Aggressive pelvic angiomyxoma: clinical and morphological characteristics and optimization of the surgical approach

Background. Aggressive angiomyxoma (AA) is a rare mesenchymal neoplasm that predominantly involves the pelvic and perineal regions in women of reproductive age.
Description of the clinical case. We report the case of a giant 300×160×60 mm AA on the left labia majora in a 41-year-old female patient. The initial differential diagnosis included Bartholin’s gland cyst and inguinal hernia; however, comprehensive examination involving ultrasound, CT, and contrast-enhanced MRI revealed a heterogeneous mass with infltrative growth into the retroperitoneal space. The MRI fndings demonstrated characteristic “layered” changes, and histological examination confrmed the diagnosis: diffuse proliferation of tumor cells within a myxoid stroma, expressing CD34, HMGA2, and estrogen/progesterone receptors. Treatment involved a two-stage surgical procedure: external mobilization of the tumor followed by laparoscopic resection of the retroperitoneal component and pelvic foor defect reconstruction. Histopathological analysis of the resection margins confrmed negative (tumor-free) margins. Lymph node examination revealed no metastases.
Conclusion. MRI is the gold standard for diagnosis of aggressive angiomyxoma, allowing for the assessment of the tumor extent and its relationship with the pelvic foor. The management of aggressive angiomyxoma remains challenging. Radical tumor excision characterized by a high recurrence rate (40–50 %) is shifting toward organ-preserving approaches. Hormonal therapy, combining GnRH agonists with aromatase inhibitors, is an effective strategy to reduce tumor size and recurrence risk. However, this approach is limited by its temporary effect and associated side effects, underscoring the need for a multidisciplinary strategy and further research to standardize treatment and clarify the prognostic role of HMGA2 expression markers for assessing recurrence risks.

1. Maciejczyk A., Bartecki K., Czarnecka A.M., Szumera-Ciećkiewicz A., Rutowski P., Świtaj T. Hormonal treatment of aggressive angiomyxoma. Curr Probl Cancer. 2025; 58: 101223. doi: 10.1016/j.currproblcancer.2025.101223.

2. Aguilar-Frasco J., Ruben-Castillo C., Rodríguez-Quintero J.H., Medina-Franco H. Aggressive angiomyxoma: giant recurrence successfully treated with wide excision and adjuvant therapy with GnRH analogue. BMJ Case Rep. 2018; 11(1): e226973. doi: 10.1136/bcr-2018-226973.

3. Wang Y., Bu X., Liu Y., Xing Y., Tong Q. Characteristics and treatment strategies of aggressive angiomyxoma in women: A retrospective review of 87 cases. Front Surg. 2023; 10: 966971. doi: 10.3389/fsurg.2023.966971.

4. Navitski A., Adams L., Brzezinska B.N., Suhner J., Sliker T., Moideen P., Barrett A., Abualruz A.R., Johnson M.S., Rungruang B. A tale of two vulvar angiomyxomas: Two cases and review of literature. Gynecol Oncol Rep. 2023; 47: 101204. doi: 10.1016/j.gore.2023.101204.

5. Gulino F.A., Gulisano M., Ettore C., Giorlandino A., Russo E., Ettore G. Aggressive Angiomyxoma of the Vulva: Which Is the Best Management Strategy? Description of a Case Report and Review of Literature of the Last Ten Years. J Clin Med. 2023; 12(5): 1726. doi: 10.3390/jcm12051726.

6. Das B.P., Baruah D., Medhi K.B., Talukder B. An aggressive angiomyxoma of vulva – A rare entity – A case report. J Midlife Health. 2016; 7(3): 140–43. doi: 10.4103/0976-7800.191020.

7. Saito A., Komiyama S., Nagashima M., Kugimiya T., Mukai T. Recurrent Aggressive Angiomyxoma That Responded to the GonadotropinReleasing Hormone (GnRH) Antagonist Relugolix. Cureus. 2025; 17(3): e81270. doi: 10.7759/cureus.81270.

8. Kumari S., Verma G.K., Gulati A., Mehta P. Vulval Aggressive Angiomyxoma: A Giant Tumour Arising from Hidradenitis Suppurativa Scars. Indian J Dermatol. 2024; 69(6): 475–77. doi: 10.4103/ijd.ijd_633_23.

9. Zhao C.Y., Su N., Jiang Y.X., Yang M. Application of ultrasound in aggressive angiomyxoma: Eight case reports and review of literature. World J Clin Cases. 2018; 6(14): 811–19. doi: 10.12998/wjcc.v6.i14.811.

10. Peterknecht E., Agerbak E., Mohamedahmed A., Stonelake S., Kulkarni K., Peravali R., Zaman S. Aggressive angiomyxoma of the ischioanal fossa in a post-menopausal woman. Ann R Coll Surg Engl. 2021; 103(2): e59–e64. doi: 10.1308/rcsann.2020.7008.

11. Lin X.M., Wang L., Wang Q. Aggressive angiomyxoma of pelvis: A case report and literature review. Medicine (Baltimore). 2022; 101(46): e31617. doi: 10.1097/MD.0000000000031617.

12. Tuan T.A., Trang B.H., Chau N.T.M., Duy N.Q., Anh N.H., Hung N.D., Luc C., Duc N.M. Imaging features of a rare giant intra-abdominal aggressive angiomyxoma. Radiol Case Rep. 2024; 19(9): 4007–4011. doi: 10.1016/j.radcr.2024.06.041.

13. Goyal L.D., Garg P., Badyal R., Bhalla S. Aggressive (deep) angiomyxoma of the vulva: a case report. J Med Case Rep. 2022; 18; 16(1): 71. doi: 10.1186/s13256-022-03284-z.

14. Sutton B.J., Laudadio J. Aggressive angiomyxoma. Arch Pathol Lab Med. 2012; 136(2): 217–21. doi: 10.5858/arpa.2011-0056-RS.

15. Slukhanchuk E.V., Tyan A.G., Bedzhanyan A.L., Nechitaylo L.M., Dolzhanskiy O.V., Galyan T.N. Angiomyxoma of the pelvis in women. Clinical cases. Obstetrics, Gynecology and Reproduction. 2021; 15(5): 617–26. (in Russian). doi: 10.17749/2313-7347/ob.gyn.rep.2021.234. EDN: RBDTWH.

16. Muskan V., Adhikari P., Thapa B.D., Shrestha R. Vulval Aggressive Angiomyxoma in a 19 year teenager: a case report. BMC Womens Health. 2022; 22(1): 382. doi: 10.1186/s12905-022-01847-2.

17. Yu B.R., Choi W.K., Cho D.H., Lee N.R. Aggressive angiomyxoma of the vagina: A case report and literature review. Medicine (Baltimore). 2025; 104(4): e41287. doi: 10.1097/MD.0000000000041287.