Adult renal angiomyolipomas: A retrospective analysis of the histological subtypes and their clinicoradiological correlates.

Background: Renal angiomyolipomas (AMLs) are rare, benign mesenchymal tumors of the kidney. Asian data on the prevalence of the subtypes of AMLs and their association with tuberous sclerosis are sparse prompting us to evaluate the clinicopathological characteristics of these tumors. Materials and Methods: We included cases diagnosed from 2001 to 2021 extracting demographic details, clinical presentation, syndromic association with tuberous sclerosis, and preoperative clinicoradiological features from the electronic medical records. Results: Ninety-five cases of adult renal AML were diagnosed among 2402 renal tumors, a prevalence of 3.95%. Forty tumors (42%) were detected incidentally; two patients had life-threatening retroperitoneal hemorrhage. Tuberous sclerosis complex (TSC) was associated with ten cases (10.5%). These patients were a decade younger than those in the non-TSC group (P = 0.008) and had bilateral, multiple, and larger tumors (P = 0.0009, 0.001, and 0.047, respectively). Microscopically, classic and epithelioid subtypes were seen in 87 (91.6%) and 8 cases (8.4%), respectively. Hemorrhage was more common in the epithelioid subtype (P = 0.13). HMB-45, melan-A, and smooth muscle actin immunohistochemistry were useful in cases which lacked the prototypical classic histology and for confirming a diagnosis of epithelioid AML. Conclusions: The prevalence of renal AML in our series was four times higher, and the mean age at diagnosis was a decade earlier than that reported in Western literature but similar to data from two Asian countries. Similar studies from other countries will help ascertain if these differences in prevalence can be attributed to ethnic differences.

Surgical revascularization of bilateral renal artery stenosis due to fibromuscular dysplasia.

Fibromuscular dysplasia (FMD) is a noninflammatory disease affecting small- and medium-sized arteries of the renal and the carotids. It affects the renal arteries in nearly 60%-75% cases. The primary clinical manifestation of renal FMD is hypertension. Medial fibroplasia represents the most common dysplastic lesion. We report two cases who presented with hypertension and renal insufficiency and on evaluation was found to have bilateral renal artery stenosis. Stenting of the renal vessels was not possible due to the narrowed caliber of the vessel and inability to cannulate the renal arteries. They underwent renal artery revascularization with a splenorenal end to end anastomosis. The renal parameters and blood pressure of both the patients stabilized subsequently. Renal revascularization can be a good option for patient having failed angioplasty with stenting.

Total ureterectomy and ileal ureteric replacement for TCC ureter in a solitary kidney.

Traditional treatment of upper tract transitional cell carcinoma (TCC) is nephroureterectomy with a bladder cuff. This is in keeping with the nature of the disease, in that TCC is a panurothelial disease. However, there are a few rare occasions when this would mean making a subject anephric, as in a TCC in a solitary kidney or bilateral synchronous/metachronous disease. We present a case of a patient with a dysplastic, poorly functioning left kidney and with a TCC of the ureter on the right side.