Huge Aggressive Angiomyxoma: a Case Report and Literature Review

Huge aggressive angiomyxoma

HUGE AGGRESSIVE ANGIOMYXOMA: A CASE REPORT AND LITERATURE REVIEW

Han-Ching Lin, Chia-Chu Liu, Wan-Yi Kang,1 Hung-Lung Ke, Ching-Chia Li, Wen-Jeng Wu, and Chun-Hsiung Huang Departments of Urology and 1Pathology, Kaohsiung Medical University Chung-Ho Memorial Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.

Angiomyxoma occurs mostly in 30- to 40-year-old females and is described histologically as a mesenchymal tumor, composed of fibroblasts within a strong myxoid background. It occurs mainly in the female pelvis, vulva or perineum, and grows slowly. Treatment is surgical excision. Unfortunately, there is a relatively high rate of recurrence because the exact extent of the tumor is difficult for the surgeon to determine. We report a case of aggressive angiomyxoma combined with uterine myoma, and discuss the characteristics of its images. Surgical excision of the tumor was performed, and adjuvant treatment was given for local recurrence.

Key Words: aggressive angiomyxoma, female, diagnosis, treatment (Kaohsiung J Med Sci 2006;22:301–4)

In 1983, Steeper and Rosai described aggressive angio- a solid mass with heteroechogenicity in the pelvic cavity. myxoma, primarily in the female pelvis and perineum, Abdominal computed tomography (CT) showed a huge as a distinctive variant of myxoid tumors with prominent cystic mass in the pelvis and uterine myoma (Figure 1). vascular component [1]. The tumor occurs predominantly Magnetic resonance imaging (MRI) also revealed a huge in the premenopausal female. We present a case of aggres- soft tissue mass (18.5 × 7.8 × 11 cm) in the pelvic cavity, sive angiomyxoma of the pelvis that coexisted with a huge which showed low signal intensity on T1-weighted MRI, uterine myoma. After surgical resection, the patient was high signal on T2-weighted MRI with heterogeneous followed-up in our outpatient department; if local recurrence enhancement after contrast administration (Figure 2). Some occurs, she will be treated with gonadotropin releasing foci showed strong enhancement within the mass. Uterine hormone (GnRH) agonist. A review of the literature is also myoma was also found. The diagnosis of retroperitoneal outlined. liposarcoma or right ovary cystadenocarcinoma was favored by our radiologist and biopsy was recommended. After discussion with the patient, we decided to perform radical CASE REPORT tumor excision due to high malignant potency. During exploratory laparotomy, a huge teardrop-shaped A 39-year-old unmarried, nulliparous woman presented mass was found in the pelvis, with invasion of the levator with a history of nonspecific abdominal symptoms and a ani. Extended tumor excision and myomectomy were right lower abdominal mass for 2 weeks. She came to our performed smoothly. Histologic examination of the gynecologic clinic for help. Abdominal sonography showed specimen showed a myxoid tumor with sparse infiltrates of polymorphic cells clustering around blood vessels. Immunohistochemical studies showed strong staining for Received: December 1, 2005 Accepted: January 25, 2006 estrogen and progesterone receptors (Figure 3). Staining for Address correspondence and reprint requests to: Dr Wen-Jeng Wu, desmin and vimentin was intermediate, and staining for Department of Urology, Kaohsiung Medical University Chung-Ho Memorial Hospital, 100 Tzyou 1st Road, Kaohsiung 807, Taiwan. S-100 protein was negative. The histologic diagnosis was E-mail: [email protected] aggressive angiomyxoma.

After the operation, no stool or urine incontinence was composed of scattered spindle cells and abundant medium- noted. The patient recovered well and returned to normal sized vessels embedded in a myxoid matrix [1], arises from daily activity. Pelvic CT (Figure 4) was performed in our connective tissues of the perineum or lower pelvis, but outpatient department after 6 months, and revealed no rarely arises directly from any pelvic or perineal viscus evidence of local recurrence. If local recurrence does occur, [4,5]. The lesions are characterized by a predominantly adjuvant hormone therapy with GnRH agonist will be given. myxoid pattern, different modes of presentation and locally infiltrative behavior with tendency for multiple recurrences. DISCUSSION Two factors may contribute to the high rate of local recurrence. First, the tumor is not diagnosed before initial Aggressive angiomyxoma is a mesenchymal tumor that surgery, and the extent of the tumor is frequently not per- affects women in > 90% of cases [2,3]. The tumor, which is ceived. Second, the strategic location of the tumor around

Figure 1. Pelvic computed tomography with contrast enhancement Figure 3. Immunohistochemical stains show that the tumor cells are shows a huge soft tissue mass (arrow) with a swirled appearance, immunoreactive for progesterone and estrogen receptors, vimentin and occupying the hemi-pelvis and compressing the bladder. desmin.

