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

Abstract

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.

Keywords

• angiomyolipomas
• hmb-45
• perivascular epithelioid cell tumors
• renal
• tuberous sclerosis