Aggressive angiomyxoma explained

Angiomyxoma
Field:vascular system

Angiomyxoma is a myxoid tumor involving the blood vessels.

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]

Genetics

Pathology

Microscopy

Immunochemistry

Immunohistochemical studies show strong staining for desmin, estrogen receptors, and progesterone receptors. Staining for actin, CD34 and smooth muscle actin are intermediate. Staining for S-100 protein is negative.

Diagnosis

Differential diagnosis

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]

See also

Notes and References

  1. Mandal S, Dhingra K, Roy S, Khurana N . Aggressive angiomyxoma of the vulva presenting as a pedunculated swelling . Indian J Pathol Microbiol . 51 . 2 . 259–60 . 2008 . 18603701 . 10.4103/0377-4929.41677. free .
  2. Mathieson A, Chandrakanth S, Yousef G, Wadden P . Aggressive angiomyxoma of the pelvis: a case report . Can J Surg . 50 . 3 . 228–9 . June 2007 . 17568501 . 2384290 . 2008-08-24 . https://web.archive.org/web/20160805151812/https://www.cma.ca/multimedia/staticContent/HTML/N0/l2/cjs/vol-50/issue-3/pdf/pg228.pdf . 2016-08-05 . dead .
  3. 18295664. 2008. Rawlinson. N. J.. Aggressive angiomyxoma with t(12;21) and HMGA2 rearrangement: Report of a case and review of the literature. Cancer Genetics and Cytogenetics. 181. 2. 119–24. West. W. W.. Nelson. M. Bridge. J. A.. 10.1016/j.cancergencyto.2007.11.008. 2396496.
  4. 17654722. 2007. Medeiros. F. Frequency and characterization of HMGA2 and HMGA1 rearrangements in mesenchymal tumors of the lower genital tract. Genes, Chromosomes and Cancer. 46. 11. 981–90. Erickson-Johnson. M. R.. Keeney. G. L.. Clayton. A. C.. Nascimento. A. G.. Wang. X. Oliveira. A. M.. 10.1002/gcc.20483.
  5. 16568309. 2006. Micci. F. Deregulation of HMGA2 in an aggressive angiomyxoma with t(11;12)(q23;q15). Virchows Archiv. 448. 6. 838–42. Panagopoulos. I. Bjerkehagen. B. Heim. S. 10.1007/s00428-006-0186-5.
  6. 11550285. 2001. Nucci. M. R.. Chromosomal translocation t(8;12) induces aberrant HMGIC expression in aggressive angiomyxoma of the vulva. Genes, Chromosomes and Cancer. 32. 2. 172–6. Weremowicz. S. Neskey. D. M.. Sornberger. K. Tallini. G. Morton. C. C.. Quade. B. J.. 10.1002/gcc.1179.
  7. 22247641. 2012. Geng. J. Aggressive angiomyxoma: An unusual presentation. Korean Journal of Radiology. 13. 1. 90–3. Cao. B. Wang. L. 10.3348/kjr.2012.13.1.90. 3253408.
  8. Adwan H, Patel B, Kamel D, Glazer G . A solitary encapsulated pelvic aggressive angiomyxoma . Ann R Coll Surg Engl . 86 . 6 . W1–3 . November 2004 . 16749950 . 1964274 . 10.1308/14787080465 .
  9. RAEBURN C . The histogenesis of four cases of angiomyxoma of the auricle . J. Clin. Pathol. . 5 . 4 . 339–44 . November 1952 . 13011222 . 1023673 . 10.1136/jcp.5.4.339.

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