Characteristics and treatment strategies of aggressive angiomyxoma in women: A retrospective review of 87 cases

Abstract

ObjectiveAggressive angiomyxoma (AAM) is a rare kind of soft tissue tumor. The purpose of this study is to summarize the clinical manifestations and treatment strategy of AAM in women.MethodWe searched the case reports on AAM in EMBASE, Web of Science and PubMed, China biomedical database, Wanfang database, VIP database, and China National Knowledge Internet from the start of database construction to November 2022 without any language restrictions in place. Then, the obtained case data were extracted, summarized, and analyzed.ResultA total of 74 articles were retrieved involving 87 cases. The age ranges of onset were 2–67 years. The median age at onset was 34 years. The size of the tumor varied greatly among individuals, and about 65.5% of them were asymptomatic. MRI, ultrasound, and needle biopsy were used for diagnosis. Surgery was the primary mode of treatment, but it was prone to relapse. Gonadotropin-releasing hormone agonist (GnRH-a) might be used to reduce the tumor size before the operation and prevent recurrence after the operation. For patients who are unwilling to receive surgical treatment, GnRH-a alone could be attempted.ConclusionDoctors should consider the possibility of AAM in women with genital tumors. For surgery, it must be ensured that the negative surgical margin is recommended and achieved for preventing recurrence, but we should not ignore the impact of the excessive pursuit for a negative margin on the patient’s reproductive function protection and postoperative recovery. Long-term follow-up is necessary regardless of whether patients receive medical treatment or surgical treatment.

Second recurrence of aggressive vulvar angiomyxoma: a case report

“…13 Nevertheless, local recurrence of angiomyxoma after local postoperative radiotherapy was noted in a literature review. 14 Considering the two recurrences of the lesion in the present case, we recommended postoperative radiotherapy to prevent further recurrences; however, this was not performed because the patient declined.…”

Atypical Vaginal Aggressive Angiomyxoma in a Virgin: A Case Report from South Jordan

“…In their study, none of the 24 patients who underwent CT examinations were diagnosed with AA. 9 Despite this finding, the importance of relying on various imaging modalities in assessing the tumor spread into surrounding tissues cannot be overlooked.…”

“…AA generally have a locally invasive and infiltrating growth pattern in the surrounding tissues. 7 , 9 Some sources describe their behavior as displacing adjacent organs rather than truly invading them. 8 However, in our patient, the mass was well-defined and limited to the superficial soft tissue, which is unusual.…”

“…Smooth muscle immunoreactivity (SMA) highlights the presence of myoid bundles, while immunostaining for the S-100 protein was absent. 4 , 11 , 12 Neoplastic cells in AA commonly express estrogen and progesterone receptor positivity, 9 in correlation with its prevalence in women of reproductive age. 13 …”

Imaging features of a rare giant intra-abdominal aggressive angiomyxoma

“…Imaging features of alternating hypointense bands forming layered patterns on T2 weight (T2W) were observed in 83% of cases [ 6 ]. A literature review by Wang and colleagues found that only 7 out of 65 AAM patients were accurately diagnosed initially, accounting for 10.77% of cases [ 7 ]. However, by combining factors of epidemiology, clinical progression, and tumor characteristics on image findings, diagnosing AAM pretreatment is not unachievable.…”