Angiosarcoma Cyril Fisher, MD, DSc, FRCPath Key Facts Terminology Malignant mesenchymal neoplasm of cells recapitulating variable morphologic and functional features of endothelial cells Clinical Issues Deep soft tissues Lower extremities,…

Cellular Neurothekeoma Cyril Fisher, MD, DSc, FRCPath Key Facts Terminology Multinodular dermal tumor composed of nests of rounded cells separated by delicate fibrous septa Differs from dermal nerve sheath myxoma…

Schwannoma (Neurilemmoma) Amitabh Srivastava, MD Key Facts Terminology Encapsulated, benign peripheral nerve sheath tumor composed predominantly of Schwann cells Clinical Issues Common between 20-50 years of age Affects males and…

Neurofibroma Amitabh Srivastava, MD Key Facts Terminology Benign peripheral nerve sheath tumor with heterogeneous admixture of axons, Schwann cells, perineurial cells, and fibroblasts Most are sporadic; neurofibromatosis type 1 (NF1)…

Neuromas Cyril Fisher, MD, DSc, FRCPath Key Facts Terminology Neuroma represents proliferation of peripheral nerve fibers in which ratio of axons to Schwann cell fascicles approaches 1:1 Solitary circumscribed neuroma…

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…