Abdominal Imaging
ª Springer Science+Business Media, Inc. 2005 Published online: 28 November 2005
Abdom Imaging (2006) 31:383–386 DOI: 10.1007/s00261-005-0378-5
Importance of accurate preoperative diagnosis in the management of aggressive angiomyxoma: report of three cases and review of the literature A. Wiser,1 J. Korach,1 W. H. Gotlieb,1 E. Fridman,2 S. Apter,3 G. Ben-Baruch1 1
Gynecology Oncology Department, Division of Obstetrics and Gynecology, Chaim Sheba Medical Center, Tel Hashomer, and Sackler School of Medicine, Tel Aviv University, 52621 Tel Hashomer, Israel 2 Department of Pathology, Chaim Sheba Medical Center, Tel Hashomer, and Sackler School of Medicine, Tel Aviv University, 52621 Tel Hashomer, Israel 3 Department of Diagnostic Imaging, Chaim Sheba Medical Center, Tel Hashomer, and Sackler School of Medicine, Tel Aviv University, 52621 Tel Hashomer, Israel
Abstract Background: Aggressive angiomyxoma is a benign but locally aggressive tumor that occurs mostly in young women. Because excision is often incomplete, the risk of local recurrence is high. This report describes differences in presentation and the importance of accurate preoperative diagnosis of this rare neoplasm. Methods and results: We describe three cases with different presentations. Two were initially misdiagnosed, and local recurrence necessitated reoperation. Accurate diagnosis in the third case was followed by complete excision, with no recurrence. Conclusion: Aggressive angiomyxoma should be considered in the differential diagnosis of young women who present with a well-defined mass in the pelvic tissue. Accurate preoperative diagnosis should alert the surgeon to the need for wide excision, which is essential for prevention of local recurrence. Key words: Aggressive angiomyxoma—CT scan—MRI
Aggressive angiomyxoma is a rare benign tumor that arises from the connective tissue of the perineum or lower pelvis. It was first described in 1983 by Steeper and Rosai [1] as a rare neoplasm with characteristic clinical, gross, and microscopic features in young women. However, it can present with a variety of clinical signs. Its Correspondence to: G. Ben-Baruch; email:
[email protected]
presentation simulates that of a Bartholin gland cyst, vaginal cyst, or hernia, which is often erroneously diagnosed in these cases. One hundred cases have been reported in the literature. About 90% of patients are women, usually of reproductive age [2, 3]. A few cases have been described in males, typically in sites within the genital tract [4]. The tumor presents as a large multilobular or polypoid mass or swelling. On gross examination it is rubbery and white or soft and gelatinous. The tumor grows slowly, and its benign nature is suggested by the histology and by the fact that it shows no tendency to metastasize [5]. However, it is locally aggressive and tends to recur [1, 6]. The literature contains only one report of metastases to the lung and mediastinum, described in a 63-year-old woman with a pelvic tumor [7]. The microscopic differential diagnosis of aggressive angiomyxoma includes neurofibroma, intramuscular myxoma, angiomyofibroblastoma, myxoid smooth muscle tumor, spindle-cell lipoma, myxoid malignant fibrous histiocytoma, myxoid liposarcoma, and embryonal rhabdomyosarcoma. Most patients present a similar microscopic picture of a hypocellular tumor composed of stellate and spindleshaped mesenchymal cells, embedded in a loose myxoid stroma with a few collagen fibers, and lacking necrosis or mitotic figures. The cells are small and bland and lack nuclear atypia. Invasion of skeletal muscle and fat can be seen, and small nerves are trapped within the neoplasm. Small or medium-size veins and arteries within the tumor are often grouped together, some of them displaying
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Fig. 1. Case 2. MRI findings. Coronal T2-weighted fast spinecho (repetition time 5620 ms, echo time 35.472 ms) image shows a high intensity perineal (arrow in A) and intrapelvic (arrows in B) tumor. The mass is hyperintense relative to muscle with swirled strands of lower signal intensities. Axial T1-weighted images (repetition time 540 ms, echo time 9 ms)
before (C) and after (D) intravenous administration of gadolinium. The mass is isointense to muscle, extends into the adjacent fat, and displaces the anal canal (arrow in C) and shows marked enhancement that accentuates swirling within the mass (arrow in D).
