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Aggressive Angiomyxoma As the Cause of Lower Urinary Tract Symptoms

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Aggressive Angiomyxoma As the Cause of Lower Urinary Tract Symptoms □ Case Report □ Aggressive Angiomyxoma as the Cause of Lower Urinary Tract Symptoms Sang Hyub Lee, Youn Wha Kim1, Sung-Goo Chang Korean Journal of Urology Vol. 50 No. 12: 1258-1261, 1 From the Departments of Urology and Pathology, School of Medicine, Kyung December 2009 Hee University, Seoul, Korea DOI: 10.4111/kju.2009.50.12.1258 Aggressive angiomyxoma (AAM) is a rare, benign tumor. It usually ： involves the connective tissue of the perineal regions in women of Received May 29, 2009 Accepted：November 27, 2009 reproductive age. In this report, we present a case of AAM in a 66-year-old female, which presented itself as a retrovesical tumor on pelvic magnetic Correspondence to: Sung-Goo Chang resonance imaging and caused lower urinary tract symptoms. The tumor Department of Urology, Kyung was resected en bloc and the patient’s voiding symptoms disappeared. Hee University Medical Center, 1, Hoegi-dong, Dongdaemun-gu, (Korean J Urol 2009;50:1258-1261) Seoul 130-702, Korea TEL: 02-958-8533 Key Words: Myxoma, Neoplasms, Pelvic neoplasms FAX: 02-959-6048 E-mail: [email protected] Ⓒ The Korean Urological Association, 2009 Aggressive angiomyxoma (AAM) usually occurs in the geni- ultrasonography (TVUS) and pelvic MRI were performed. tal and perineal area of female patients and most commonly TVUS revealed a 5.1x4.7x3.4 cm sized homogeneous mass that in the third to fifth decades of life [1]. A retrovesical tumor was compressing the urinary bladder (Fig. 1A, B). On the is defined as “a tumor arising from retrovesical tissue excluding pelvic MRI, the tumor was located in the retrovesical space and the pelvic organs such as rectum, bladder, prostate, seminal it was thought to be arising from the retrovesical tissue, such vesicle, vagina or uterus,” and may or may not cause lower as the bladder, vagina, or uterus. However, we could not rule urinary tract symptoms [2]. This report discusses a rare case out that the tumor might have originated from the urethra. On of AAM that caused lower urinary tract symptoms and the T1-weighted image, the tumor was of homogeneous and presented itself as a retrovesical tumor on pelvic magnetic low intensity, similar to the image of the muscles. On the resonance imaging (MRI). T2-weighted image, the mass was heterogeneous and of intermediate intensity (Fig. 1C, D). The tumor was thought to CASE REPORT be a leiomyoma, a rhabdomyosarcoma, or a neurogenic tumor, as suggested by these MRI findings. A 66-year-old female patient underwent abdominal pelvis In the operation field, the tumor was adhered to the posterior ultrasonography (A-P sono) for a routine check. The A-P sono bladder wall. However, it was easily separated from the bladder showed a mass between the urinary bladder and the vagina. For wall and was resected en bloc. The resected tumor was 5.5x4x3 further evaluation of this mass, the patient visited the Depart- cm in size. Grossly, the tumor had a soft, smooth and elastic ment of Urology. She presented with frequency of urination and surface (Fig. 2). The cut surface showed whitish-yellow homo- nocturia of three times per night. Her voiding diary showed that geneous lesions. Microscopically, the mass was made up of her functional bladder capacity was less than 200 ml. Upon rather well-demarcated tumor tissues, which showed low physical examination, her abdomen was soft and movable and cellularity of relatively uniform, small, satellite and spindly there was no tenderness. The mass was palpable through the cells that were set in a loosely collagenous, myxoid matrix with vagina, and it was soft and round. The laboratory findings were scattered vessels of varying caliber. The tumor cells had scant, within normal limits and her tumor markers, such as CA 19-9, pale, eosinophilic cytoplasm with poorly defined borders and AFP, and CEA, were within normal ranges. Transvaginal relatively bland nuclei with open chromatin and a single, small 1258 Sang Hyub Lee, et al：Aggressive Angiomyxoma 1259 Fig. 1. (A, B) Transvaginal ultra- sonography showing a huge tumor compressing the urinary bladder. (C) T1-weighted magnetic reso- nance image (MRI) showing a mass with low signal intensity posterior to the urinary bladder. (D) T2- weighted sagittal MRI showing a mass with intermediate signal inten- sity. DISCUSSION AAM is a rare and benign tumor that involves the connective tissue of the perineal regions in women of reproductive age [1]. Because the patient’s age was 66 years old, this case occurred much later than in an average case. In Korea, several cases of AAM have been reported. However, only one case of AAM after menopause has been reported [3]. AAM was first described by Steeper and Rosai in 1983 [4]. They described nine cases of soft tissue tumor in the female genitalia and named it aggressive angiomyxoma. Since then, Fig. 