Renal lymphangiomatosis: literature analysis on research progress and presentation of four cases

[摘要] Renal lymphangioma is a rare disease (1-3), being more ofa malformation rather than a true tumor. It is caused by aninability of the renal lymphatic system to communicate withthe normal lymphatic system, resulting in the lymphaticdilation of the kidney, renal capsule, and perirenal areaand the formation a single or multilocular cystic mass.Due to the relative rarity of the disease, the currentdisease understanding is based on a few case reports andmultiple case series reports. The misdiagnosis rate ofrenal lymphangioma is high, as it is easily confused withother renal cystic diseases (e.g., hydronephrosis, polycystickidney disease, and parapelvic cyst) (4-6). Imagingplays an important role in its diagnosis and differentialdiagnosis. Although renal lymphangioma is itself benign,studies (7-11) have found that it may cause more seriouscomplications (e.g., hypertension, renal vein thrombosis,renal insufficiency, glomerulonephritis, and ascites). Thenatural history of renal lymphangioma is largely unknown.Only reports by Meredith et al. (12) and Antonopouloset al. (13) and our first report of 3 related cases (2 sistersand their father) support the hypothesis of family diseaseheredity. Thus, to further inform clinical diagnosisand treatment, we selected 4 case reports, conducteda retrospective analysis on published Chinese andinternational articles on this rare disease; describedthe clinical and laboratory characteristics, imagingmanifestations, treatment methods, and other factors ofrenal lymphangioma; and classified them accordingly.

[学科分类] 外科医学