Metastatic retroperitoneal epithelioid angiomyolipoma

Abstract

Angiomyolipoma (AML) is a rare benign neoplasm that usually arises in the kidneys, but may rarely originate in sites such as the retroperitoneum, liver and bone. It is characterised by an intimate admixture of blood vessels, smooth muscle and fat. This multiphasic composition allows for its recognition on ultrasound and CT scan examination. Metastases are exceedingly uncommon, and only one other case of metastatic extrarenal AML exists in the literature. Histological variants of AML such as epithelioid angiomyolipoma (EAML) are considered to be locally aggressive. A unique case of an EAML of the retroperitoneum that metastasised to the liver and bone in an 80-year-old woman is described.

The applicability of Ki-67 marker for renal epithelioid angiomyolipoma: experience of ten cases from a single center

“…In the current study, the female/male ratio was 2.3:1 which indicated that women comprised the majority of this disease. On the other hand, angiomyolipomas are detected in approximately 40-80% of patients with TSC and are mostly bilateral and asymptomatic [7]. The age and gender of patients are uneventful.…”

Retroperitoneal Angiomyolipoma: Rare Entity

“…Sixty-nine percent of patients with retroperitoneal ERAMLs presented symptomatically with nonspecific abdominal pain, 13% presented with incidentalomas, and another 13% with abdominal fullness. Retroperitoneal ERAMLs differed widely with respect to size, ranging from 6 cm 3 to 7980 cm 3 and weighing between <1 kg and 11 kgs [1].Additionally, certain ERAMLs variants, most notably the epitheloid variants, are thought to be the most aggressive, suggesting a higher likelihood of metastatic transformation and distant spread [8]. Histologically, angiomyolipomas are described as having three tissue components: convoluted thick walled blood vessels, smooth muscle cells and mature adipose tissue; the proportion of the tissue components is variable, one or the other may prevail, thus three subtypes were described: angiomatous, myomatous, or lipomatous type [2].…”

“…The second case involved an 80-year-old female who developed metastasis to liver and bone one year following surgical resection of a retroperitoneal AML. Although malignant transformation is difficult to predict, high mitotic activity within the primary tumor was a common factor in both metastatic cases [8].…”

Extrarenal Retroperitoneal Angiomyolipoma: A Rare Case

“…The diagnosis of renal AML is established using computed tomography (CT), showing negative fat density [between -10 and -100 Hounsfield unit (HU)] with an accuracy rate of 86% (3,4). In extrarenal AMLs, negative density may not be revealed due to the low amount of fat tissue, which complicates diagnosis (5). In our study, we discussed the rarely encountered case of a retroperitoneal AML.…”