Retrospective analysis of clinicopathological features and prognosis for aggressive angiomyxoma of 27 cases in a tertiary center: a 14-year survey and related literature review

PurposeAggressive angiomyxoma (AAM) was identified as a distinct clinicopathological entity in 1983. Since then, a few cases of its occurrence in the scrotum have been reported. This case series was performed to increase clinicians' understanding of the clinical features and treatment of AAM in the scrotum.MethodsWe evaluated the clinical presentations, treatments, and follow-up of two patients with AAM in the scrotum in our hospital and 34 cases reported in the literature.ResultsAmong the 36 patients, the average age was 48.3 ± 20.6 years old (range from 1 to 81); the average maximum diameter of the tumor was 8.36 cm (1.6–25 cm); the site of one (2.78%) patient was located in the epididymis, two (5.56%) in the testes, five (13.89%) in the spermatic cord, and 28 (77.77%) in the scrotum. The clinical symptoms were generally non-specific and 20 patients inadvertently discovered their slow-growing painless masses. The treatments for all these patients were surgical excision once the tumor had been found and one case underwent excision followed by radiotherapy. The median follow-up time for the remaining 32 cases was 24.5 months (1 to 84 months). Recurrence occurred in three cases (9.09%) at the primary sites and no cases of distant metastasis.ConclusionAAM of the scrotum can occur in middle-aged and elderly men. The clinical manifestation generally involves a long history of asymptomatic masses or swelling in the scrotum. Ultrasound is the most commonly used diagnostic technique but magnetic resonance imaging may be more effective. The mainly treatment is surgical excision and postoperative histopathological examination is still the gold standard for its diagnosis. Although it is locally aggressive, metastasis is extremely rare in males.

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Aggressive Angiomyxoma in the Scrotum: A Case Series and Literature Review

Sun

Lian

et al. 2022

“…At this time, partial resection is acceptable, but follow-up observation is required. Zhu et al conducted a retrospective analysis of 27 AAM cases and found a recurrence rate of 37%, and in their study found that the surgical margin was an independent factor affecting PFS ( 26 ). Nowadays, hormone therapy, vascular embolization and radiation therapy have gradually started to be applied, which can effectively reduce the recurrence rate of the disease.…”

Section: Discussionmentioning

confidence: 99%

Aggressive angiomyxoma: The first case report in skull

Cao

Liu²,

Liu³

et al. 2022

Aggressive angiomyxoma (AAM) is a rare mesenchymal tumor primarily growing in the soft tissue of the pelvis and perineum in women of reproductive age. It is a benign tumor that still has a probability of being accompanied by localized invasion. Although negative margins of resection are difficult to achieve due to the invasive nature of the tumor and the lack of a well-defined capsule, the first line of treatment for AAM is surgery. The diagnosis of AAM is difficult to make due to a lack of specific manifestations and specific tumor markers. In this study, we reported a case of aggressive angiomyxoma in a 2-year-old girl that rarely develops in the skull with craniocerebral compression. The patient initially had a mass on her head that attracted the attention of her family, and then she began to have episodic headaches. Surgery was performed after hospitalization, and the tumor recurred 1 year after the operation, around the originally affected skull.

“…However, it is not clear whether the recurrence rate is associated with the surgical margin status ( 52 ). In women, the recurrence rates of 71%, 85%, and 94% have been observed within the first 3, 5, and 7 years of local excision, respectively ( 53 ). As shown in Table 1 , 79 of the 85 male patients were followed-up after surgery, and the data revealed only 4 recurrences (4.7%), excluding 2 deaths (one died intraoperatively and one died because of pulmonary tuberculosis).…”

Section: Discussionmentioning

confidence: 99%

Case report and literature review: Rare male aggressive angiomyxoma of the scrotum

Chen

Wei²,

Chen³

et al. 2022

Aggressive angiomyxoma (AAM) is an uncommon locally infiltrative tumor that frequently occurs in the pelvic soft tissues of female patients; it has a high rate of local recurrence. However, AAM is extremely rare in males. Herein, we present the case of a 70-year-old man with a gradually enlarging painless mass in the scrotum. The patient underwent local excision of the scrotal AAM, with no local relapse after 17 months of follow-up. In addition to the present case, the clinicopathological features of males with AAM reported in literature (to the best of our knowledge) are discussed in this report. The literature review revealed that the gross morphology, clinical process, and histopathology of AAM in males resemble those of AAM in females. In particular, estrogen receptor/progesterone receptor has been shown to be expressed in male patients, which may provide an option for hormone therapy. Moreover, in males, a lower recurrence rate has been observed after surgery to remove the tumor. However, more data are needed to validate this observation. This report emphasizes the importance of considering AAM as the differential diagnosis of myxoid neoplasms in male genital areas.