2. The gross findings are a rather well-circumscribed gray- about 200 cases of this tumor have been reported. Although white mass with a soft, smooth and elastic surface. some male cases have been reported [5], almost all of the reported tumors arose from the perineum in young female nucleoli. Multinucleated giant cells were rarely observed. patients. Only a few cases of AAM have been discovered in Mitotic figures were infrequent. On the basis of these histologic the retrovesical area in older women. findings, this case was diagnosed as AAM (Fig. 3). Immuno- Preoperative diagnosis of tumors in this region is usually histochemically, the tumor cells showed diffuse staining for based on imaging studies, but such tumors seldom get diag- vimentin, desmin, estrogen, and progesterone receptors and nosed as AAM. The MR features of AAMs have been focal staining for actin (Fig. 4). After the surgery, the patient’s described as isointense or low signal intensity on T1-weighted nocturia disappeared and her functional bladder capacity images and high signal intensity on T2-weighted images. The increased to 350 ml. At 1 year of follow-up, the patient was voluted pattern of signal intensity on the T2-weighted images free from recurrence. is reported as a typical finding in AAMs. The high signal on 1260 Korean Journal of Urology vol. 50, 1258-1261, December 2009 Fig. 3. Pathologic findings show dispersed spindle-shaped cells and thick-walled blood vessels with a myxoid stroma [(A) x100, (B) x400]. Fig. 4. Immunohistochemical analyses show positive results for (A) estrogen receptor, (B) desmin, and (C) progesterone receptor. the T2-weighted MR image is likely to be due to the high water T1-weighted images and showed intermediate signal intensity content and myxoid matrix of the tumor [6]. In our case, on the T2-weighted images. however, the tumor did not show a low signal intensity on the AAM is derived from myofibroblasts as a phenotypic variant Sang Hyub Lee, et al：Aggressive Angiomyxoma 1261 of the basic fibroblast with cytoskeletal adaptation appropriate neering Foundation (KOSEF) grant funded by the Korea to situations such as wound healing. The locally invasive Government (MOST) [No. 20090091346]. character and induction of associated neovascularity are not surprising in a neoplasm of this histogenesis [7]. REFERENCES One of the most important findings for AAM are the immunohistochemical analyses. AAM must be histologically 1. Fetsch JF, Laskin WB, Lefkowitz M, Kindblom LG, Meis- differentiated from other benign and malignant myxoid neo- Kindblom JM. Aggressive angiomyxoma: a clinicopathologic study of 29 female patients. Cancer 1996;78:79-90. plasms, such as angiofibroblastoma, cutaneous myxoma, my- 2. Hatano K, Tsujimoto Y, Ichimaru N, Miyagawa Y, Nonomura xoid neurofibroma, myxoid leiomyoma, pelvic fibromatosis, N, Okuyama A. Rare case of aggressive angiomyxoma pre- myxoid liposarcoma, and low-grade myxoid malignant fibrous senting as a retrovesical tumor. Int J Urol 2006;13:1012-4. histiocytoma. For differential diagnosis, the reactivity for 3. Lee CJ, Yun GB, Kim HY, Kim WG, Kim SH, Park ED, et desmin, smooth muscle actin, muscle-specific actin, vimentin, al. Aggressive angiomyxoma of the vagina after menopause. CD34, S100 protein, and estrogen and progestin receptors must Korean J Gynecol Oncol Colposc 2003;14:167-71. 4. Steeper TA, Rosai J. Aggressive angiomyxoma of the female be confirmed [1,8]. Reactivity for estrogen and progesterone pelvis and perineum. Report of nine cases of a distinctive type receptors suggests that this tumor may be a hormone-responsive of gynecologic soft-tissue neoplasm. Am J Surg Pathol 1983;7: neoplasm. 463-75. According to previous studies, local recurrence of this tumor 5. Kim TH, Kim YS, Myung SC, Shin HO, Lee TJ, Yoo SM, is common, with a recurrence rate of 50-70%, even if the whole et al. An aggressive, rapidly increasing angiomyxoma of the scrotum. Korean J Urol 2006;47:553-5. mass is resected en bloc [9]. The tumor was therefore termed 6. Jeyadevan NN, Sohaib SA, Thomas JM, Jeyarajah A, She- “aggressive” angiomyxoma. Most recurrences of AAM have pherd JH, Fisher C. Imaging features of aggressive angio- occurred within the first 3 years of the initial diagnosis [10]. myxoma. Clin Radiol 2003;58:157-62. However, one case of AAM that recurred 14 years after the 7. Smith HO, Worrell RV, Smith AY, Dorin MH, Rosenberg RD, en-bloc resection has been reported, so it is necessary to Bartow SA. Aggressive angiomyxoma of the female pelvis and perineum: review of the literature. Gynecol Oncol 1991;42: maintain long-term follow-up and to carefully monitor with 79-85. imaging studies [8]. In our case, the follow-up period has been 8. Smirniotis V, Kondi-Pafiti A, Theodoraki K, Kostopanagiotou only 1 year, so it is too early to predict future recurrence. G, Liapis A, Kourias E. Aggressive angiomyxoma of the In conclusion, this case demonstrates that an AAM may pelvis: a clinicopathologic study of a case.
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