 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

 myxomaID5203.html myxomaID5203.html  Haldar K, et al., Aggressive angiomyxoma: A case series and literature review, Eur J Surg Oncol (2009), doi: /j.ejso  Chunyanca et al. World J Surg Oncol. 2006; 4: 60. Published online 2006 September 1. doi: /  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 Sep;9(5):723-5.