Figure 2. Magnetic resonance imaging T2-weighted image shows a Figure 4. Pelvic computed tomography 6 months after operation shows teardrop-shaped mass (star) in the pelvis with heterogeneous enhancement. no evidence of recurrence.

302 Kaohsiung J Med Sci June 2006 • Vol 22 • No 6 Huge aggressive angiomyxoma the urethra, vagina, and sphincter, and the rectum — with also indicates hormonal imbalance. Therefore, follow-up extension above and below the pelvic diaphragm — makes for local recurrence was mandatory for this patient, and complete resection difficult and does not lend itself to ei- adjuvant GnRH agonist will be given if local recurrence ther abdominal or perineal surgical approaches alone. For should occur. these reasons, most recurrences are likely to be related to inadequate resection and residual tumor [6]. Imaging studies of these tumors are important to REFERENCES determine the extent and optimal surgical approach. Outwater et al found that the characteristics of angiomyxoma 1. Steeper TA, Rosai J. Aggressive angiomyxoma of the female has some variation on CT and MRI [7]. On CT, the tumor has pelvis and perineum: report of nine cases of a distinctive type of gynecologic soft tissue neoplasm. Am J Surg Pathol 1983;7: a well-defined margin and attenuation less than that of 463–75. muscle. On T2-weighted MRI, the tumor has high signal 2. Smith HO, Worrell RV, Smith AY, et al. Aggressive intensity. The attenuation on CT and high signal intensity angiomyxoma of the female pelvis and perineum: review of on MRI are likely to be related to the loose myxoid matrix the literature. Gynecol Oncol 1991;42:79–85. and high water content of angiomyxoma [7]. Similarly, we 3. Fetsch JF, Laskin WB, Lefkowitz M, et al. Aggressive also found this tumor to have a swirled appearance on angiomyxoma: a clinicopathologic study of 29 female patients. Cancer 1996;78:79–90. enhanced CT (Figure 1). CT and MRI can accurately reveal 4. Amr SS, el-Mallah KO. Aggressive angiomyxoma of the vagina. whether a tumor transverses the pelvic diaphragm. This Int J Gynaecol Obstet 1995;48:207–10. determination is critical to choose a surgical approach: 5. Yaghoobian J, Zinn D, Ramanathan K, et al. Ultrasound and perineal or abdominal or both. computed tomographic findings in aggressive angiomyxoma The tumor occurs predominantly in the premenopausal of the uterine cervix. J Ultrasound Med 1987;6:209–12. female, suggesting that estrogen may stimulate its growth. 6. Mascarenhas L, Knaggs J, Clark J, et al. Aggressive angiomyxoma of the female pelvis and perineum: case report and literature This concept is supported by a previous case report of review. Am J Obstet Gynecol 1993;169:555–6. rapid growth of an angiomyxoma during pregnancy [8], a 7. Outwater EK, Marchetto BE, Wagner BJ, Siegelman ES. state of increased estrogen and progesterone production. Aggressive angiomyxoma: findings on CT and MR imaging. The success of adjuvant therapy with a GnRH agonist AJR Am J Roentgenol 1999;172:435–8. following complete surgical excision of an angiomyxoma 8. Htwe M, Deppisch LM, Saint-Julien JS. Hormone-dependent, [9] gave us another choice if local recurrence should occur. aggressive angiomyxoma of the vulva. Obstet Gynecol 1995;86: 697–9. The patient’s aggressive angiomyxoma is clearly a dramatic 9. Brooks SE, Balidimos I, Reuter KL, et al. Aggressive example of an estrogen and progesterone receptor-positive angiomyxoma of the vulva: impact of GnRH agonist. Medscape tumor. Uterine myoma existing in this nulliparous female Womens Health 1998;3:1–5.

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