Lymphangioma

Background

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
Factor VIII
Ulex europaeus

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.

Commonly Used Terms

Angiosarcoma

Lymphangioendothelioma

Soft tissue tumors

Internet Links

Last Updated 4/1/2001

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 Factor VIII Ulex europaeus