Angiomyolipoma is a benign hamartomatous tumor. It constitutes the most frequent renal disease encountered in Bourneville’s tuberous sclerosis, the bilateral character of which is highly suggestive. We report the observation of a 48-year-old patient with Bourneville’s tuberous sclerosis with bilateral angiomyolipoma.

Only approximately 5% of urothelial tumors arise in the upper urinary tract, > 90% of which are urothelial (formerly called transitional cell) carcinomas. Upper tract urothelial carcinoma (UTUC) arise from the renal pelvis and the ureter. Genetic and environmental risk factors may contribute to the development of UTUC. Exposure to or consumption of tobacco, phenacetine, aristolochic acid, aromatic amines, and arsenic contaminated drinking water are known risk factors for urothelial carcinogenesis. Clinical features include hematuria, flank pain, hydronephrosis, palpable flank mass, renal insufficiency, hypertension and a high propensity for distant metastasis. Paraneoplastic syndrome may include hypercalcemia, thrombocytopenia and leukemoid reaction. Because estimated 60% of UTUCs are invasive at diagnosis, appropriate diagnosis and management is most important. The predominant sites of distant metastasis at diagnosis of UTUC were the lungs, lymph nodes, bone and liver. Cutaneous metastasis is extremely rare and indicates the late manifestation of a systemic spread. Combinations of urine cytology, cystoscopy, ureteroscopic biopsy, and computed tomography are currently diagnostic tools. Surgery with radical nephroureterectomy and excision of a bladder cuff is the gold standard for locally invasive tumors. Neoadjuvant or adjuvant chemotherapy with cisplatin- or platinum-based regimens may improve survival in patients with metastatic disease. Checkpoint inhibitors immunotherapy targeting programmed cell death 1 receptor and its ligand have provided a new treatment option, particularly for patients with progressive UTUC disease following platinum-based chemotherapy. Tumor grade and stage are major prognostic factors for oncological outcomes. As potential tumor recurrence, repeated surveillance by urine cytology, cystoscopy, upper tract imaging and ureteroscopy following treatment are required.

Introduction: Bilateral pelvic kidneys occur when the two kidneys fail their ascent and remain in the pelvic area during embryologic development. It is a rare even observed for 10% of patients with renal ectopia. The aim of this case is to report a rare renal abnormality.

Case report: A 60 year-old male presented with intermittent pain in his left lower abdomen since the past 4 years. Physical examination and biological tests were normal. Contrast enhanced CT revealed bilateral pelvic kidneys with a stone in left kidney pelvis and hydronephrosis.

Conclusions: This congenital abnormality is usually found incidentally but may be painful when complications are associated. The best evaluation of the patients is the key for their proper management.