dr james c wittig, orthopedic oncologist, new york, new jersey
Patient Education
Lymphangioma

GENERAL INFORMATION

Lymphangioma is a benign vascular lesion. It is believed to originate from a non communication between the lymphatics and venous system.

CLINICAL DATA

 A benign vascular lesions
 Appear to show lymphatic differentiation
• Thin-walled architecture
• Jagged poorly circumscribed outline
• Pale proteinaceous fluid
• Commonly association with stromal lymphoid component
 3 types
• Lymphangioma circumcript
• Lymphangioma proliferation affecting the skin
• Cavernous lymphangioma
• Lymphatic proliferation characterized by large, dilated lymphatic channels
• Progressive lymphangioma
• Rare lymphatic proliferation occurring in the skin
• Characterized by dissecting vascular channels
 Usually affect age 40 to 70 years
 Slight female preference

Differential Diagnosis
 Lipoma
• Lymphangiectasia
• Neurofibromatosis
• Hemangioma
• Gorham’s Disease


CLINICAL PRESENTATION

Signs/Symptoms
 Lymphangioma circumscriptum
• Multiple, cutaneous, thin-walled vesicles over large area
• Cavernous lymphangioma
• Soft, spongy masses of variable size
• Progressive lymphangioma
• Reasonably circumscribed, pinkish red cutaneous macule
• Progressive enlargment over years
• May reach considerable size (>10 cm)

Prevalence
• ~5% of vascular tumors
• No discernable predilection for any race or sex

Age
• Progressive lymphangioma
• Mainly in adults
• Lymphangioma circumscriptum and cavernous lymphangioma
• Predominantly in infants and children

Sites
• Lymphangioma circumscriptum
• Proximal limb and limb girdle
• Commonly thigh and buttocks
• Cavernous lymphangioma
• Neck
• Upper trunk
• Oral cavity
• Inguinal region
• Progressive lymphangioma
• Usually extremities
• May occur on the trunk


RADIOGRAPHIC PRESENTATION

Plain x-ray
 Reveals a large soft tissue mass (may depict calcification)

CT
• Unilocular or multiocular mass of water attenuation
• Lesion walls and septa are of uniform thickness

MRI
• Heterogeneous low signal intensity similar to muscle on T1W
• High signal intensity, greater than fat on T2W

PATHOLOGY

Gross
 All types are very similar, often display features of both
• Vary between
• Well-circunscribed lesions
• Made up one or more interconnecting cysts
• Ill-defined spongelike compressible lesions
• Composed of microscopic cysts
• Sometimes present with infiltrative borders
• Rarely exceeds the 13 cm Ø


Microscopic
 Lymphangioma circumscriptum
• Dilated lymphatic channels
• Immediately below the dermis
• Also within deeper dermis and subcutis
• Associated epidermal hyperplasia
• Lymphocytic infiltrate
• Lymphatic endothelium
• Flattened and attenuated
• No atypia
• Progressive lymphangioma
• Thin-walled lymphatic channels
• In the dermis
• Ramify or dissect between dermal collagen
• Endothelial cells
• Tend to have hyperchromatic nuclei
• No multilayering, atypia, mitotic activity
• Cavernous lymphamgioma
• Large, dilated lymphatic spaces
• Attenuated endothelial lining
• Contains variable amounts of lymph
• Also contains lymphocytes and red blood cells
• Larger lymphatic spaces
• Often show marked interstitial fibrosis
• May show diffuse stromal inflammatory changes

Immunohistochemistry
 Positive for 
• CD31
• CD34
• D2-40
• Patchy factor VIII
 Negative
• Ki-67

PROGNOSIS

Biological Behavior
 Believed to be result of developmental malformations
• Lymphangioma circumscriptum
• Not agressive
• Not infiltrative
• Prone to repeated recurrence
• Perhaps due to unwillingness of surgeon to perform radical procedure
• Progressive lymphangioma
• Not agressive
• May be slight infiltrative
• Usually only into surrounding soft tissue
• Following complete excision, no tendency towards local recurrence
• No metastases
• Little recurrence in progressive lymphangioma
• Repeated recurrences likely in lymphangioma circumscriptum and cavernous lymphangioma
• Due to difficulties associated with excision

TREATMENT

• Non surgical
• Sclerotherapy 
• Observation
• Intralesional bleomycin therapy 
• Complete surgical excision
• May be extremely difficult in some cases (large, deep and complex masses)
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