Giant Deep Aggressive Angiomyxoma of The Vulva:  A Rare Case Report

Udjaja Claudio; Putra Very Great Eka; Septarina Faiza Rizky Aryani

doi:10.15562/bmj.v11i2.3466

Giant Deep Aggressive Angiomyxoma of The Vulva: A Rare Case Report

Udjaja Claudio ,

Putra Very Great Eka ,

Septarina Faiza Rizky Aryani ,

pdf |
DOI: https://doi.org/10.15562/bmj.v11i2.3466 |
Published: 2022-08-08

Abstract

Background: Deep Aggressive Angiomyxoma (DAA) is a rare benign tumor with about 250 cases reported worldwide. It was first reported in 1983. This tumor is commonly found in women of reproductive age, with the highest incidence in the 3rd and 4th decades of life. DAA is most often found in the pelvic, genital, inguinal region, or perineum.

Case Report: Nulligravida woman 52 years old, with enlargement of the vulva that had felt since ± 20 years ago. A mass was found on local examination of the right vulva with a size of ±50x40x10cm, which was supple, mobile, and with no pain. Enlarged inguinal lymphadenopathy was also observed. MRI showed a well-defined lesion at the right vulvar region (which visualized the size of ± AP 14.95 x LL 31.80 x CC 15. 3 cm) with the signal intensity hypointense on T1WI, hyperintense on T2WI and FatSat, restricted on DWI, with no enhancement was seen. A wide excision was conducted, and post-excision measurements showed a right vulvar mass size was 50x42x10cm, with 14.8kg in weight.

Conclusion: DAA is a rare benign tumor that can disturb patients. The nature of the tumor should be evaluated carefully using radiological and pathological approaches. A wide excision can remove the tumor completely, but control and follow-up are mandatory to evaluate the completeness of the procedure and monitor for any relapse.

References

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. Vol. 7, American Journal of Surgical Pathology. 1983. p 463-75
Rani S, Kumar N, Prabhjot P, Kaur SJ, Kundal RK. Aggressive Angiomyxoma of Vagina: A Rare Case Report. Ann Int Med Dent Res. 2017;4(1):256–60.
Mallick S, Samantaray SP, Durgapal P, Dash N. Deep angiomyxoma of retroperitoneum presenting as a perineal lump in a young woman. BMJ Case Rep. 2013;1–4.
Chapel ADB, Jennifer A. Vulva , vagina & female urethra Mesenchymal neoplasms Aggressive angiomyxoma. 2021. page. 1–7. Available at: https://www.pathologyoutlines.com/topic/vulvaaggressiveangiomyxoma.html
Haldar K, Martinek IE, Kehoe S. Aggressive angiomyxoma: A case series and literature review. Eur J Surg Oncol [Internet]. 2010;36(4):335–9. Available at: http://dx.doi.org/10.1016/j.ejso.2009.11.006
Sirasagi A, Arakeri S. Deep Aggressive Angiomyxoma of Pelvic Soft Tissue: A Rare Case Report. J Obstet Gynecol India. 2014;64(6):438–9.
Rhomberg W, Jasarevic Z, Alton R, Kompatscher P, Beer G, Breitfellner G. Aggressive angiomyxoma: Irradiation for recurrent disease. Strahlentherapie und Onkol. 2000;176(7):324–6.
Shinohara N, Nonomura K, Ishikawa S, Seki H, Koyanagi T. Medical management of recurrent aggressive angiomyxoma with gonadotropin-releasing hormone agonist. Int J Urol. 2004;11(6):432–5.
Aguilar-Frasco J, Ruben-Castillo C, Rodríguez-Quintero JH, Medina-Franco H. Aggressive angiomyxoma: Giant recurrence successfully treated with wide excision and adjuvant therapy with GnRH analogue. BMJ Case Rep. 2018;11(1):10–2.
Zou R, Xu H, Shi Y, Wang J, Wang S, Zhu L. 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. Arch Gynecol Obstet [Internet]. 2020;302(1):219–29. Available at: https://doi.org/10.1007/s00404-020-05592-5
Kim HM, Pyo JE, Cho NH, Kim NK, Kim M. MR Imaging of Aggressive Angiomyxoma of the Female Pelvis : Case Report. Investig Magn Reson Imaging [Internet]. 2008;206–10. Available at: https://www.i-mri.org/Synapse/Data/PDFData/0040JKSMRM/jksmrm-12-206.pdf
Outwater EK, Marchetto BE, Wagner BJ, Siegelman ES. Aggressive angiomyxoma: findings on CT and MR imaging. Am J Roentgenol [Internet]. Februari 1999;172(2):435–8. Available at: http://www.ajronline.org/doi/10.2214/ajr.172.2.9930798
Surabhi VR, Garg N, Frumovitz M, Bhosale P, Prasad SR, Meis JM. Aggressive Angiomyxomas: A Comprehensive Imaging Review With Clinical and Histopathologic Correlation. Am J Roentgenol [Internet]. Juni 2014;202(6):1171–8. Available at: http://www.ajronline.org/doi/abs/10.2214/AJR.13.11668
Chen H, Zhao H, Xie Y, Jin M. Clinicopathological features and differential diagnosis of aggressive angiomyxoma of the female pelvis. Medicine (Baltimore) [Internet]. Mei 2017;96(20):e6820. Available at: https://journals.lww.com/00005792-201705190-00013
Nucci MR, Fletcher CDM. Vulvar mesenchymal neoplasms and tumor-like conditions [Internet]. Third Edition. Robboy's Pathology of the Female Reproductive Tract. 2009. p 95-109 Available at: http://dx.doi.org/10.1016/B978-0-7020-4497-7.00006-6
McCluggage WG, Patterson A, Maxwell P. Aggressive angiomyxoma of pelvic parts exhibits oestrogen and progesterone receptor positivity. J Clin Pathol. 2000;53(8):603–5.

