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…

Progressive Lymphangioma David Cassarino, MD, PhD Key Facts Terminology Benign, localized proliferation of lymphatic vessels Also known as Acquired progressive lymphangioma and benign lymphangioendothelioma Clinical Issues Usually presents on lower…

Papillary Endothelial Hyperplasia (Masson Tumor) Amitabh Srivastava, MD Key Facts Terminology Benign, reactive, intravascular papillary endothelial proliferation Clinical Issues Wide site distribution; located in deep dermis or subcutaneous tissue Macroscopic…

Angiokeratoma Steven D. Billings, MD Key Facts Terminology Ectatic superficial dermal blood vessels associated with reactive epidermal hyperplasia Clinical Issues Solitary and multiple angiokeratomas Angiokeratoma of Fordyce Angiokeratoma of Mibelli…

Capillary, Venous, and Cavernous Hemangiomas Amitabh Srivastava, MD Key Facts Terminology Benign vascular tumors composed of blood vessels lined by plump to flattened endothelial cells with no atypia Etiology/Pathogenesis May…

Verrucous Hemangioma Steven D. Billings, MD David Cassarino, MD, PhD Key Facts Clinical Issues Presents in children Dark blue nodule Surface has warty appearance Most common on the extremities Microscopic…