Deep (Aggressive) Angiomyxoma of Vulva: A Case Report
Aggressive angiomyxoma (AAM) is rare, locally aggressive mesenchymal tumor that has a high propensity for local recurrence
that exceeds 35%. It involves mainly the pelvis, vulva, perineum, vagina and urinary bladder in women of the reproductive age
group. It presents clinically mostly as a vulval polyp and simulates a vulvar abscess, bartholin gland cyst, vaginal cyst or an
inguinal hernia. As a result, complete excision is preferable if this can be accomplished without undo morbidity. In instances
where complete removal would be associated with high morbidity or when preservation of fertility is desired, incomplete removal
may be acceptable if the risks of recurrence and additional surgical interventions are understood. All patients require long-term
follow-up. Gross examination usually reveals a large mass, commonly >10 cm and not infrequently >20 cm. AAMs generally
have fairly uniform, low to moderate cellularity, and they contain relatively small, stellate-shaped and spindled cells, set in a
loosely collagenous, myxomatousstroma with scattered vessels of varying caliber and a variety of entrapped regional structures.
We report a case of AAM in a 40-year-old female presenting with a vulval mass on right labia majora.
perineum. Report of nine cases of a distinctive type of gynecologic softtissue
neoplasm. Am J Surg Pathol 1983;7:463-75.
2. Tavassoli FA, Devilee P. Pathology and Genetics: Tumours of the Breast
and Female Genital Organs. World Health Organization Classifi cation of
Tumours. Lyon: IARC Press; 2003. p. 315-29.
3. Haldar K, Martinek IE, Kehoe S. Aggressive angiomyxoma: A case series
and literature review. Eur J Surg Oncol 2009;10:10-6.
4. Magtibay PM, Salmon Z, Keeney GL, Podratz KC. Aggressive
angiomyxoma of the female pelvis and perineum: A case series.
Int J Gynecol Cancer 2006;16:396-401.
5. Chan YM, Hon E, Ngai SW, Ng TY, Wong LC. Aggressive angiomyxoma
in females: is radical resection the only option? Acta Obstet Gynecol
6. 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.
7. Siassi RM, Papadopoulos T, Matzel KE. Metastasizing aggressive
angiomyxoma. N Engl J Med 1999;341:1772.
8. 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;34:1072-4.
9. Cinel L, Taner D, Nabaei SM, Dogan M. Aggressive angiomyxoma of the
vagina. Report of a distinctive type gynecologic soft tissue neoplasm. Acta
Obstet Gynecol Scand 2000;79:232-3.
10. Smith HO, Worrell RV, Smith AY, Dorin MH, Rosenberg RD, Bartow SA.
Aggressive angiomyxoma of the female pelvis and perineum: Review of the
literature. Gynecol Oncol 1991;42:79-85.
11. Mathieson A, Chandrakanth S, Yousef G, Wadden P. Aggressive
angiomyxoma of the pelvis: A case report. Can J Surg 2007;50:228-9.
12. Outwater EK, Marchetto BE, Wagner BJ, Siegelman ES. Aggressive
angiomyxoma: Findings on CT and MR imaging. AJR Am J Roentgenol
13. Güngör T, Zengeroglu S, Kaleli A, Kuzey GM. Aggressive angiomyxoma
of the vulva and vagina. A common problem: Misdiagnosis. Eur J Obstet
Gynecol Reprod Biol 2004;112:114-6.