Renal Lymphangiectasis
Aleksandr Vasilyev, MD, PhD
Key Facts
Terminology
Hamartomatous or acquired obstructive lesion resulting in cystic dilatation of kidney lymphatics
Clinical Issues
No age or gender predilection; may be exacerbated by pregnancy
May present with flank pain, abdominal mass or ascites, hematuria &/or proteinuria, hypertension, polycythemia, renal vein thrombosis
Microscopic Pathology
Cyst lining is negative for epithelial markers and positive for vascular &/or lymphatic markers: CD31, CD34, podoplanin (D2-40)
Top Differential Diagnoses
Polycystic kidney disease
Cystic dysplasia
Cystic nephroma
Wilms tumor
 Kidney with lymphangiectasis shows multiple thin-walled cysts with clear fluid content. The cysts displace renal parenchyma, making it difficult to distinguish lymphangiectasis from other cystic diseases. |
 CT scan of a patient with renal lymphangiectasis shows perinephric accumulations of fluid bilaterally  , although there is significant asymmetry in the degree of cystic change. |
TERMINOLOGY
Synonyms
Renal lymphangioma, hygroma renalis, renal lymphangiectasis pericalyceal lymphangiectasis, peripylic lymphangiectasis
Definitions
Hamartomatous or acquired obstructive lesion resulting in cystic dilatation of kidney lymphatics
ETIOLOGY/PATHOGENESIS
Developmental Anomaly
Malformation of kidney lymphatic drainage system has been suggested
Lymphatic Obstruction
Lymphatic obstruction secondary to trauma or inflammation in renal pelvis has also been suggested
Additional Associations
Rare occurrence in siblings suggests genetic component
Sometimes found in association with generalized lymphangiomatosis
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