Background

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 Self-healing pseudoangiosarcoma
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 CD34 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
Angiosarcoma	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.

Angiosarcoma

Lymphangioma

Soft tissue tumors

Last Updated 4/1/2001

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