Aggressive Angiomyxoma (AA) is a very rare tumor. It was first described in 1983 and since then only about 250 cases have been reported

Women are affected more than men (1:6.6), typically in the reproductive years

Genetic abnormality in chromosome 12 is found in some AA› 12q15 involving HMGA2 gene

Locations of the tumor are typically in the pelvis and perineum of women› Scrotum and inguinal area for men

Can be found in men and there are case reports of AA mimicking inguinal hernias and hydrocele

Case reports also found with AA in maxillary sinus and the larynx (1each)

No associated risk factors have been identified

These are tumors of myxoid or mesenchymal origin, i.e. connective tissue. They are typically S100 negative.› Often positive IHC for desmin, vimentin, alpha

smooth muscle actin The tumor is soft and does not have discrete

borders. In fact the tumor often has soft finger like projections that extend into surrounding tissue

Appears hypocellular on pathology and is of mesenchymal cell origin. There are bland spindled and stellate cells and a background of myxoid stroma. Blood vessels are prominent

Typically estrogen & progesterone receptor +

No specific or typical features. Often will be an asymptomatic perineal or vulvar mass. Sometimes will be a pelvic mass seen on imaging.

Sometimes can appear cystic and can be confused with Bartholin’s cyst

Very slow growing, often not noticed until the size of the tumor is quite large

Obesity can result in vulvar edema that can be mistaken for AA – although typically bilateral and involves superficial tissues as well

As the tumor is not encapsulated it is difficult to resect. Local recurrence is very common (up to 70% in some series).

Metastasis is very rare. In one case series they report only 2 with metastasis to the lungs.

Can cause obstruction of bowel or of bladder/ureters if advanced peritoneal presentation. Some can develop complete obstruction of the intestinal tract

Typically diagnosis is not made until surgical excision and pathology obtained

Imaging will show some characteristic findings. US shows hypoechoic mass, can appear cystic. MRI shows hypo-intense mass compared to muscle on T1 but hyperintense on T2. CT can be variable, but typically homogenous mass that sometimes has cystic components

Surgical excision – often can be problematic as the tumor is very locally invasive. Goal is complete excision.› One series showed 50% vs 40% pts remained

disease free at 10 years of those who had complete vs incomplete resection

› However generally felt that recurrence is less if 1cm tumor free margins are obtained

Reportedly not life threatening, but two cases of metastasis have been reported and these patients died.

GNRH antagonists have been used with success, although these are case reports, no formal trials have been done

The tumors have very low mitotic activity and slow growth rates, which is why adjuvant therapy with chemo or radiation is not typically pursued.› Only a few case reports of XRT, yielding mixed

results. No cases reporting use of chemotherapy Recurrence occurs within 3 years in 70% of

the cases but can be as late as 14 years F/u is clinical but should also include

imaging studies, preferably MRI

Typically felt to be benign, although as mentioned before very locally invasive

Our patient did have metastatic disease and as such extensive surgical excision was not attempted

Often require repeat surgery to remove recurrent tumor.› Size at diagnosis plays a large role in

prognosis

Pseudomyxoma peritonei – there are two types, benign and malignant. Both are locally aggressive and have many relapses/recurrences.

Lipoma Myxoid sarcoma Intramuscular myxoma Myxoid neurofibroma Angiomyofibroblastoma

http://atlasgeneticsoncology.org/Tumors/AggresAngiomyxomaID5203.html

Haldar K, et al., Aggressive angiomyxoma: A case series and literature review, Eur J Surg Oncol (2009), doi:10.1016/j.ejso.2009.11.006

Chunyanca et al. World J Surg Oncol. 2006; 4: 60. Published online 2006 September 1. doi: 10.1186/1477-7819-4-60.

Fujita Y - J Am Acad Dermatol - 01-FEB-2008; 58(2 Suppl): S40-1

Wu, et al. Scrotal aggressive angiomyxoma mimicking inguinal hernia. Asian J Androl. 2007 Sep;9(5):723-5.