PEComa adjacent to the cecal pole

Case contributed by Dr Domenico Nicoletti

Presentation

Hard swelling of the right iliac fossa.

Patient Data

Age: 30 years
Gender: Female
Ultrasound

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.

Annotated image

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

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.

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Case information

rID: 67292
Published: 27th Mar 2019
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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