Basicmedical Key

Kaposi Sarcoma Thomas Mentzel, MD Key Facts Terminology Locally aggressive endothelial neoplasm associated with human herpes virus 8 Clinical Issues Most typical site of involvement is skin Mucosal membranes, lymph…

Epithelioid Hemangioendothelioma Cyril Fisher, MD, DSc, FRCPath Key Facts Terminology Vascular neoplasm with metastatic potential composed of epithelioid endothelial cells Clinical Issues Rare vascular tumor Superficial or deep soft tissue…

Atypical Vascular Lesion Thomas Mentzel, MD Key Facts Terminology Vascular proliferation after radiotherapy, excluding obvious angiosarcoma, arising predominantly in breast Clinical Issues Exact incidence is difficult to establish Small papules,…

Papillary Intralymphatic Angioendothelioma (Dabska Tumor) David Cassarino, MD, PhD Julie C. Fanburg-Smith, MD Key Facts Terminology Dabska tumor Endovascular papillary angioendothelioma Clinical Issues Typically occur in children (minority in adults)…

Glomus Tumors Thomas Mentzel, MD Key Facts Terminology Perivascular myogenic mesenchymal neoplasm composed of cells closely resembling smooth muscle cells of normal glomus body Clinical Issues Distal extremities, especially in…

PEComas Elizabeth A. Montgomery, MD Key Facts Terminology Mesenchymal neoplasm composed of distinctive perivascular epithelioid cells (PEComa) Includes angiomyolipoma (AML), clear cell “sugar” tumor of lung (CCST), lymphangioleiomyomatosis (LAM), clear…

Kaposiform Hemangioendothelioma Steven D. Billings, MD Key Facts Clinical Issues Presents in infants and young children Deep tumors, often multiple nodules Associated with Kasabach-Merritt phenomenon (especially retroperitoneal tumors) Consumptive coagulopathy…

Lymphangioma and Superficial Lymphangioma David Cassarino, MD, PhD Lester D. R. Thompson, MD Key Facts Terminology Lymphangioma circumscriptum (superficial lymphangioma) (LAC) Lymphangiomatosis (systemic angiomatosis) (LAS) Cystic lymphangioma (cystic hygroma) Deep…

Bacillary Angiomatosis David Cassarino, MD, PhD Key Facts Terminology Reactive vascular proliferation associated with Bartonella (gram-negative coccobacilli) bacterial infection Etiology/Pathogenesis Most patients are immunosuppressed, especially HIV/AIDS Also associated with organ…

Myxoinflammatory Fibroblastic Sarcoma Elizabeth A. Montgomery, MD Key Facts Clinical Issues Slow-growing infiltrative mass usually affecting distal extremities (hands and feet) of adults Recurrences are common, but metastases are rare…