medial hypertrophy. Blood vessels are often widely dilated, varying in caliber and usually larger than 10 mm in diameter. Extravasation of red blood cells is common. Immunohistochemically, the tumor cells are focally positive for desmin, smooth muscle actin, muscle-specific actin, and vimentin. CD34, estrogen, and progesterone receptors are frequently present. Between 1997 and 2003, three women diagnosed with aggressive angiomyxoma were hospitalized in our ward. This report describes differences in presentation, diagnostic difficulties, and the importance of accurate preoperative diagnosis of this rare neoplasm.
5.8-cm mass with no clear borders was found by physical examination. Computed tomography (CT) demonstrated extension of the mass into the ischiorectal fossa and penetration of the crus of the clitoris and the obturator muscle. At operation the mass was completely removed. She remained free of recurrence during 4 years of followup. During that period she gave birth to her second child.
Case reports Case 1 A 25-year-old generally healthy woman, gravida 1 para 1, presented with swelling on the left labia majora. Physical examination revealed a mass 5.3 cm in diameter, which was not sensitive to touch. It was diagnosed as a Bartholin abscess and treated with antibiotics but did not improve. One year later she underwent surgery for removal of a persistent cyst on the left labia majora. A 7.6-cm gray elastic spongy tissue was excised and diagnosed postoperatively as aggressive angiomyxoma. Two months later surgical treatment was again needed. A soft
Case 2 A generally healthy 40-year-old woman, gravida 5 para 5, presented 1 year after the diagnosis and removal of an aggressive angiomyxoma of the right buttock in another hospital. One year later she presented to our clinic with a recurrence. On admission, physical examination revealed a gelatinous mass, 10 cm in diameter, with no clear borders, on her right buttock. Magnetic resonance imaging (MRI) showed a perineal and intrapelvic tumor. The mass was hyperintense on T2weighted images, with a swirled strand of lower signal intensity and isointense relative to muscle on T1weighted images that extended into the adjacent fat and displaced the anal canal. Marked enhancement was seen after intravenous administration of gadolinium that accentuated swirling within the mass. These features are shown in Figure 1.
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At operation, the mass was located to the right of the rectum and the rectovaginal septum, closely adherent to levator muscles reaching up to the obturator fossa, the pelvic floor, and the right parametrium at the level of the cervix. Extensive surgery was performed to ensure complete removal of the tumor (Fig. 2A). The histologic finding of this tumor was a spindle nature of the neoplastic cells and lack of mitotic activity (Fig. 2B), with interdigitation protrusion of tumor into the underlying adipose tissue (Fig. 2C). She is presently without evidence of disease, 1 year after surgery.
Case 3 A 48-year-old woman, gravida 5 para 5, with non– insulin-dependent diabetes mellitus was found to have an asymptomatic 2-cm pedunculated mass in the posterior vaginal fornix during routine gynecologic examination. Removal of the polypoid mass demonstrated aggressive angiomyxoma. To remove the base of the polyp and based on her personal preference following informed consent, she underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and wide resection of part of the posterior wall of the vagina that contained the mass. She has been followed for 4 years, with no recurrence.
