This is a benign proliferation of lymph vessels. Unlike its cousin, the hemangioma, these tumors are rare. However, they are important to recognize because they may mimic many vascular tumors including angiosarcomas and Kaposi's sarcoma.
These tumors may occur anywhere but usually present in the head and neck in children and infants. Visceral organs such as the lungs and gastrointestinal tract may also be involved. In the soft tissue, these tumors present as a soft fluctuant mass that varies in size.
SYNONYMS Lymphangioma INCIDENCE Rare AGE RANGE-MEDIAN Birth 50-60%
<2 years 90%
SEX (M:F) Slight male predominance
EPIDEMIOLOGIC ASSOCIATIONS CHARACTERIZATION Hydrops fetalis Cystic hygroma Turner's syndrome Cystic hygroma Noonan's syndrome Cystic hygroma Familial pterygium colli Cystic hygroma Fetal alcohol syndrome Cystic hygroma
GROSS APPEARANCE/CLINICAL VARIANTS CHARACTERIZATION Cystic hygroma Head and neck
May present with airway and feeding problems
10% may extend into the mediastinum
Intrabdominal May not manifest until adult life although 60% present by 5 years of age Cutaneous Lymphangioma circumscriptum Multiple small vesicles or nodules on localized portions of the body Deep Deep soft tissue
HISTOLOGICAL TYPES CHARACTERIZATION General Well circumscribed lesions made up of one or more interconnecting cysts
Lined by bland endothelial cells
Cystic hygromas Smaller spongelike lesions Cavernous lymphangiomas With larger cysts
SPECIAL STAINS/IMMUNOPEROXIDASE/OTHER CHARACTERIZATION Vascular markers CD31
PROGNOSIS AND TREATMENT CHARACTERIZATION Prognostic Factors
Benign but may cause morbidity if tumors are large, impinge upon critical structures, or become secondarily infected
Cavernous tumors are more difficult to excise and are prone to recurrence
Treatment Surgical removal
For neonatal lesions, surgery usually delayed until 18-24 months
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
Soft tissue tumors
Last Updated 4/1/2001
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