LAUSR

Purpose Aggressive angiomyxoma (AAM) is a rare and often misdiagnosed tumor that is characterized by frequent local recurrences. This study aimed to investigate the clinicopathological characteristics, surgical experiences, and prognosis for aggressive angiomyxoma to improve the accuracy of diagnosis and develop treatment strategies for decreasing recurrence rates. Methods Clinicopathological data and follow-up information for 27 patients with AAM diagnosed and treated at the Shengjing Hospital of China Medical University between January 2006 and October 2019 were retrospectively analyzed. Results The median age at disease onset among 27 patients was 39 years. The male to female ratio was 1:4.4. Painless and slow-growing mass was the most common symptom. Masses occurring in the perineum and pelvic cavity accounted for 81.5% (22/27). All of the 27 patients underwent surgical treatment. Surgical approaches included transperineal and transvaginal resection. Large pelvic masses were treated with combined abdominoperineal surgery. The postoperative recurrence rate was 37%. Kaplan–Meier survival analysis revealed that 5-year progression-free survival (PFS) rate was 64.4% and the median PFS was 132.0 ± 29.6 (95% CI 72.9–190.1). Multivariate Cox proportional analysis found that surgical margin is an independent prognostic factor for PFS ( P  = 0.018). None of the patients experienced distant metastasis. Conclusions Clinical manifestations of AAM are non-specific. Laboratory testing, imaging examinations, and immunohistochemistry are helpful for diagnosis and differential diagnosis. Surgical approach can be determined according to the relationship between the tumor and adjacent organs and infiltration degree. The development of personalized treatment strategies should aim to achieve a complete resection on the premise of preserving the structure and function of important organs to maintain patient quality of life.