Emergency embolization of actively bleeding renal angiomyolipoma in a patient of tuberous sclerosis

Abstract

Renal angiomyolipoma is a benign hamartomatous lesion which contains varying proportions of abnormal blood vessels along with muscle and fat component. Tuberous sclerosis predisposes to angiomyolipoma, and when associated, the lesion is often large with increased risk of hemorrhage and complications. When this occurs, transarterial embolization and surgery are the treatment options. Such a patient presented to us with acute onset of symptoms, and on account of severe anemia with active bleeding, transarterial embolization was performed. This was followed by elective nephrectomy without any complications. We present the imaging aspects of tuberous sclerosis and emphasize role of interventional radiology in this case.

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

“…[ 1 8 9 ] Although benign, acute life-threatening presentation with spontaneous hemorrhage leading to shock and hypotension, as seen in two of our patients, has been reported before. [ 10 11 ] The sporadic form of AML, seen in 89.5% of cases, is typically detected incidentally on routine imaging for other illnesses or at autopsy and occurs as small, fat-rich, well-circumscribed tumors. AMLs associated with tuberous sclerosis, occurring in 10.5% of our cases, are frequently multiple and are associated with inactivating mutations in TSC1 and TSC2, leading to hyperactivation of the mammalian target of rapamycin signaling pathway.…”

Tuberous sclerosis-associated renal angiomyolipoma: A report of two cases and review of the literature

“…Overall, the number of sporadic RAMLs is four times that of the cases of TSC-associated RAML (3). Furthermore, TSC-associated RAMLs are more likely to be large, multifocal, and complicated by flank pain, abdominal pain, retroperitoneal hemorrhage or hematuria (1)(2)(3)6,11,12). TSC-associated RAMLs have a greater risk of hemorrhage compared with sporadic RAMLs (12)(13)(14).…”

“…In short, RAML is common in patients with TSC, but rare in the general population (2). Notably, TSC-associated RAMLs are typically bilateral and multifocal, larger, and more likely to lead to potentially life-threatening hemorrhage compared with isolated RAML (1). TSC is a rare autosomal dominant disease with incomplete penetrance; it is characterized by a constellation of findings affecting multiple organ systems, and has a worldwide prevalence of ~1/6,000 to 1/12,000 individuals (2,(6)(7)(8).…”

“…Renal angiomyolipoma (RAML) is a type of benign hamartoma that may occur sporadically or be associated with tuberous sclerosis complex (TSC) (1)(2)(3). It has been reported that the number of sporadic angiomyolipomas is four times that of the cases associated with TSC (3,4).…”

Associação entre hemimegalencefalia e esclerose tuberosa em pacientes com epilepsia

“…Além disso, o aumento contínuo do tamanho do AMLR pode levar à compressão do tecido renal remanescente, aumentando o risco de desenvolvimento de doença renal crônica (REDKAR et al, 2012).Alguns casos relatados ilustram o potencial de hemorragia devido à ruptura espontânea do AMLR que ameaça a vida de pacientes com ET. A hemorragia decorre do crescimento do tumor, do aumento do suprimento de sangue, que pode resultar em angiectasias e incremento de aneurismas arteriais(PAREKH et al, 2014). Como consequência, as complicações renais são a principal causa de morte em adultos comET (KINGSWOOD et al, 2017;SPARKS et al, 2011) DE PAUW et al, 2003)…”