Comment Aggressive angiomyxoma is a rare benign tumor that arises from the connective tissue of the perineum or lower pelvis. The reported range of recurrence of aggressive angiomyxoma has decreased in recent years from 67% to 80% [1, 6] to 33% to 56% [8, 9]. The formerly high recurrence rate has been attributed to incomplete surgical excision and was usually reported within 3 years of the first operation [10]. This is similar to the first two patients described in this report, both of whom were misdiagnosed and, as a consequence, whose first excisions were incomplete, with recurrence within 2 months to 1 year. Our third patient, in whom the preoperative diagnosis of aggressive angiomyxoma was correct, underwent a complete excision of the tumor with no recurrence. Preoperative misdiagnosis of aggressive angiomyxoma is not infrequent because this rare neoplasm has no universally typical symptoms. However, a retrospective evaluation of our first patient shows that the clinical manifestation of the vulva tumor is not typical to a Bartholin cyst. When the vulva swelling is not well defined, preoperative CT scan or MRI of the pelvis might raise the suspicion for the tumor. Angiomyxoma appears on cross-sectional imaging as a well-defined mass. On CT the tumor is hypodense relative to the muscle. On T2-weighted MRI, it has high
Fig. 2. Case 2. A Cut surface of a pathologic specimen. The tumor is soft, gelatinous, and encapsulated. B Hematoxylin and eosin, high magnification. The detail highlights the bland, spindled nature of the neoplastic cells and lack of mitotic activity. C Intermediate magnification of the deep edge of this aggressive angiomyxoma. Note the interdigitating protrusions of tumor into the underlying adipose tissue. Such irregular outlines are thought to account for the predisposition of the tumor to recur after excision.
signal intensities. These appearances likely relate to the loose myxoid matrix and high water content of angiomyxoma. The tumor enhances after intravenous contrast
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injection on CT and MRI. A distinctive appearance of swirled strands of lower density on CT and lower signal intensity on MRI within the tumor have also been described [11, 12]. CT and MRI can accurately reveal extension of the tumor from the perineum into the pelvis [11, 12]. In summary, aggressive angiomyxoma should be distinguished from other, more common neoplasms of the pelvic tissues. When the mass is not well defined or has atypical behavior, it seems to us that preoperative CT scan or MRI of the pelvis might raise the suspicion for tumor, improve preparation before surgery, and direct the surgeon toward complete excision. References 1. Steeper TA, Rosai J (1983) Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm. Am J Surg Pathol 7: 463–475 2. Smith HO, Worrell RV, Smith AY, et al. (1991) Aggressive angiomyxoma of female pelvic and perineum: review of the literature. Gynecol Oncol 43:79–85
3. Fishman A, Otey LP, Poindexter AN III, et al. (1995) Aggressive angiomyxoma of the pelvis and peritoneum. A case report. J Reprod Med 40:665–669 4. Kaur A, Makhija PS, Vallikad E, et al. (2000) Multifocal aggressive angiomyxoma: a case report. J Clin Pathol 53:798–799 5. Smirniotis V, Kondi-Pafiti A, Theodoraki K, et al. (1997) Aggressive angiomyxoma of the pelvis: a clinopathologic study of a case. Clin Exp Obstet Gynecol 24:209–211 6. Begin LR, Clement PB, Kirk ME, et al. (1989) Aggressive angiomyxoma of pelvic soft parts: a clinicopathologic study of nine cases. Hum Pathol 16:621–638 7. Siassi RM, Papadopoulos T, Matzel KE (1999) Metastasizing aggressive angiomyxoma. N Engl J Med 341:1772 8. Fetsch JF, Laskin WB, Lefkowitz M, et al. (1996) Aggressive angiomyxoma: a clinopathologic study of 29 female patients. Cancer 78:79–90 9. Skalova A, Michal M, Husek K, et al. (1993) Aggressive angiomyxoma of pelvioperineal region. Immunohistological and ultrastructural study of seven cases. Am J Dermatopathol 15:446–451 10. Chan YM, Hon E, Ngai SW, et al. Aggressive angiomyxoma in female: is radical resection the only option? Acta Obstet Gynecol Scand 79:216–220 11. Outwater EK, Marchetto BE, Wagner BJ, Siegelman ES (1999) Aggressive angiomyxoma: findings on CT and MR imaging. AJR 172:435–438 12. Jeyadevan NN, Sohaib SA, Thomas JM, et al. (2000) Imaging features of aggressive angiomyxoma. Clin Radiol 58:157–162
