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Aggressive angiomyxoma


Source: http://en.wikipedia.org/wiki/Aggressive_angiomyxoma
Updated: 2017-03-04T23:30Z
Angiomyxoma
Aggressive angiomyxoma - intermed mag.jpg
Micrograph of an aggressive angiomyxoma. Core biopsy. H&E stain.
Classification and external resources
Specialty{{#statements:P1995}}
ICD-O8841/1
Patient UKAggressive angiomyxoma
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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]

History

Aggressive angiomyxoma was originally described in 1983,[3] but the term "angiomyxoma" dates back to at least 1952.[4]

Prognosis

Although it is a benign tumour and does not 'invade' the neighbouring tissue, it has a tendency to recur after surgical excision so it is termed as aggressive. The recurrence can be as close as six months from initial resection. It will not be surprising to find cases coming to tertiary medical centers with history of having a labial mass (sometimes misdiagnosed as gartner's cyst) and have multiple surgical excisions from various doctors. There is no proven medical therapy and people have tried various sorts of chemotherapy like Tamoxifen, Leuprolide and even full blown chemotherapy.

Pathology

Microscopy

  • Vascular appearance of tumor[5]
  • Hypocellular mesenchymal lesion
  • Spindled and stellate cells with an ill-defined cytoplasm
  • Cells loosely scattered in a myxoid stroma
  • No evidence of nuclear atypia and mitosis
  • Numerous, thin-to-thick wall vessels of different sizes
  • Myxoid, hypocellular background
  • Bland cytological appearance of spindle cells

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.

Differential diagnosis

Genetics

  • HMGA2 rearrangement
    • by translocation t(12;21)(q15;q21.1)[6][7]
    • by translocation t(11;12)(q23;q15)[8]
    • by translocation t(8;12)(p12;q15)[9]
  • t(5;8)(p15;q22)[10]

See also

Internal links

External links

Digital slides

Additional images

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. PMID 18603701. doi:10.4103/0377-4929.41677. 
  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 2384290Freely accessible. PMID 17568501. 
  3. ^ 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. PMC 1964274Freely accessible. PMID 16749950. doi:10.1308/14787080465. 
  4. ^ RAEBURN C (November 1952). "The histogenesis of four cases of angiomyxoma of the auricle". J. Clin. Pathol. 5 (4): 339–44. PMC 1023673Freely accessible. PMID 13011222. doi:10.1136/jcp.5.4.339. 
  5. ^ Geng, J; Cao, B; Wang, L (2012). "Aggressive angiomyxoma: An unusual presentation". Korean Journal of Radiology. 13 (1): 90–3. PMC 3253408Freely accessible. PMID 22247641. doi:10.3348/kjr.2012.13.1.90. 
  6. ^ 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. PMC 2396496Freely accessible. PMID 18295664. doi:10.1016/j.cancergencyto.2007.11.008. 
  7. ^ 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. PMID 17654722. doi:10.1002/gcc.20483. 
  8. ^ 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. PMID 16568309. doi:10.1007/s00428-006-0186-5. 
  9. ^ 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. PMID 11550285. doi:10.1002/gcc.1179. 
  10. ^ 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. PMID 11550285. doi:10.1002/gcc.1179. 

External links

a video of an angiomyxoma being excised on YouTube

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