Ileal angiomyolipoma manifested by small intestinal intussusception

Abstract

Angiomyolipomas (AMLs), a form of benign mesenchymal hamartoma, arise primarily in the kidneys of patients with or without tuberous sclerosis. Extra-renal AMLs are very rare and are most commonly found in the liver. AMLs of the small intestine are exceedingly rare. Here, a case of a 28-year-old man, who presented with ileal intussusception caused by ileal AML is reported. The clinicopathological and immunohistochemical findings of ileal AMLs are discussed and the literature on small intestinal AMLs is reviewed.

Figure 1

Enhanced abdominal CT and photograph of the pathological specimen. A: A soft tissue mass with fatty-tissue attenuation in the terminal ileum, and with intussusception is shown (arrow); B: Grossly, an approximate 3 cm × 3 cm × 2.5 cm sized polypoid mass was located in the ileum, 60 cm from the ileocecal valve (arrow).

Figure 2

Photomicrograph of the histopathological specimen. A: The specimen was composed of three tissue components: mature adipose tissue (arrowhead) and smooth muscle surrounding thick-walled, medium-sized, vascular channels (arrow) (HE, × 40); B: Immunohistochemistry (IHC) staining showed that the proliferating smooth muscle cells were positive for α-SMA (IHC, × 40); C: Smooth muscle cells showed immunoreactivity for desmin (IHC, × 40); D: The scattered small blood vessels showed immunoreactivity for CD34 (IHC, × 40).