title: [Epithelioid hemangioendothelioma of the lung (IVBAT)--clinicopathological and immunohistochemical analysis of 11 cases].
abstract: Epithelioid hemangioendothelioma (EHE) of the lungs (syn.: intravascular bronchioloalveolar tumor/IVBAT) is a neoplasm with a vascular line of differentiation. This study provides a clinicopathological analysis of 11 cases of this tumor. There were 7 female and 4 male patients. Patients' age ranged from 33 to 83 years (mean: 49.8, median: 48 years) showing 2 peaks (third/fourth and sixth/eighth decades). The typical radiological appearance revealed multiple bilateral lung nodules from 0.5 to 2 cm. In 2 cases, a striking perivascular and intravascular growth pattern associated to pulmonary veins was evident. All tumors were characterized by epithelioid tumor cells with nests, cords and strands embedded in a sclerosing, hyaline matrix with alveolar, bronchiolar and vascular extension. Necrosis was identified in 8 tumors and ossification in 2 tumors. The mitotic index was below one mitosis in 10 HPF. By immunohistochemistry, all tumors were positive for CD 31, CD 34, and vimentin, 60% positive for factor VIII, and negative for pancytokeratin and calretinin. Follow-up was available for 8 patients with a range of 6 to 105 months (mean: 51.5 months, median: 52 months): 3 patients died (2 certainly from their tumors), 4 are alive disease-free, and one is alive with metastases. EHE of the lungs (IVBAT) represents an intermediate-grade, malignant, mesenchymal neoplasm with a characteristic histological appearance and immunohistochemical profile.
authors.affiliation: Institut für Pathologie der Ruhr-Universität Bochum, Berufsgenossenschaftliche Kliniken Bergmannsheil.
authors.forename: B
authors.initials: B
authors.lastname: Einsfelder
authors.forename: C
authors.initials: C
authors.lastname: Kuhnen
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis*; D001706:Biopsy; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D004730:Endothelium, Vascular / Q000473:pathology; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000175:diagnosis / Q000401:mortality / Q000473:pathology*; D006801:Humans; D008168:Lung / Q000473:pathology; D008175:Lung Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D008940:Mitotic Index; D009336:Necrosis; D009361:Neoplasm Invasiveness; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis / Q000401:mortality / Q000473:pathology*; D009999:Ossification, Heterotopic / Q000175:diagnosis / Q000473:pathology; D011379:Prognosis; D011667:Pulmonary Veins / Q000473:pathology; D016019:Survival Analysis
chemical_list: D014408:Biomarkers, Tumor