It can affect the
vulva[1] and other parts of the
pelvis. The characteristic feature of this tumor is its frequent local recurrence and it is currently regarded as a
non-metastasizing benign tumor.[2]
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Prognosis
Although it is a
benign tumour and does not invade neighbouring tissues, it has a tendency to recur after surgical excision so it is termed "aggressive". Recurrence can occur as early as six months from initial resection. Patients frequently present at tertiary medical centers with a history of
labial mass (sometimes misdiagnosed as
Gartner's cyst), with multiple surgical excisions from several surgeons. There is no standard medical therapy; agents reported to be effective in case reports include systemic hormonal therapy with
SERMs such as
tamoxifen or
LHRH agonists (
leuprolide), and
cytotoxic ("traditional")
chemotherapy, as well as
radiation therapy especially for recurrent disease.
History
Aggressive angiomyxoma was originally described in 1983,[8] but the term angiomyxoma dates back to at least 1952.[9]
^Medeiros, F; Erickson-Johnson, M. R.; Keeney, G. L.; Clayton, A. C.; Nascimento, A. G.; Wang, X; Oliveira, A. M. (2007). "Frequency and characterization of HMGA2 and HMGA1 rearrangements in mesenchymal tumors of the lower genital tract". Genes, Chromosomes and Cancer. 46 (11): 981–90.
doi:
10.1002/gcc.20483.
PMID17654722.
^Micci, F; Panagopoulos, I; Bjerkehagen, B; Heim, S (2006). "Deregulation of HMGA2 in an aggressive angiomyxoma with t(11;12)(q23;q15)". Virchows Archiv. 448 (6): 838–42.
doi:
10.1007/s00428-006-0186-5.
PMID16568309.
^
abNucci, M. R.; Weremowicz, S; Neskey, D. M.; Sornberger, K; Tallini, G; Morton, C. C.; Quade, B. J. (2001). "Chromosomal translocation t(8;12) induces aberrant HMGIC expression in aggressive angiomyxoma of the vulva". Genes, Chromosomes and Cancer. 32 (2): 172–6.
doi:
10.1002/gcc.1179.
PMID11550285.