DOI: http://dx.doi.org/10.18203/2320-1770.ijrcog20202357

Aggressive angiomyxoma of the vulva - a rare entity: case report and review of literature

Amrita Gaurav, Poonam Gill, Kavita Khoiwal, Sandipan Chowdhuri, Dhriti Kapoor, Jaya Chaturvedi

Abstract


Aggressive angiomyxoma (AA) is an extremely rare locally invasive mesenchymal tumor with a high risk of recurrence. Till date, only about 350 cases reported worldwide. Because of the rarity it should be considered as differential diagnosis whenever patient present with vulvovaginal growth. The diagnosis is clinched on histopathology. These are hormone-dependent and have estrogen and progesterone receptors. Hence sometimes GnRH agonists are used for ovarian estrogen secretion suppression but long-term use is not advocated due to side effects. A 45-year-old P4 L4 perimenopausal female presented to the GOPD with a 4×4×3 cms pedunculated painless globular mass on right labia majora. On palpation, the globular mass was firm, non-tender and with a smooth surface. Mass was excised and on gross histopathology, cut sections showed white myxoid areas. On microscopy epidermal lined tissue with stellate and spindle-shaped mesenchymal cells was found, embedded in a loose myxoid stroma with few collagen fibers. The cells were small and bland and lacked nuclear atypia. Small to medium-sized blood vessels were present with the thickened wall. Entrapped nerves and adipocytes were also present. No necrosis or mitosis was identified. All these features were suggestive of an aggressive angiomyxoma. Immunohistochemistry markers ER, PR, CD34, desmin, SMA were all positive. Imaging was done to rule out metastatic lesions and wide local excision was done around the stump with laparoscopic bilateral oophorectomy. Aggressive angiomyxoma is a rare disease. In women with asymptomatic growth in the vulvovaginal region, perineum or pelvis, aggressive angiomyxoma should be considered as a differential diagnosis. Ideal treatment is a wide local excision to prevent local recurrences, which are common and a hypoestrogenic milieu is created by either GnRH Agonists or by bilateral oophorectomy due to their hormone-sensitive nature.


Keywords


Angiomyxoma, Hormone dependent, Locally invasive, Recurrent

Full Text:

PDF

References


Romero R, Kalache KD, Kada MM, Jindal SR, Khemani UN. Aggressive angiomyxoma of the vulva: an uncommon entity. Indian Dermatol Online J. 2012;3:128-30.

Qi S, Li B, Pengetal J. Aggressive angiomyxoma of the liver: a case report. Inter J Clin Exper Med. 2015;8:15862-5.

Geng J, Cao B, Wang L. Aggressive angiomyxoma: an unusual presentation. Korean J Radiol. 2012;13:90-3.

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

Kim HM, Pyo JE, Cho NH, Kim NK, Kim M. MR imaging of aggressive angiomyxoma of the female pelvis: case report. J Korean Soc Magnetic Res Med. 2008;12:206-10.

Theofano O, Kim CS, Vitez SF, Gurp JV, Misra N. A case report of aggressive angiomyxoma in pregnancy: do hormones play a role? Case Reports in Obstet Gynecol. 2016:Article ID 6810368.

Dragoumis K, Drevelengas A, Chatzigeorgiou K, Assimakopoulos E, Venizelos I, Togaridou E, et al. Aggressive angiomyxoma of the vulva extending into the pelvis: report of two cases. J Obstet Gynaecol Res. 2005;31:310-3.

Fetsch JF, Laskin WB, Lefkowitz M, Kindblom LG, Meis-Kindblom JM. Aggressive angiomyxoma: a clinicopathologic study of 29 female patients. Cancer. 1996;78:79-90

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.

Haldar K, Martinek IE, Kehoe S. Aggressive angiomyxoma: a case series and literature review. Eur J Surg Oncol. 2010;36(4):335–339.

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.

Outwater EK, Marchetto BE, Wagner BJ, Siegelman ES. Aggressive angiomyxoma: findings on CT and MR imaging. AJR Am J Roentgenol. 1999;172:435-8.

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

Narayama C, Ikeda M, Yasaka M, Sagara YS, Kan-no Y, Hayashi I, et al. Aggressive angiomyxoma of the vulva with No recurrence on a 5-year follow up: a case report. Tokai J Exp Clin Med. 2016;41:42-5.

Schoolmeester JK, Fritchie KJ. Genital soft tissue tumors. J Cutan Pathol. 2015;42:441-51.

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

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

Chan IM, Hon E, Ngai SW, Ng TY, Wong LC. Aggressive angiomyxoma in females: is radical resection the only option? Acta Obstetricia et Gynecologica Scandinavica. 2000;79:216-20.

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. The Am J Surg Pathol. 1983;7:463-75.

Simo M, Zapata C, Esquius J, Domingo J. Aggressive angiomyxoma of the female pelvis and perineum: report of two cases and review of the literature. Br J Obstet Gynecol. 1992;99:925-7.

Han-Geurts IJ, van Geel AN, van Doorn L, M den Bakker, Eggermont AM, Verhoef C. Aggressive angiomyxoma: Multimodality treatments can avoid mutilating surgery. Eur J Surg Oncol. 2006;32:1217‑21.

Srinivasan R, Mohapatra N, Malhotra S, Rao SJ. Aggressive angiomyxoma presenting as a vulval polyp. Indian J Cancer. 2007;44:87-9.

Fine BA, Munoz AK, Litz CE, Gershenson DM. Primary medical management of recurrent aggressive angiomyxoma of the vulva with a gonadotropin-releasing hormone agonist. Gynecol Oncol. 2001;81:120-22.

Rhomberg W, Jasarevic Z, Alton R, Kompatscher P, Beer G, Breitfellner G. Aggressive angiomyxoma: irradiation for recurrent disease. Strahlen Therapieund Onkol. 2000;176:324-6.

Suleiman M, Duc C, Ritz S, Bieri S. Pelvic excision of large aggressive angiomyxoma in a woman: irradiation for recurrent disease. Inter J Gynecol Cancer. 2006;16:356-60.