Endovascular papillary angioendothelioma of childhood

Synonym(s)

Dabska tumor; malignant endovascular papillary angioendothelioma; papillary intralymphatic angioendothelioma

Definition

Low-grade angiosarcoma of the skin of children, with a distinctive histologic architecture of anastomosing vascular channels with intravascular papillary outpouchings

Pathogenesis

Unclear cell of origin, but tumor marker studies suggest resemblance to lymphangi-oma

Clinical manifestation

Slow-growing, intradermal nodule that is violaceous, pink, or bluish-black in color

Differential diagnosis

Reactive angioendotheliomatosis; benign intravascular endothelial hyperplasia; reti-form hemangioendothelioma; glomeruloid hemangioma; infantile hemangioma; Kaposi's sarcoma; angiolymphoid hyper-plasia

Therapy

Surgical excision; lymph node dissection if regional nodes are involved

References

Schwartz RA, Dabski C, Dabska M (2000) The Dabska tumor: a thirty-year retrospect. Dermatology 201(1):1-5

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