Angiomyolipoma with caval extension and regional nodal involvement: Aggressive behaviour or just rare natural history? Case report and review of literature

Abstract

Renal angiomyolipoma (AML) is predominantly a non-aggressive benign tumour. Cases of more aggressive AMLs are present in the literature. We present 2 cases of aggressive AML behaviour. The first case is an AML with vascular extension in a young female and the second case is of AML found in regional lymph nodes in a female with a left renal AML and renal cell carcinoma.

Ten uncommon and unusual variants of renal angiomyolipoma (AML): radiologic–pathologic correlation

“…On rare occasions, these neoplasms may show extra-renal or perinephric extension with involvement of lymph nodes. 71,72 Locally aggressive AML have been documented to show extension into venous structures, including the inferior vena cava (IVC) 73 (Fig 16). This locally aggressive type of AML should not be confused with an AML arising within the renal sinus, which can also have an unusual appearance; however, these are not associated with vascular complications.…”

Renal Angiomyolipoma With Caval Extension and Pulmonary Fat Embolism

“…Renal AML is a benign mesenchymal tumor of the kidney and composed adipose tissue, vascular elements, and smooth muscle. 1 , 8 The term AML was first used by Morgan et al in 1951 and the renal lesion that histologically corresponds to a renal AML was first described by Grawitz in 1900. 7 AMLs are most commonly sporadic (80%) and a total of 20% are associated with tuberous sclerosis complex or lymphangioleiomyomatosis.…”

“…Renal angiomyolipoma (AML), a PEComa, is the most common mesenchymal tumor of the kidney comprised of mature adipose tissue, dysmorphic blood vessels, and smooth muscle. 1 , 2 This unusual benign tumor is found in 0.1% to 0.3% of the general population with 2 distinct groups: classic triphasic AML and monotypic epithelioid AML. 1 The mean age at presentation is 43 years, with a female predominance (male/female ratio of 4:11).…”

Angiomiolipoma renal gigante y linfangioleiomiomatosis pulmonar esporádica no filiada. A propósito de un caso

“…Gran volumen tumoral, lo que aumenta el riesgo de ruptura y sangrado espontaneo secundario por un lado; masa que a su vez condiciona restricción mecánica extrínseca de la ventilación, influyendo en la fisiopatología de los síntomas pulmonares de la paciente, y 2. Reporte de algunos casos de comportamiento agresivo y/o atípico 7 , era candidata a nefrectomía izquierda, 24 horas después de embolización selectiva de arteria lobar superior renal, en búsqueda de disminución tanto de volumen tumoral y riesgo de sangrado intraoperatorio [1][2][3]6,8 (fig. 2).…”