AJR:202, June 2014 1171 JOURNAL CLUB: Aggressive Angiomyxomas: A Comprehensive Imaging Review With Clinical and Histopathologic Correlation Venkateswar R. Surabhi 1 Naveen Garg 2 Michael Frumovitz 3 Priya Bhosale 2 Srinivasa R. Prasad 2 Jeanne M. Meis 4 Surabhi VR, Garg N, Frumovitz M, Bhosale P, Prasad SR, Meis JM 1 Department of Radiology, The University of Texas Health Science Center at Houston, 6431 Fannin St, MSB 2.130, Houston, TX 77030. Address correspondence to V. R. Surabhi (venkateswar.r.surabhi@uth.tmc.edu). 2 Department of Radiology, The University of Texas M. D. Anderson Cancer Center, Houston, TX. 3 Department of Gynecologic Oncology and Reproductive Medicine, The University of Texas M. D. Anderson Cancer Center, Houston, TX. 4 Department of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston, TX. Genitourinary Imaging • Original Research This article is available for credit. AJR 2014; 202:1171–1178 0361–803X/14/2026–1171 © American Roentgen Ray Society Keywords: aggressive angiomyxoma, laminated, mesenchymal, MRI, multicompartmental DOI:10.2214/AJR.13.11668 Received August 4, 2013; accepted after revision September 17, 2013. is not appreciated on clinical examination and can often be mistaken for Bartholin or vaginal cyst, lipoma, or perineal hernia until imaging studies are performed [3]. Surgical resection is the mainstay of treatment. Be- cause of the infiltrative nature of aggressive angiomyxomas, adequate excision with a rim of surrounding normal tissue may be diffi- cult to achieve, leading to frequent local re- currences in more than 35% of patients [10]. Estrogen- and progesterone-receptor positive aggressive angiomyxomas tend to respond to therapy with gonadotropin-releasing hor- mone (GnRH) agonists, both preoperatively [10, 11] and after recurrence [12, 13]. Long- term follow-up is warranted in patients be- cause tumor recurrence can occur very late in some cases [14, 15]. We undertook a retrospective study of 16 patients with aggressive angiomyxomas to evaluate the clinical and cross-sectional im- aging characteristics of aggressive angio- A ggressive angiomyxomas are rare mesenchymal tumors that were first described in 1983 by Steeper and Rosai [1]. Aggres- sive angiomyxomas principally affect wom- en of reproductive age with a peak incidence in the fourth to fifth decades of life [2, 3]. Aggressive angiomyxomas usually involve the deep soft tissues of the vulvovaginal re- gion, pelvis, and perineum of women and analogous sites (inguinoscrotal region) in men [2–4]. Aggressive angiomyxomas have also been rarely described in the abdomen [5, 6], kidney [7, 8], and soft tissues of the extremities [9]. Most aggressive angiomyxo- mas present either as a painless mass or as a mass causing local pressure effect [3]. Ag- gressive angiomyxomas are usually large and measure more than 10 cm in size at the time of diagnosis. The frequently large pel- vic component of the aggressive angiomyxo- mas deep in relation to the pelvic diaphragm OBJECTIVE. Aggressive angiomyxomas are rare infiltrative mesenchymal neoplasms that commonly recur locally. The purpose of this study was to conduct a retrospective review of im- aging findings of aggressive angiomyxomas with clinicopathologic correlation in 16 patients. MATERIALS AND METHODS. CT and MRI studies and clinical data of 16 patients with histopathologic evidence of aggressive angiomyxoma who had been referred to our insti- tutions from January 2002 through January 2012 were retrospectively reviewed. The tumors were evaluated with respect to location, morphology, attenuation or signal intensity, and en- hancement characteristics. RESULTS. The most common location was the pelvis and perineum with the mass on ei- ther side of the pelvic diaphragm (12/16, 75%). The characteristic “laminated” appearance was seen in 10 of 12 patients on MRI. Aggressive angiomyxomas showed only mild diffu- sion restriction and mild 18 F-FDG avidity in both of the two patients who underwent DWI and PET/CT, in keeping with histologic low-mitotic activity. Imaging features, such as collateral vessels and fingerlike growth pattern, were seen in seven of 16 (44%) aggressive angiomyxo- mas. Internal cystic degeneration was seen in three of 16 (19%) aggressive angiomyxomas. CONCLUSION. The finding of a large multicompartmental tumor with a characteris- tic internal laminated morphology or extension on either side of the pelvic diaphragm should alert the radiologist to the possible diagnosis of aggressive angiomyxoma. Imaging features, such as large peripheral vessels and cystic degeneration are less common, but presence of these features in the background of laminated morphology should not deter the radiologist from suggesting a diagnosis of aggressive angiomyxoma. 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