Angiomyofibroblastoma and Potential Mimicking Soft Tissue Tumors That May Occasionally Present in the Retroperitoneum: An Approach to the Differential Diagnosis With Report of an Unusual Index Case Abutting the Kidney.

ABSTRACT

The retroperitoneal soft tissues, extending from the pelvic floor to the level of the diaphragm, are the source of a variety of mesenchymal neoplasms with overlapping features and distinct clinical behaviors, making their distinction of crucial importance. Herein, we report a rare retroperitoneal angiomyofibroblastoma (AMFB) that presented as a right abdominal mass in a 25-year-old woman and that clinically simulated a primary renal carcinoma. The patient underwent complete surgical resection showing a well-circumscribed tumor adjacent to but separate from the right kidney. It was comprised of irregular, often anastomosing islands and cords of plump to spindled cells in a collagenous stroma with numerous thin-walled vessels. The tumor cells clustered around the vessels and admixed with moderate numbers of adipocytes. There was neither significant nuclear atypia nor mitotic activity. Immunohistochemically, the tumor cells showed strong reactivity for desmin, diffuse expression of estrogen and progesterone receptors, retained nuclear expression of retinoblastoma protein, and absent CD34 expression. The immunomorphological features were these of a "lipomatous variant" of AMFB of the retroperitoneum. The occurrence of AMFB in the retroperitoneum is unexpected since it mostly develops in the lower genital tract of young women, making its recognition in this rare location difficult. As a consequence, more common pelvic or retroperitoneal soft tissue neoplasms may represent the primary diagnostic considerations. We, therefore, review a variety of soft tissue tumors occurring in the pelvis/retroperitoneum that, to some degree, may mimic AMFB, and present key findings to assist in accurate diagnosis.