Systemic angioendotheliomatosis of the lung.
Systemic angioendotheliomatosis is a very rare malignant disease characterized by intravascular neoplastic proliferation of reticuloendothelial cells. According to the clinical features three different types of prognostic value can be distinguished: Exclusive skin involvement; Progressive skin lesions disseminating to internal organs; Aggressive and lethal type affecting primarily internal organs. Until now about 60 cases of systemic angioendotheliomatosis have been reported in the literature. Here we describe the first well-documented case with almost isolated lung involvement. The presentation of morphologic and clinical signs and differential-diagnostic considerations are followed by a critical review of the literature.
Remberger
K
K
Nawrath-Koll
I
I
Gokel
J M
JM
Haider
M
M
D000368:Aged; D002196:Capillaries / Q000473:pathology; D003937:Diagnosis, Differential; D004727:Endothelium / Q000473:pathology; D006390:Hemangioendothelioma / Q000098:blood supply / Q000209:etiology / Q000473:pathology*; D006801:Humans; D008175:Lung Neoplasms / Q000098:blood supply / Q000209:etiology / Q000473:pathology*; D008297:Male