was diagnosed with pleural EHE and summarize the limited published data concerning this rare neoplasm in order to improve the diagnosis and its management.…”