A Giant Aggressive Angiomyxoma of the Pelvis Misdiagnosed as Incarcerated Femoral Hernia: A Case Report and Review of the Literature

Abstract

Aggressive angiomyxoma (AA) is an uncommon mesenchymal tumor that is mostly derived from the female pelvic and perineal regions. AA is a locally infiltrative slow growing tumor with a marked tendency to local recurrence. Painless swelling located around the genitofemoral region is the common symptom; thus, it is often misdiagnosed as a gynecological malignancy or a groin hernia. A 35-year-old female patient who previously underwent surgery for left femoral hernia operation resulting in surgical failure was reoperated for a giant AA located in the pelvis. The tumor was completely excised with free margins. Histopathologic examination revealed an AA. The tumor size was measured as 24 × 12 × 6 cm with a weight of 4.2 kg. Immunohistochemically, the cells show positive staining with vimentin, desmin, estrogen, and progesterone receptor. S100, MUC4, CD34, and SMA were negative in the tumor cells. AA should be considered in the differential diagnosis of any painless swelling located in the genitofemoral region, particularly in women of reproductive age. The principle treatment should be complete surgical excision with tumor-free margins. Long-term follow-up and careful monitoring are essential due to its high tendency of local recurrence in spite of wide excision of the tumor. Adjuvant antihormonal therapy yields promising results for preventing recurrence.

Low-grade Fibromyxoid Sarcoma of the Vulva and Vagina

“…Before considering individual entities, we note that MUC4 immunohistochemistry is a valuable adjunct in this broad differential, as it is positive only in LGFMS and a minority of monophasic synovial sarcoma,13,23,24 with the caveat that data on MUC4 staining in superficial angiomyxoma, CA, and angiomyofibroblastoma are not available. In addition, FUS::CREB3L2 fusion is diagnostic of LGFMS in this differential, and molecular testing for FUS (or, rarely, EWSR1 15) rearrangement can be performed in difficult cases 3,6,7…”

Aggressive angiomyxoma: The first case report in skull

“…The tumors vary in size from 1 to 60 cm and are often tan, bulky, and rubbery with a glossy, gel-like surface ( 11 ). Histologically, AAMs consist of a myxoid matrix with mixed collagen fibers and dilated thick-walled vessels ( 19 ). Microscopically, the tumor consists of bland spindle cells, loosely arranged in a myxoid edematous stroma, with vascular proliferation of various sizes ( 20 ).…”

Surgical Resection of Retroperitoneal Aggressive Angiomyxoma: Case Report and Review of the Literature

“…They questioned the benefits of radical surgery, which did not seem to significantly reduce the recurrence rate when compared to R1 resections [ 12 ]. Sozutek, et al presented a case of giant aggressive angiomyxoma of the pelvis misdiagnosed as incarcerated femoral hernia [ 19 ]. Our case had a similar extension of the pelvic tumor through the femoral ring into the femoral area.…”