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aaPulmonary epithelioid haemangioendothelioma (PEH) is a rare tumour of the lung. The disease was originally reported by DAIL and LIEBOW [1] in 1975 as "intravascular bronchoalveolar tumour" (IVBAT), and the majority (65%) patients of the reported 20 patients showed multiple bilateral nodular lesions. In addition, one patient had bronchoalveolar cell carcinoma. The recognition of Weibel-Palade bodies in the cytoplasm of tumour cells of IVBAT by electron microscopy, along with the immunoreactivity of the cytoplasm of tumour cells for factor VIII-related antigen, prompted the realization that IVBAT probably derived from vascular endothelial cells [2][3][4][5][6][7][8][9]. Forty-one patients with soft-tissue neoplasms exhibiting histopathological features similar to IVBAT were reported by WEISS and ENZINGER [10] in 1982. The tumour was termed epithelioid haemangioendothelioma [2,[9][10][11][12], and by 1986 had been reported to occur also in the liver, head and neck area, oral mucosa, bone, mediastinum, diaphragm and brain [13].We reported on two asymptomatic patients with IVBAT (PEH) in the mid-1980s [14,15], and observed partial spontaneous regression of the disease during follow-up in both. In order to evaluate more thoroughly the clinicopathological spectrum of PEH, 21 patients with PEH recruited in Asia (Japan, Korea, Taiwan and Singapore) were studied, who included an additional patient with partial spontaneous regression [16]. The description of all of these patients and their prognostic features form the basis of the present report. Methods Study populationTo obtain data on the clinical and pathological features of PEH in as many patients as possible, a survey was carried out of the Japanese literature of the past 15 yrs and letters were sent to Departments of Medicine, Respiratory Medicine, Radiology and Pathology of 230 major hospitals in Japan. In addition, letters were sent to five major hospitals in Korea, Taiwan, Hong Kong and Singapore. In order to confirm the diagnosis of PEH, the histopathological specimens from 23 patients were reviewed at an International Meeting on PEH held in 1994 in Kyoto, Japan. Six out of the 23 patients have been reviewed previously by one of the present authors (M. Kitaichi) [14][15][16][17].The clinical summary of patients with PEH focused on the features described in the Results section at the time of the first examination by their primary physicians (see Acknowledgements) and at the time of the last clinical contact.The patients were also questioned about the type and duration of therapy, if any. The clinical onset of PEH was defined as the time of onset of pulmonary symptoms attributable to the disease. Clinical improvement or deterioration was assessed by chest radiography. Histopathological and immunohistochemical studiesHistopathological features were analysed according to 18 items [2,9]. The cellularity of tumour cells was graded from Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression. M. Kitaichi, S. Nag...
aaPulmonary epithelioid haemangioendothelioma (PEH) is a rare tumour of the lung. The disease was originally reported by DAIL and LIEBOW [1] in 1975 as "intravascular bronchoalveolar tumour" (IVBAT), and the majority (65%) patients of the reported 20 patients showed multiple bilateral nodular lesions. In addition, one patient had bronchoalveolar cell carcinoma. The recognition of Weibel-Palade bodies in the cytoplasm of tumour cells of IVBAT by electron microscopy, along with the immunoreactivity of the cytoplasm of tumour cells for factor VIII-related antigen, prompted the realization that IVBAT probably derived from vascular endothelial cells [2][3][4][5][6][7][8][9]. Forty-one patients with soft-tissue neoplasms exhibiting histopathological features similar to IVBAT were reported by WEISS and ENZINGER [10] in 1982. The tumour was termed epithelioid haemangioendothelioma [2,[9][10][11][12], and by 1986 had been reported to occur also in the liver, head and neck area, oral mucosa, bone, mediastinum, diaphragm and brain [13].We reported on two asymptomatic patients with IVBAT (PEH) in the mid-1980s [14,15], and observed partial spontaneous regression of the disease during follow-up in both. In order to evaluate more thoroughly the clinicopathological spectrum of PEH, 21 patients with PEH recruited in Asia (Japan, Korea, Taiwan and Singapore) were studied, who included an additional patient with partial spontaneous regression [16]. The description of all of these patients and their prognostic features form the basis of the present report. Methods Study populationTo obtain data on the clinical and pathological features of PEH in as many patients as possible, a survey was carried out of the Japanese literature of the past 15 yrs and letters were sent to Departments of Medicine, Respiratory Medicine, Radiology and Pathology of 230 major hospitals in Japan. In addition, letters were sent to five major hospitals in Korea, Taiwan, Hong Kong and Singapore. In order to confirm the diagnosis of PEH, the histopathological specimens from 23 patients were reviewed at an International Meeting on PEH held in 1994 in Kyoto, Japan. Six out of the 23 patients have been reviewed previously by one of the present authors (M. Kitaichi) [14][15][16][17].The clinical summary of patients with PEH focused on the features described in the Results section at the time of the first examination by their primary physicians (see Acknowledgements) and at the time of the last clinical contact.The patients were also questioned about the type and duration of therapy, if any. The clinical onset of PEH was defined as the time of onset of pulmonary symptoms attributable to the disease. Clinical improvement or deterioration was assessed by chest radiography. Histopathological and immunohistochemical studiesHistopathological features were analysed according to 18 items [2,9]. The cellularity of tumour cells was graded from Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression. M. Kitaichi, S. Nag...
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.
This report describes a case of pulmonary epithelioid haemangioendothelioma presented as multi-organ metastases including lung, liver and bone in a 56-year-old woman with an initial diagnosis made with thoracoscopic wedge biopsy. The diagnosis is confirmed through immunohistochemistry. This is a rare disease, with approximately 90 cases described in the English literature and approximately 10 cases reported in China. The case of PEH presented as multiple pulmonary nodules and metastasing to liver and bone is rare in the English 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 report may contribute to the data on clinical findings and natural history of this rare tumor.
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