Agressive angiomyxoma : Case report

J Clin Exp Invest www.jceionline.org Vol 3, No 3, September 2012 1 Merkezefendi Devlet Hastanesi, Kadın Hastalıkları ve Doğum Kliniği, Manisa, Türkiye 2 Merkezefendi Devlet Hastanesi, Patoloji Laboratuvarı, Manisa, Türkiye Correspondence: Fatma Eskicioğlu, Merkezefendi Devlet Hastanesi, Kadın Hastalıkları ve Doğum Kliniği, Manisa, Türkiye Email: feskicioglu@yahoo.com Received: 19.07.2012, Accepted: 31.07.2012 Copyright © JCEI / Journal of Clinical and Experimental Investigations 2012, All rights reserved JCEI / 2012; 3 (3): 420-422 Journal of Clinical and Experimental Investigations doi: 10.5799/ahinjs.01.2012.03.0193


INTRODUCTION
Aggressive angiomyxoma was described by Steper and Rosai first time in 1983.Aggressive angiomyxoma is a rare mesenchymal neoplasm. 1Aggressive angiomyxoma is a slow-growing locally infiltrative neoplasm and has a high rate of local recurrence. 2t is most often diagnosed in women in reproductive age and detected in external genital, perineum, and pelvis.Aggressive angiomyxoma has low rate of metastasis in distant regions in the body. 2 The ratio of occurrence of aggressive angiomyxoma in women compared to men is 6 to 1. 3 In men, aggressive angiomyxoma is most often detected in inguinoscrotal regions. 4The main treatment method is wide-local excision.Recurrence rates ranging from 30% to 40% after excision in couple have been reported in literature. 5

CASE REPORT
A 44 year-old female patient (G4P2) was admitted with complaint about painless vulvar mass located in right labium majus for the last two years and growing more rapidly in recent months.In gynecological examination, a rubber-soft, mobile, polyploidy mass with 4 cm diameter is observed in the right distal labium majus.This mass had smooth borders.There were no pathological findings in vagina, cervix, uterus and ovaries.Surgical local excision was performed for the initial diagnosis of lipoma.A jelly-like mass (approximately 3x6x2cm) with smooth borders was excised.It is worth mentioning that the lesion infiltrated into the pelvis.Pathological analysis revealed aggressive angiomyxoma.Microscopically, mid-size hyalinized veins with thick walls were observed among spindled cells inside myxoid stroma (Figure1).Around the veins, collections of myoid composed of mainly smooth muscle cells were found.Stromal cells and collections of myoid were stained with desmin and vimentin (Figure 2).Most of the veins and star-like cells were positive with CD34.However when stained with ES100, they were negative.When stained with estrogen and progesterone receptors, they were positive.
Following the surgery, 3.75 mg per month GnRH analogues were administered to the patient for three months.The patient was informed about the risk of recurrence.The patient was controlled for two years and no recurrence was detected.

DISCUSSION
Aggressive angiomyxoma is a rare mesenchymal and benign tumor which was first described by Steeper and Rosai. 1 Aggressive angiomyxoma is often diagnosed in women in the fourth decade of their life. 3Aggressive angiomyxoma, rarely diagnosed in men, is most often detected in their inguinoscrotal regions. 3,4In female patients, it is most often detected in external genital, perineum, and pelvis.Aggressive angiomyxoma, growing slowly, has low rate of the remote metastasis. 2 The presenting symptom of the patients is usually painless and slow-growing mass.Due to its location and appearance, aggressive angiomyxoma is usually misdiagnosed as bartolin cyst, lipoma or vaginal cysts.In previous case reports, the diameters of the neoplasms generally have ranged from 5 to 23 cm. 9 Jelly-like soft masses are found adhered to the pelvic muscle and fat tissues.The most outstanding feature of aggressive angiomyxoma is its ability of local infiltration.Therefore, the treatment modality is wide-local excision.The rate of local recurrence after the surgery is approximately %30-40. 5e methods to establish a final diagnosis are pathological analysis and immunohistochemical stain.Microscopically, these tumors show low to moderate cellularity and are composed of relatively uniform, small, stellate and spindled cells, set in a loosely collagenous, myxoedemayous matrix with scattered vessels of varying caliber and entrapped regional structures.The tumor cells have scant, pale, eosinophilic cytoplasm with poorly defined borders and relatively bland nuclei with an open chromatin pattern and a single, small, centrally located nucleolus.Multinucleated cells rarely are observed.Mitotic figures are infrequent.A characteristic finding that is seen in most cases is the presence of loosely organised island of well-developed myxoid cells around the larger nerve segments and vessels.Tumor cells usually show immunoreactivity for vimentin, desmin, estrogen, progesterone receptor proteins and variable levels of immunreactivity for actin and CD34. 10,11However no immunreactivity is observed in case of ES100 protein.In our case we observed immunreactivity for desmin and CD34, estrogen progesterone receptor proteins.In accordance with the literature, no immunreactivity for ES100 protein was observed.
Angiomyofibroblastoma, a mesenchymal neoplasm with similar clinical, morphological and histopathological, should be considered in the differential diagnosis of the angiomyxoma.While aggressive angiomyxoma invades pelvic soft tissues, angiomyofibroblastoma is a local-restricted lesion residing on the surface of vulva.In contrast to aggressive angiomyxoma, the stroma of angiomyofibroblastoma is lack of hyaluronic acid and contains small and mid-size blood veins.3][14] Thus, simple local excision is enough in case of angiomyofibroblastoma.Angiomyofibroblastoma is more likely to be smaller than 5 cm in diameter.However, there are several aggressive angiomyxoma cases with almost 60 cm long diameter in literature. 12ere are publications suggesting treatment with GnRH agonist after surgery for primary or recurrent cases. 15,16However, there is no consensus about the effective duration of the treatment in reducing recurrence rate after surgery.In our case, 3.75 mg GnRH analog was administered to the patient for three months.
Taken into consideration the present publications, aggressive angiomyxoma should be suspected when polyploidy masses are observed in pelvic and perineal regions.Before surgery, ultrasound or magnetic resonance imaging (MRI) screening could be useful to find out any invasion into the neighboring tissues.Histopatological analysis should be suggested to establish a final diagnosis.After the surgery, clinical follow-up, ultrasound and MR monitoring should be utilized in terms of detection of recurrence.There is insufficient evidence to support utilizing GnRH analogs to prevent recurrence and to accept as a treatment modality for patients, who are not able to be operated.