adrenal lymphangioma

Adrenal lymphangiomas, also known as cystic adrenal lymphangiomas, are rare, benign cystic adrenal lesions.

Epidemiology

Adrenal lymphangiomas are extremely rare; prevalence is estimated at 0.06% . They can occur at any age, with a peak incidence between the 3 and 6 decades of life. According to one series, there may be a slight right-sided and female predilection .

Clinical presentation

They usually remain asymptomatic throughout life and are almost always incidentally detected. Rarely, patients may have abdominal pain .

Pathology

They supposedly occur as a result of a developmental abnormality of lymphatic channels .

Gross appearance

Their appearance is that of a lymphangioma occurring anywhere else in the body, i.e. a multilocular cyst.

Immunohistochemistry

• D2-40 cytoplasmic staining tends to be positive (for lining of lymphatic vessels) 
• AE1/AE3 staining tends to be negative

Radiographic features

• typically seen as cystic lobulated lesions of fluid attenuation with thin enhancing walls
• tend to present as relatively large lesions with an average size around 3 cm
• internal septations may be present, and when present, enhance after contrast administration
• may have calcifications, which tend to present in one of two patterns: thick curvilinear or scattered punctate, and can involve the septation(s)

Treatment and prognosis

They are benign lesions, although if they become symptomatic, surgical resection is warranted. Unnecessary surgery, on the other hand, can be the ultimate consequence of unfamiliarity with these rare lesions.

Differential diagnosis

The differential diagnosis for adrenal lymphangiomas is that of adrenal cystic tumors, most of which are rare in themselves, and includes:

• adrenal cyst
• adrenal hemangioma
• cystic pheochromocytoma
• schwannoma with cystic degeneration
• cystic adenoma
• metastatic adenocarcinomas
• adrenal hydatid cyst

Siehe auch:

• Lymphangiom