[1] 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. Vol. 7, American Journal of Surgical Pathology. 1983. p 463-75

[2] Rani S, Kumar N, Prabhjot P, Kaur SJ, Kundal RK. Aggressive Angiomyxoma of Vagina: A Rare Case Report. Ann Int Med Dent Res. 2017;4(1):256–60.

[3] Mallick S, Samantaray SP, Durgapal P, Dash N. Deep angiomyxoma of retroperitoneum presenting as a perineal lump in a young woman. BMJ Case Rep. 2013;1–4.

[4] Chapel ADB, Jennifer A. Vulva , vagina & female urethra Mesenchymal neoplasms Aggressive angiomyxoma. 2021. page. 1–7. Available at: https://www.pathologyoutlines.com/topic/vulvaaggressiveangiomyxoma.html

[5] Haldar K, Martinek IE, Kehoe S. Aggressive angiomyxoma: A case series and literature review. Eur J Surg Oncol [Internet]. 2010;36(4):335–9. Available at: http://dx.doi.org/10.1016/j.ejso.2009.11.006

[6] Sirasagi A, Arakeri S. Deep Aggressive Angiomyxoma of Pelvic Soft Tissue: A Rare Case Report. J Obstet Gynecol India. 2014;64(6):438–9.

[7] Rhomberg W, Jasarevic Z, Alton R, Kompatscher P, Beer G, Breitfellner G. Aggressive angiomyxoma: Irradiation for recurrent disease. Strahlentherapie und Onkol. 2000;176(7):324–6.

[8] Shinohara N, Nonomura K, Ishikawa S, Seki H, Koyanagi T. Medical management of recurrent aggressive angiomyxoma with gonadotropin-releasing hormone agonist. Int J Urol. 2004;11(6):432–5.

[9] Aguilar-Frasco J, Ruben-Castillo C, Rodríguez-Quintero JH, Medina-Franco H. Aggressive angiomyxoma: Giant recurrence successfully treated with wide excision and adjuvant therapy with GnRH analogue. BMJ Case Rep. 2018;11(1):10–2.

[10] Zou R, Xu H, Shi Y, Wang J, Wang S, Zhu L. 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. Arch Gynecol Obstet [Internet]. 2020;302(1):219–29. Available at: https://doi.org/10.1007/s00404-020-05592-5

[11] Kim HM, Pyo JE, Cho NH, Kim NK, Kim M. MR Imaging of Aggressive Angiomyxoma of the Female Pelvis : Case Report. Investig Magn Reson Imaging [Internet]. 2008;206–10. Available at: https://www.i-mri.org/Synapse/Data/PDFData/0040JKSMRM/jksmrm-12-206.pdf

[12] Outwater EK, Marchetto BE, Wagner BJ, Siegelman ES. Aggressive angiomyxoma: findings on CT and MR imaging. Am J Roentgenol [Internet]. Februari 1999;172(2):435–8. Available at: http://www.ajronline.org/doi/10.2214/ajr.172.2.9930798

[13] Surabhi VR, Garg N, Frumovitz M, Bhosale P, Prasad SR, Meis JM. Aggressive Angiomyxomas: A Comprehensive Imaging Review With Clinical and Histopathologic Correlation. Am J Roentgenol [Internet]. Juni 2014;202(6):1171–8. Available at: http://www.ajronline.org/doi/abs/10.2214/AJR.13.11668

[14] Chen H, Zhao H, Xie Y, Jin M. Clinicopathological features and differential diagnosis of aggressive angiomyxoma of the female pelvis. Medicine (Baltimore) [Internet]. Mei 2017;96(20):e6820. Available at: https://journals.lww.com/00005792-201705190-00013

[15] Nucci MR, Fletcher CDM. Vulvar mesenchymal neoplasms and tumor-like conditions [Internet]. Third Edition. Robboy's Pathology of the Female Reproductive Tract. 2009. p 95-109 Available at: http://dx.doi.org/10.1016/B978-0-7020-4497-7.00006-6

[16] McCluggage WG, Patterson A, Maxwell P. Aggressive angiomyxoma of pelvic parts exhibits oestrogen and progesterone receptor positivity. J Clin Pathol. 2000;53(8):603–5.

Giant Deep Aggressive Angiomyxoma of The Vulva: A Rare Case Report

Abstract

References

How to Cite

Search Panel