This rare proliferation of lymphatic vessels is important because its histologic appearance may mimic many vascular tumors including angiosarcoma and Kaposi's sarcoma. These tumors present in the skin, usually on the lower extremities but the head and neck region may be affected. It is a solitary, well-circumscribed, slowly enlarging red or pigmented patch or plaque. It is present for about 3 years but has been noted from 1 week to 25 years. Most are about 4 cm in size.
SYNONYMS Benign lymphangioendothelioma
Acquired progressive lymphangioma
INCIDENCE Rare AGE RANGE-MEDIAN 17-90 years
Median 54 years
SEX (M:F) Equal
HISTOLOGICAL TYPES CHARACTERIZATION General
Anastomosing, widely dilated vascular structures developing within the superficial dermis
With growth, the vascular spaces collapse and dissect the dermal collagen, resembling angiosarcoma
Lining endothelial layer composed of bland cells
Occasional papillary projections of endothelial cells
SPECIAL STAINS/IMMUNOPEROXIDASE/OTHER CHARACTERIZATION Vascular markers CD31
Factor VIII related antigen
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES Kaposi's sarcoma
Vascular spaces less widely dilated and less thick walled vessels
Lymphangioma variant of KS often admixed with other variants of KS such as patch, plaque, and nodular stage
More common in elderly
Lining endothelial cells consistently show cytologic atypia
PROGNOSIS AND TREATMENT CHARACTERIZATION Prognostic Factors Recurrence is rare Treatment Complete excision
Am J Surg Pathol 200;24:1047-1057.
Soft tissue tumors
Last Updated 4/1/2001
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