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Original Report |
1
Department of Radiology, Box 3808, Duke University Medical Center, Durham, NC
27710.
2
Department of Pathology, Duke University Medical Center, Durham, NC
27710.
Received April 6, 2000;
accepted after revision May 17, 2000.
Address correspondence to H. P. McAdams.
Abstract
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CONCLUSION. Pleural epithelioid hemangioendothelioma is an uncommon malignancy that typically affects older men, who present with chest pain and dyspnea. This lesion manifests on chest radiographs and CT scans with unilateral pleural fluid and nodular pleural thickening and appears similar to diffuse pleural carcinomatosis or mesothelioma.
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CT scans were obtained in all patients. In three patients, scans were obtained on a helical scanner (CT/i; General Electric Medical Systems, Milwaukee, WI) with 10-mm collimation, a pitch ratio of 1, a 10-mm reconstruction interval, and standard- and lung-reconstruction algorithms. All images were obtained with standard mediastinal and lung window settings. IV iodinated contrast material was not administered. In one patient, CT was performed to exclude a diagnosis of pulmonary embolism on a helical scanner (QX/i; General Electric Medical Systems) using 2.5-mm collimation, 15-mm per rotation table speed, a 1-mm reconstruction interval, and standard- and lung-reconstruction algorithms. IV contrast material was administered at a rate of 4 mL/sec for a total volume of 150 mL of 300 mg I/mL contrast material. Every second image was obtained in standard lung and mediastinal windows. CT images were reviewed for the same findings as those on the chest radiographs by two thoracic radiologists; findings were recorded by consensus. Additionally, CT scans were evaluated for findings of chest wall or diaphragm invasion.
MR imaging was performed in one patient on a scanner equipped with a 1.5-T magnet (General Electric Medical Systems). T1-weighted images (TR/TE, 550/20) were obtained in the axial, sagittal, and coronal planes, and fast spin-echo images (3157/104) were obtained in the sagittal plane. Gadolinium was not administered. MR images were reviewed for the same findings as the chest CT scans by two thoracic radiologists; findings were recorded by consensus.
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Three of the four patients died with progressive disease. Two died of progressive respiratory compromise; postmortem examination (limited to the thorax) in one of the two patients confirmed extensive pleural involvement and metastases to mediastinal lymph nodes. The third patient died of progressive liver failure; postmortem examination in this patient confirmed extensive pleural involvement (Fig. 1A,1B,1C,1D,1E) and metastases to the lung, mediastinal lymph nodes, liver, and retroperitoneal lymph nodes. The average time from onset of symptoms to death was 10 months (range, 1-19 months). The fourth patient was lost to follow-up.
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Histopathologic Findings
Histopathologic examination of biopsy or autopsy material in all patients
showed short strands or nests of epithelioid tumor cells, many with
intracytoplasmic lumina, in a myxoid and hyaline stromal matrix. Some of the
intracytoplasmic lumina featured erythrocytes or fragments thereof.
Immunohistochemistry was performed in all patients and results for all tumors
were negative for cytokeratin, and all tumors displayed immunoreactivity to
some or all of the vascular-endothelial markers CD31, CD34, and factor VIII
(Fig.
1A,1B,1C,1D,1E).
Electron microscopy was performed in two patients and confirmed characteristic
features of endothelial differentiation, including pinocytotic vesicles and
Weibel-Palade bodies (Fig.
1A,1B,1C,1D,1E).
Chest Radiographs
On chest radiographs, all affected patients had right-sided pleural
effusions that ranged from small to moderate in size (Fig.
2A,2B,2C).
Pleural thickening or mass was seen radiographically in only one patient, who
had extensive pleural disease extending over the apex of the right hemithorax.
All patients had some degree of volume loss in the right hemithorax. No
patient had definite findings of mediastinal lymphadenopathy, rib or vertebral
body destruction, or pulmonary nodules on chest radiographs.
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Chest CT
Chest CT confirmed small to moderate-sized pleural effusions in all
patients (Figs.
2A,2B,2C,3,4A,4B).
In three, the effusion appeared partially loculated (Figs.
2A,2B,2C
and
4A,4B).
Smooth and nodular pleural thickening was seen in all patients (Figs.
2A,2B,2C,3,4A,4B).
In two, pleural thickening was confined to the lower aspect of the right
hemithorax (Fig.
2A,2B,2C),
and in two it was diffuse (Fig.
4A,4B).
One patient had CT findings suggestive of invasion of the diaphragm
(Fig. 3). No patient had
evidence of chest wall invasion on CT. Findings of extensive mediastinal
invasion by the pleural tumor were seen in two patients (Fig.
4A,4B).
