Rapidly fatal lymphangioma-like Kaposi's sarcoma. 1980

M R Leibowitz, and M Dagliotti, and E Smith, and J F Murray

A 34-year-old black South African male was treated for lymphangioma-like clinical Kaposi's sarcoma. The disease followed a rapidly fatal course and was characterized by unexplained recurrent pleural effusions and severe refractory anaemia due to the presence of dilated vascular channels in the bone marrow. Definitive diagnosis was only made at autopsy. Lymphangiomatous changes in the skin without spindle cell formation caused diagnostic difficulties.

UI MeSH Term Description Entries
D008200 Lymphangiectasis A transient dilatation of the lymphatic vessels. Lymphangiectases
D008202 Lymphangioma A benign tumor resulting from a congenital malformation of the lymphatic system. Lymphangioendothelioma is a type of lymphangioma in which endothelial cells are the dominant component. Lymphangioendothelioma,Lymphangioma, Cavernous,Endothelioma, Lymphatic,Cavernous Lymphangioma,Cavernous Lymphangiomas,Endotheliomas, Lymphatic,Lymphangioendotheliomas,Lymphangiomas,Lymphangiomas, Cavernous,Lymphatic Endothelioma,Lymphatic Endotheliomas
D008297 Male Males
D010996 Pleural Effusion Presence of fluid in the pleural cavity resulting from excessive transudation or exudation from the pleural surfaces. It is a sign of disease and not a diagnosis in itself. Effusion, Pleural,Effusions, Pleural,Pleural Effusions
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D012514 Sarcoma, Kaposi A multicentric, malignant neoplastic vascular proliferation characterized by the development of bluish-red cutaneous nodules, usually on the lower extremities, most often on the toes or feet, and slowly increasing in size and number and spreading to more proximal areas. The tumors have endothelium-lined channels and vascular spaces admixed with variably sized aggregates of spindle-shaped cells, and often remain confined to the skin and subcutaneous tissue, but widespread visceral involvement may occur. Kaposi's sarcoma occurs spontaneously in Jewish and Italian males in Europe and the United States. An aggressive variant in young children is endemic in some areas of Africa. A third form occurs in about 0.04% of kidney transplant patients. There is also a high incidence in AIDS patients. (From Dorland, 27th ed & Holland et al., Cancer Medicine, 3d ed, pp2105-7) HHV-8 is the suspected cause. Kaposi Sarcoma,Kaposi's Sarcoma,Multiple Idiopathic Pigmented Hemangiosarcoma,Kaposis Sarcoma,Sarcoma, Kaposi's
D012878 Skin Neoplasms Tumors or cancer of the SKIN. Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm

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