Presentation
Hard swelling of the right iliac fossa.
Patient Data
Vascularized hypoechoic mass in the right iliac fossa.
In the right iliac fossa, there is a hyperdense oval solid mass, adherent to the cecum and the mesocolon. This lesion is vascularized and is inhomogeneous due to the presence of fluid. Around it is a small amount of free fluid.
The mass shows enhancement and restriction of diffusion. It is heterogeneous, with tortuous and dilated vessels near the lower portion, arising from branches of the superior mesenteric vein. It is not separable from the anterior wall of the cecal fundus.
The mass shows enhancement and restriction of diffusion. It is heterogeneous, with tortuous and dilated vessels near the lower portion, arising from branches of the superior mesenteric vein. It is not separable from the anterior wall of the cecal fundus.
Pathology analysis shows a pecoma or multifocal angiomyolipoma.
Case Discussion
Angiomyolipoma and PEComa are rare tumors arising from perivascular epithelial cells (PECs), with distinctive morphological features.
The kidney is the most frequent site of origin.
Angiomyolipomas are mesenchymal tumors made up of abnormal blood vessels, spindle cells, and mature adipocytes, believed to be derived from perivascular epithelioid cells (PEC). PEComas are a family of mesenchymal tumors arising from PECs, including angiomyolipomas, as well as pulmonary lymphangioleiomyomatosis.