Renal Lesions in Tuberous Sclerosis

Abstract

The renal lesion classically associated with tuberous sclerosis is angiomyolipoma. Renal cysts are less frequent, occurring alone or in conjunction with angiomyolipomas. We reviewed the records of 274 patients with tuberous sclerosis registered at our clinic. Of 95 patients evaluated for renal lesions 51 (54 per cent) had positive findings: 45 had angiomyolipomas, 17 had renal cysts and 11 had both lesions. These lesions usually are multiple and bilateral, and are diagnosed most effectively with computerized tomography or ultrasound. Surgery (10 kidneys) was done for life-threatening hemorrhage or suspected malignant lesions.

Embolisation of Bleeding Renal Angiomyolipoma in Pregnancy

“…AML appears in two distinctive settings, as part of the tuberous sclerosis complex (TSC) and sporadically in individuals who otherwise have no clinical features of that entity. In the former group, these tumours are often bilateral, have multicentric foci and tend to bleed more often than sporadic variant [3,4]. Steiner et al demonstrated that this group have worse outcome with faster growth rates (67% versus 21%) and earlier need for operating intervention (50% versus 28%) [5].…”

Guidelines and care pathways for genetic diseases: the Scottish collaborative project on tuberous sclerosis

“…[30][31][32] Expert recommendations also support investigation of the kidneys by ultrasound. [12][13]22,[25][26] Asymptomatic hepatic hamartomas were found in 23.5% of children with TS.…”

“…Renal Renal involvement occurs in approximately 60% of patients with TS. [30][31][32] Angiomyolipomas (AMLs) are the commonest lesions occurring in approximately 50% of individuals with TS, 30 followed by renal cysts found in approximately 15-18%. 30,52 There may also be a risk of renal cell carcinoma (RCC).…”

“…[30][31][32] Angiomyolipomas (AMLs) are the commonest lesions occurring in approximately 50% of individuals with TS, 30 followed by renal cysts found in approximately 15-18%. 30,52 There may also be a risk of renal cell carcinoma (RCC). 18 Regular monitoring of the kidneys is indicated 18,20,52-62 and ultrasound scanning seems to be the most appropriate modality in most cases.…”

Tuberous Sclerosis: A Case Report

“…They may be found independently or together: they may be unilateral, bilateral, single or multiple. Angiomyolipomas are benign in nature and asymptomatic but spontaneous rupture and subsequent hemorrhage in retroperitoneum may occur 11 . In the heart, the most frequent and characteristic type of tumor is cardiac rhabdomyomas.…”