Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression

Abstract

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...

Highly aggresive pulmonary epithelioid hemangioendothelioma: a case report

“…Además, también se ha descrito la sobre-expresión de múlti-ples proteínas señalizadoras, como VEGF, VEGFR-2 y VEGFR-3, lo que sugiere que fár-macos que inhiben estas vías pueden jugar algún papel en el tratamiento sistémico 9 . El derrame pleural, la presencia de sín-tomas respiratorios o la anemia han sido identificados como factores pronósticos clínicos independientes en varios artícu-los, habiéndose descrito una supervivencia mediana inferior a 12 meses cuando alguno de estos elementos estaba presente [10][11][12] . También la presencia de células fusiformes se ha establecido como un marcador de mal pronóstico 12 .…”

Hemangioendotelioma epitelial: um tumor raro com apresentação atípica

“…Apresenta um grau de malignidade baixo a intermédio [3][4][5]7,8 . Nos doentes com envolvimento de predomínio a nível pulmonar, o achado imagiológico característico é a existência de múltiplas lesões nodulares bilaterais, de tamanho inferior a 1 cm e com distribuição perivascular 2,4,7 . O envolvimento multiorgânico documentado nomeadamente pulmonar, hepático, ósseo, cerebral, supra-renal, ganglionar e dos tecidos moles pode dever-se a origem multiorgânica ou metastização à distância.…”

“…O curso clinico e o prognóstico são imprevisíveis 4,7,8 . São factores de mau prognóstico sintomatologia respiratória na altura do diagnóstico 2,4 , presença de doença multicêntrica, metastização hepática ou ganglionar 2,7 , anemia, hemorragia alveolar e derrame pleural hemático 8 . A sobrevida dos casos descritos na literatura varia entre 2 e 24 anos 4 .…”

“…O hemangioendotelioma epitelial (HE) é uma neoplasia rara com origem no endotélio vascular [2][3][4]7,8 . A apresentação pulmonar foi descrita pela primeira vez em 1975 por Dail e Liebow, que consideraram tratar-se de uma forma intravascular de carcinoma bronquioloalveolar [1][2][3][4][5]7 .…”

“…A apresentação pulmonar foi descrita pela primeira vez em 1975 por Dail e Liebow, que consideraram tratar-se de uma forma intravascular de carcinoma bronquioloalveolar [1][2][3][4][5]7 . O desenvolvimento do conhecimento na área da microscopia electrónica e imunohistoquímica permitiu determinar a sua origem endotelial [2][3][4]7 . O HE pode desenvolver-se em vários órgãos e tecidos tais como: pulmões, fígado, osso, cérebro, gânglios linfáticos e tecidos moles.…”