Aggressive angiomyxoma

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Angiomyxoma
Aggressive angiomyxoma - intermed mag.jpg
Micrograph of an aggressive angiomyxoma. Core biopsy. H&E stain.
Specialty Vascular system

Angiomyxoma is a myxoid tumor involving the blood vessels.

Contents

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

Microscopical views

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

Treatment

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

References

  1. ↑ Mandal S, Dhingra K, Roy S, Khurana N (2008). "Aggressive angiomyxoma of the vulva presenting as a pedunculated swelling". Indian J Pathol Microbiol. 51 (2): 259–60. doi: 10.4103/0377-4929.41677 . PMID   18603701.
  2. ↑ Mathieson A, Chandrakanth S, Yousef G, Wadden P (June 2007). "Aggressive angiomyxoma of the pelvis: a case report" (PDF). Can J Surg. 50 (3): 228–9. PMC   2384290 . PMID   17568501. Archived from the original (PDF) on 2016-08-05. Retrieved 2008-08-24.
  3. ↑ Rawlinson, N. J.; West, W. W.; Nelson, M; Bridge, J. A. (2008). "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. doi:10.1016/j.cancergencyto.2007.11.008. PMC   2396496 . PMID   18295664.
  4. ↑ 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. PMID   17654722.
  5. ↑ 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. PMID   16568309.
  6. 1 2 Nucci, 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. PMID   11550285.
  7. ↑ Geng, J; Cao, B; Wang, L (2012). "Aggressive angiomyxoma: An unusual presentation". Korean Journal of Radiology. 13 (1): 90–3. doi:10.3348/kjr.2012.13.1.90. PMC   3253408 . PMID   22247641.
  8. ↑ Adwan H, Patel B, Kamel D, Glazer G (November 2004). "A solitary encapsulated pelvic aggressive angiomyxoma". Ann R Coll Surg Engl. 86 (6): W1–3. doi:10.1308/14787080465 (inactive 27 October 2025). PMC   1964274 . PMID   16749950.{{cite journal}}: CS1 maint: DOI inactive as of October 2025 (link)
  9. ↑ RAEBURN C (November 1952). "The histogenesis of four cases of angiomyxoma of the auricle". J. Clin. Pathol. 5 (4): 339–44. doi:10.1136/jcp.5.4.339. PMC   1023673 . PMID   13011222.
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