Hemangioma with Kaposi's sarcoma-like features: report of two cases. 1989

G W Niedt, and M A Greco, and R Wieczorek, and W A Blanc, and D M Knowles
Department of Pathology, New York University School of Medicine, New York 10016.

We describe two children with vascular neoplasms that resembled Kaposi's sarcoma in places. Both presented with intraabdominal masses and severe thrombocytopenia. At autopsy the tumors extensively infiltrated the peritoneum and retroperitoneum and surrounded or invaded numerous organs including the kidneys, pancreas, adrenal glands, gastrointestinal tract, mesentery, and lymph nodes in both cases, and spleen or bone marrow in one case each. The neoplasms were histologically identical and displayed two patterns: dilated vascular spaces (angiomatous areas) lined by flat endothelial-like cells and areas of spindle cells forming slitlike vascular spaces similar to those described in Kaposi's sarcoma. Tumor cells in both cases expressed markers for endothelial cells. The clinical and histologic character of these neoplasms differentiates them from Kaposi's sarcoma, hemangioendothelioma, and from conventional juvenile hemangioma.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D009361 Neoplasm Invasiveness Ability of neoplasms to infiltrate and actively destroy surrounding tissue. Invasiveness, Neoplasm,Neoplasm Invasion,Invasion, Neoplasm
D010534 Peritoneal Neoplasms Tumors or cancer of the PERITONEUM. Peritoneal Carcinomatosis,Peritoneal Surface Malignancy,Carcinomatosis, Peritoneal,Malignancy, Peritoneal Surface,Neoplasm, Peritoneal,Peritoneal Carcinomatoses,Peritoneal Neoplasm,Peritoneal Surface Malignancies,Surface Malignancy, Peritoneal
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
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
D005260 Female Females
D006391 Hemangioma A vascular anomaly due to proliferation of BLOOD VESSELS that forms a tumor-like mass. The common types involve CAPILLARIES and VEINS. It can occur anywhere in the body but is most frequently noticed in the SKIN and SUBCUTANEOUS TISSUE. (from Stedman, 27th ed, 2000) Angioma,Chorioangioma,Hemangioma, Histiocytoid,Hemangioma, Intramuscular,Chorangioma,Chorangiomas,Chorioangiomas,Hemangiomas,Hemangiomas, Histiocytoid,Hemangiomas, Intramuscular,Histiocytoid Hemangioma,Histiocytoid Hemangiomas,Intramuscular Hemangioma,Intramuscular Hemangiomas
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012186 Retroperitoneal Neoplasms New abnormal growth of tissue in the RETROPERITONEAL SPACE. Neoplasm, Retroperitoneal,Neoplasms, Retroperitoneal,Retroperitoneal Neoplasm
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

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