Diffuse mediastinal lymphadenopathy was seen in one patient (Fig.
4A,4B).
This patient also had thickened interlobular septa suggesting lymphangitic
spread of tumor. At presentation, two patients had multiple pulmonary nodules
suggestive of metastatic disease (Fig.
2A,2B,2C).
These nodules ranged in size from 3 to 12 mm in diameter and were enlarged on
subsequent examinations.
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MR Imaging
MR imaging was performed in one patient and showed circumferential pleural
thickening, pleural nodularity, and loculated pleural fluid (Fig.
1A,1B,1C,1D,1E).
Additionally, findings indicated invasion of the right diaphragm, right side
of the pericardium, and mediastinum. No signs of chest wall invasion were
seen.
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In our series, the radiologic, surgical, autopsy, and histopathologic data strongly support a pleural origin for the tumors. According to the literature and our experience, pleural epithelioid hemangioendothelioma is a tumor that most commonly affects older men who are symptomatic at presentation [8, 9]. All patients in our series were symptomatic, with common complaints of chest pain and dyspnea. In contradistinction, pulmonary epithelioid hemangioendothelioma is a tumor that most commonly affects young to middle-aged women who are usually asymptomatic at presentation [3,4,5].
The prognosis of patients with epithelioid hemangioendothelioma is quite variable [11], depends on the site of origin, and cannot be predicted solely on histopathologic or clinical grounds. Pulmonary epithelioid hemangioendothelioma has a relatively good prognosis with a long life expectancy despite often extensive parenchymal involvement [3, 5]. The prognosis of pleural epithelioid hemangioendothelioma is much less optimistic. Three of our four patients died from progressive malignancy, with an average survival of 10 months. According to the literature, poor prognostic indicators in patients with epithelioid hemangioendothelioma include symptoms at presentation [4, 5], lymphangitic spread of tumor [5], he patic metastases, and peripheral lymphadenopathy [3,4,5].
Curative surgical resection is considered to be the treatment of choice when possible. In our patients, however, the disease was too advanced at presentation for curative surgery to be considered. Three of the four patients underwent pleural stripping and decortication procedures, partly for diagnostic purposes and partly for palliation. Various chemotherapeutic agents and radiation therapy regimens have been used in affected patients, without demonstrable therapeutic benefit [3, 12].
Grossly, pleural epithelioid hemangioendothelioma forms a rind of tumor around the lung that may extend into the lung parenchyma along fissures and interlobular septa. Microscopically, the tumor is characterized by tubulopapillary growth of short strands and solid nests of tumor cells in a hyaline or myxoid stroma. The epithelioid morphology and presence of intravascular lumina may be misinterpreted as carcinoma with mucin vacuoles. The characteristic immunophenotype is negative for epithelial, muscular, and neural markers, and positive for vascular markers such as factor VIII, CD31, CD34, von Willebrand's factor, and Ulex europaeus antigen (UEA-1). Ultra-structural endothelial characteristics can be confirmed by electron microscopy, which shows the presence of prominent basal lamina, pinocytotic vesicles, and Weibel-Palade bodies.
On chest radiographs, pleural epithelioid hemangioendothelioma typically manifests as pleural effusion and smooth or nodular pleural thickening. Volume loss in the affected hemithorax is also a common feature. These findings closely resemble those of diffuse pleural carcinomatosis or mesothelioma [3, 8, 9]. On CT, nodular pleural masses and loculated effusions are the most common findings. In half of our patients, pleural disease predominated in the inferior aspect of the hemithorax; in the remaining patients, the hemithorax was diffusely involved. Interestingly, all the lesions occurred in the right hemithorax.
In our series, CT or MR findings of mediastinal lymphadenopathy or mediastinal, chest wall, or diaphragm invasion were uncommon at presentation. At autopsy, however, metastases to mediastinal lymph nodes were found in two of the four patients, and one patient was found to have hepatic and retroperitoneal metastases. Multiple small pulmonary nodules, seen only on CT, were noted at presentation in two of the four patients. Autopsy in one of these patients confirmed pulmonary metastases. The nodules wee presumed to be metastases in the other patient because they enlarged on subsequent examinations.
In summary, epithelioid hemangioendothelioma of the pleura differs from its pulmonary counterpart in many respects: it typically affects men; affected patients are usually symptomatic at presentation; and it often pursues an aggressive clinical course, becomes widely metastatic and has a poor prognosis. Pleural epithelioid hemangioendothelioma usually manifests on chest radiographs and CT scans with unilateral pleural fluid and nodular pleural thickening, appearing similar to that of diffuse pleural carcinomatosis or mesothelioma. It should be considered in the differential diagnosis of a unilateral pleural effusion in an older male patient.
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