The impact of renal angiomyolipoma on estimated glomerular filtration rate in patients with tuberous sclerosis complex.

BACKGROUND: There is a growing concern that renal impairment may develop in patients with renal angiomyolipomas (AMLs) associated with tuberous sclerosis complex (TSC) as a consequence of the disease itself and/or the interventions to mitigate the risk of hemorrhage. OBJECTIVE: To assess the estimated glomerular filtration rate (eGFR) in patients with bilateral renal AMLs and the impact of tumor burden and intervention on renal function. DESIGN: Retrospective study. SETTING: Urology department of a tertiary care hospital. PATIENTS AND METHODS: All adult patients (>=18 years of age) with TSC-associated renal AMLs seen from October 1998 to June 2015. We included only patients with bilateral tumors or solitary kidneys at the last follow-up. MAIN OUTCOME MEASURES: The eGFR, renal volume, and number and type of interventions. RESULTS: We identified 12 patients (median age 27.6, interquartile range 23.7-39.9 years), a median follow-up period of 1266 days (33-3133), and a median renal size of 454.7 mL (interquartile range 344.7-1016.9 on the right side; 558.1 mL, interquartile range 253.7-1001.4 on the left). In 11 (91.7%) patients, the eGFR was > 60 mL/min/1.77 m2. Six patients had three total nephrectomies, one had a contralateral partial nephrectomy, and seven had selective arterial embolizations. Intervention was associated with a significantly reduced eGFR. The renal size did not correlate with the eGFR. CONCLUSIONS: TSC-associated renal AMLs may attain a large size but normal renal function is maintained in 92% of patients. Interventions to mitigate the risk of hemorrhage are associated with decreased renal function. LIMITATIONS: The renal size was used as a surrogate for tumor size. Other limitations were the limited number of patients and lack of split renal function testing.

Isolated renal hydatid disease in a non-endemic country: a single centre experience.

OBJECTIVE: Isolated renal hydatid disease (HD) is rare in non-endemic countries. Clinical and radiological suspicion warrants appropriate serological tests, preoperative treatment and intra-operative precautions. We present a tertiary care centre experience of isolated renal HD in a non-endemic country. METHODS: We reviewed the medical records of patients with HD treated in the past 20 years. We identified patients with the definitive diagnosis of isolated renal HD and described their management. RESULTS: Of the 119 cases with HD, 6 were found to have isolated renal involvement (5%). Their median age was 46.5 (28-70) years. Five patients presented with flank pain and 1 had an incidentally discovered renal mass. Radiologic investigations raised the suspicion of possible HD in 4 cases, while 2 cases were diagnosed as renal tumours. Computerized tomography showed complex renal cyst in 4, solid renal mass with heterogonous enhancement in 2 and calcification in 5. Eosinophilia and indirect hemagglutination test (IHA) were positive in 3 of the 4 suspected cases. Three cases were treated as renal tumours, while 3 were managed as HD. Four cases had total nephrectomy and 2 had partial nephrectomy. Histopathology revealed that all cases had renal HD. Patients were followed for a median of 7.3 (0.4-11.3) years with no evidence of recurrence. CONCLUSIONS: Isolated renal HD is a challenging preoperative diagnosis in non-endemic countries. The definitive diagnosis is only possible by histopathology. Retrospectively, HD mimicked renal tumours in half the cases and should be considered in the differential diagnosis of renal space occupying lesions.