1710 | Case Report Aggressive Vaginal Angiomyxoma Mimicking a Bladder Mass Bulent Erol,1 Eyyup Sabri Pelit,1 Sibel Bektaş,2 Aliriza Şimşek3 Corresponding Author: Eyyup Sabri Pelit, MD Dr. Erkin Cad. İstanbul Medeniyet Üni- versitesi, Göztepe Eğitim ve Araştırma Hastanesi, Kadıköy, İstanbul, Turkey. Tel: +90 506 388 3186 E-mail: dreyyupsabri@hotmail.com Received May 2013 Accepted January 2014 1 Department of Urology, Istanbul Medeniyet University, Faculty of Medicine, Istanbul, Turkey. 2 Department of Pathology, Bulent Ecevit University, Faculty of Medicine, Kozlu, Zonguldak, Turkey. 3 Department of Urology, Bulent Ecevit University, Faculty of Medicine, Kozlu, Zonguldak, Turkey. CASE REPORT Keywords: myxoma; vaginal neoplasms; diseases; diagnosis; diagnostic error; urinary blad- der neoplasms; differential. INTRODUCTION Aggressive angiomyxoma is a rare, slow-growing myxoid neoplasm that occurs al-most exclusively in the genital, perineal and pelvic regions of adult women. Be-cause of its rarity and pelvic occurrence, it is often initially misdiagnosed as a gynecological malignancy or mixed with other malignancies. CASE REPORT A 48-year-old female patient presented with a history of abdominal swelling and hyperten- sion. She had also difficulty in urination and defecation. On examination, a palpable large abdominal mass extending to the umbilicus was observed. Ultrasonography revealed intra- vesical mass and right grade IV and left grade II hydronephrosis. The computed tomography (CT) scan showed a large pelvic mass and bilateral hydronephrosis. (Figure 1). The proximal vaginal wall involvement could not be ruled out on CT scan. In CT scan images, a huge in- travesical mass was seen but it did not have the classical appearance of bladder tumor. Under general anesthesia, a cystoscopy was performed initially to confirm intravesical mass. Cysto- scopic examination was very difficult due to the huge size of the mass elevating the base of 1711Vol. 11 | No. 03 | May - June 2014 |U R O LO G Y J O U R N A L Vaginal Angiomyxoma | Erol et al the bladder and there was no papillary bladder tumor. Dou- ble J ureteral stent was inserted to the left side. Right ure- teral orifice was not seen due to compression of huge mass. Subsequently open exploration was planned. During the exploration, mass was thought to stem from posterior wall of bladder. The anterior bladder wall was incised, trans- versely. The mass was located between the posterior wall of the vagina and base of the bladder and originated from the posterior vaginal wall. Transvesical enucleation of mass was made and complete extirpation could be performed af- ter incision of posterior wall of the bladder. Right ureteral orifice was excised simultaneously due to invasion of the orifice. Right ureteroneocystostomy was performed using the Paquin technique (Figure 2). The postoperative period was uneventful. Bilateral hydronephrosis was resolved and according to control CT scan no mass has been recurred on 6-month and 2-year postoperatively (Figure 3). DISCUSSION Aggressive angiomyxoma, synonymously referred to as deep angiomyxoma by the World Health Organization (WHO), was first described in 1983 as “aggressive an- giomyxoma of the female pelvis and perineum”.(1) This neoplasm usually involves the deep soft tissues of the vul- vovaginal region, pelvis and perineum of women in the reproductive age.(2,3) The reason of high prevalence in re- productive age group is thought to be estrogen-dependent nature of the tumor and estrogen and progesterone receptor positivity.(4,5) Our patient is not in reproductive age group. Aggressive angiomyxoma is often a large lesion that fills much of the pelvis, displacing rather than directly invad- ing the pelvic structures.(6) It is a locally invasive neoplasm Figure 1. Large pelvic mass and bilateral hydronephrosis. Figure 2. Mass elevating the base of bladder and intact mucosa (A) with right ureteroneocystostomy (B). 1712 | with a distinct risk of local recurrence, especially in lesions that extend to the other pelvic organs.(7) In this case, pelvic mass was invade to bladder and ureteral orifice. Thus our patient was presented with a difficulty in urination and def- ecation and bilateral hydronephrosis. Local excision with tumor free margin is the standard treat- ment of the angiomyxoma. The excision of these tumors is difficult as they have the same consistency as that of normal connective tissue and therefore have a propensity for local recurrence (36-72%).(8) Hormonal treatment with gonado- tropin releasing hormone (LHRH) agonists, such as goser- elin acetate, might be of value in the management of ag- gressive angiomyxoma, especially in recurrent or residual cases that are not amenable to surgical resection.(9) In this case, excision of pedunculated lesion was complete and the postoperative period was uneventful. Thus there is no need for further treatment with LHRH agonist. For this lesion long-term follow-up is required due to high risk recurrence and minimal risk of metastasis.(10) In 1996, Fetsch and colleagues reported a series of 29 women with pelvic angiomyxoma. In their series, there was no metasta- sis but recurrence has been frequently observed. Eight pa- tients developed recurrent tumor, from 10 months to 7 years after the initial resection and there were no tumor related deaths.(3) In the literature 71% of recurrences have occurred within the first 3 years. There was no correlation between the size of the tumors and the chance of recurrence.(8) In our case, CT imaging is the preferred method for detecting recurrences. The first follow-up imaging was performed via ultrasonography 3 months after the operation and thereafter follow-up CT scan was performed on first and second year postoperative period. There was no recurrence and metasta- sis during the 2-year follow-up. CONCLUSION In conclusion, angiomyxoma is rare, benign and locally aggressive tumor which compresses the adjacent structure with mass affect. With the preoperative imaging methods, it is difficult to predict whether it is a bladder tumor or not. It can mimic a bladder mass in imaging modalities. Thus, it should be noted that this type of pelvic masses may be gynecological origin other than urological origin. Figure 3. Postoperative computed tomography scan showing no mass and hydronephrosis. Case Report REFERENCES 1. Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gyneco- logic soft-tissue neoplasm. Am J Surg Pathol. 1983;7:463-75. 2. Bégin LR, Clement PB, Kirk ME, Jothy S, McCaughey WT, Ferenczy A. Aggressive angiomyxoma of pelvic soft parts: a clinicopathologic study of nine cases. Hum Pathol. 1985;16:621-8. 3. Fetsch JF, Laskin WB, Lefkowitz M, Kindblom LG, Meis-Kindblom JM. Aggressive angiomyxoma: a clinicopathologic study of 29 female patients. Cancer. 1996;78:79-90. 4. Ribaldone R, Piantanida P, Surico D, Boldorini R, Colombo N, Su- rico N. Aggressive angiomyxoma of the vulva. Gynecol Oncol. 2004;95:724-8. 1713Vol. 11 | No. 03 | May - June 2014 |U R O LO G Y J O U R N A L 5. 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