Aggressive angiomyxoma of vulva: a rare and interesting entity


  • Suman Kharkwal Department of Surgical Oncology, ESIC Model Hospital & PGIMSR, Basaidarapur, New Delhi, Delhi, India
  • Sushanta Sarkar Department of Surgical Oncology, ESIC Model Hospital & PGIMSR, Basaidarapur, New Delhi, Delhi, India
  • Keerthana G. Department of Surgical Oncology, ESIC Model Hospital & PGIMSR, Basaidarapur, New Delhi, Delhi, India



Angiomyxoma, Vulva, Aggressive, Recurrent


We report a case of a 44-year-old female with aggressive angiomyxoma arising from right labia majora. The patient presented with a gradually enlarging vulvar mass and was treatment included wide local excision of the mass with R0 resection. Pre operative and final histopathology was consistent with features of aggressive angiomyxoma. Angiomyxoma is a rare, benign, locally infiltrative mesenchymal tumor that occurs in females at reproductive age. Repeated recurrence and locally invasive characteristics of the tumor makes it important to be considered as a part of the differential diagnosis especially in cases of pelvic mass in females.


Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm. Am J Surg Pathol. 1983;7(5):463-75.

Fletcher CDM, Unni KK, Mertens F. World Health Organization Classifification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press: Lyon. 2022;189-90.

Sutton BJ, Laudadio J. Aggressive angiomyxoma. Arch Pathol Lab Med. 2012;136(2):217-21.

Varras M, Akrivis C, Lekkou P, Kitsiou E, Demou A, et al. Aggressive angiomyxoma of the vulva: our experience of a rare case with review of the literature. Eur J Gynaecol Oncol. 200;7(2):188-92.

Salman MC, Kuzey GM, Dogan NU, Yuce K. Aggressive angiomyxoma of vulva recurring 8 years after initial diagnosis. Arch Gynecol Obstet. 2009;280:485-7.

Nucci MR, Fletcher CD. Vulvovaginal soft tissue tumors: Update and review. Histopathology. 2000;36:97-108.

Chihara Y, Fujimoto K, Takada S, Hirayama A, Cho M, et al. Aggressive angiomyxoma in the scrotum expressing androgen and progesterone receptors. Int J Urol. 2003;10:672-5.

Magtibay PM, Salmon Z, Keeney GL, Podratz KC. Aggressive angiomyx- oma of the female pelvis and perineum: a case series. Int J Gynecol Cancer. 2006;16(1):396-401.

Chan YM, Hon E, Ngai SW, Ng TY, Wong LC. Aggressive angiomyxoma in females: is radical resection the only option? Acta Obstet Gynecol Scand. 2000;79(3):216-20.

Haldar K, Martinek IE, Kehoe S. Aggressive angiomyxoma: a case series and literature review. Eur J Surg Oncol. 2010;36(4):335-9.

Srinivasan S, Krishnan V, Ali SZ. “Swirl sign” of aggressive angiomyxoma-a lesser-known diagnostic sign. Clin Imaging. 2014;38:751-4.

Lee KA, Seo JW, Yoon NR, Lee JW, Kim BG, Bae DS. Aggressive angiomyxoma of the vulva: a case report. Obstet Gynecol Sci. 2014;57:164-7.

Granter SR, Nucci MR, Fletcher CD. Aggressive angiomyxoma: reappraisal of its relationship to angiomyofibroblastoma in a series of 16 cases. Histopathology. 1997;30(1):3-10.

Wang YF, Qian HL, Jin HM. Local recurrent vaginal aggressive angiomyxoma misdiagnosed as cellular angiomyofifibroblastoma: a case report. Exp Ther Med. 2006;11(5):1893-5.

Sun Y, Zhu L, Chang X, Chen J, Lang J. Clinopathological Features and Treatment analysis of rare aggressive angiomyxoma of the female pelvis and perineum - A retrospective study. Pathol Oncol Res. 2017;23:131-7.

McCluggage WG, Connolly L, McBride HA. HMGA2 is a sensitive but not specific immunohistochemical marker of vulvovaginal aggressive angiomyxoma. Am J Surg Pathol. 2010;34(7):1037-42.

Rabban JT, Dal Cin P, Oliva E. HMGA2 rearrangement in a case of vulvar aggressive angiomyxoma. Int J Gynecol Pathol. 2006;25(4):403-7.

Lee MY, Da Silva B, Ramirez DC, Maki RG. Novel HMGA2-YAP1 fusion gene in aggressive angiomyxoma. BMJ Case Rep. 2019;28;12(5).

Chen H, Zhao H, Xie Y, Jin M. Clinicopathological features and differential diagnosis of aggressive angiomyxoma of the female pelvis: 5 case reports and literature review. Medicine (Baltimore). 2017;96(20):e6820.

Orfanelli T, Kim CS, Vitez SF, Van Gurp J, Misra N. A case report of aggressive angiomyxoma in pregnancy: do hormones play a role? Case Rep Obstet Gynecol. 2016;6810368.

Blandamura S, Cruz J, Faure Vergara L, Machado Puerto I, Ninfo V. Aggressive angiomyxoma: a second case of metastasis with patient's death. Hum Pathol. 2003;34(10):1072-4.






Case Reports