Aggressive angiomyxoma is an uncommon mesenchymal neoplasm characterized by a high recurrence rate, usually observed in the lower genital tract of women during their reproductive age.Seventeen cases of aggressive angiomyxoma confirmed by pathology from January 2007 to December 2021 in Beijing Chao-yang Hospital were included. We collected clinical data and summarized the clinical and immunohistochemical features.All seventeen included patients were females, aged between 23 and 57 years (mean, 37.7 years; median, 42 years). Fourteen patients were newly diagnosed and three were recurrent. The tumors were located in vulva (58.8 %), vagina (23.5 %), buttock (11.8 %), and cervix (5.9 %). The tumors size were 2 to 15 cm in greatest dimension (mean 8 ± 4.4 cm, median 6 cm). Follow-up data was available for nine patients, which ranged from 25 to 124 months (mean, 82 months; median, 80 months). At the end of follow-up, no other recurrence or metastasis was reported. Immunohistochemical analysis showed immunoreactive for estrogen (10/11) and progesterone (8/11) receptor, desmin (6/8), smooth muscle actin (4/10), and vimentin (4/4), S-100 (1/8) and CD34 (1/7). The Ki67 level was less than 5 % in five cases.AAM is a hormone-sensitive, distinct rare mesenchymal neoplasm with high incidence of local recurrence. Surgery is the preferred treatment, with complete resection being an essential prerequisite for minimizing the risk of recurrence.Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.

Nine case of a distinctive soft tissue tumor of the female pelvis and perineum are described. They were characterized by their occurrence in young women (ages 21-38), large size (up to 60 X 20 cm), locally infiltrative nature, and grossly gelatinous appearance. The initial clinical impression was usually that of a Bartholin gland cyst. The microscopic appearance was that of spindle or stellate cells widely separated by a loose myxoid stroma focally rich in collagen fibrils, a prominent vascular component, including many large thick-walled vessels without an arborizing pattern, and foci of proliferating glandular elements in two cases. Mitotic activity was exceedingly low. Ultrastructural study of the spindle cells showed features consistent with myofibroblastic differentiation. Four patients developed large local recurrences; one tumor recurred twice, 14 and 15 years after initial excision. No distant metastases have been documented to date, and all patients are alive and well. The differential diagnosis of this unusual tumor includes myxoma, myxoid liposarcoma, sarcoma botryoides, myxoid variant of malignant fibrous histiocytoma, nerve sheath myxoma, and other soft tissue tumors with secondary myxoid changes. We have chosen the term "aggressive angiomyxoma" for this neoplasm to emphasize the neoplastic nature of the blood vessels and its locally infiltrative and recurrent nature.

To discuss the clinical experiences of aggressive angiomyxoma (AAM) by summarizing clinical information of patients with this disease in China.Chinese articles concerning AAM were retrospectively reviewed and analyzed.A total of 93 cases in 44 well-documented articles had been reported, among which 11 were male and 82 were female, with a male to female ratio of 1:7.45. The age of the females varied from 14 to 55 years (mean [SD], 37.17 [10.39] years; median, 38.5 years), whereas that of the males ranged from 15 to 65 years (mean [SD], 44.75 [17.61] years; median, 55 years), which was significantly higher than that of the females (t = 2.082, P = 0.041). The most common sites were the perineum in females and the scrotum in males. None of the cases could be accurately diagnosed as AAM preoperatively. The mean minimum diameter of the tumors was 4.62 (3.51) cm, and the maximum was 9.12 (8.23) cm. All the specimens showed typical pathological features of AAM as reported previously. Immunohistochemistry indicated that AAM tended to be strongly positive for vimentin, smooth-muscle actin, and CD34 but mostly negative for S-100 and CD68. The duration of the postoperative follow-up was 3 to 96 months (mean [SD], 28.5 [18.86] months; median, 9.5 months) in 44 cases, with a recurrence rate of 31.82%.Aggressive angiomyxoma must be considered in the differential diagnosis of any female with an asymptomatic perineal mass. A complete margin-free excision should be achieved to avoid recurrence. Long-term follow-up is quite necessary because of the high rate of local recurrence.

Aggressive angiomyxoma is a type of mesenchymal tumor occurring predominantly in the pelvic and perineal region. The aim of our study was to reveal our experience with gonadotropin-releasing hormone (GnRH) treatment in patients with angiomyxoma and provide a comprehensive review of management.This study is a case-series including seven female patients diagnosed with aggressive angiomyxoma from a single institution, between 2012 and 2020. Follow-up after surgery was ranged between 2-45 months with an average of 17.6 months. Resection was performed in all patients without any complications, and five had received GnRH analogue (Goserelin acetate) therapy after surgery. Immunohistochemistry analyses showed positivity for smooth muscle actin and desmin in all cases, while both estrogen receptor (ER) and progesterone receptor (PR) positivity were identified in 6 patients. None of the seven patients had recurrence during follow up period.The mean treatment of aggressive angiomyxoma is surgery, and the use of GnHR analogues in cases with positive ER and PR may be effective in preventing recurrence.Aggressive Angiomyxoma, Gonadotropin-Releasing Hormone, Soft Tissue Neoplasm.