Aggressive angiomyxoma

"Angiomyxoma" redirects here. For the tumor sometimes called "Angiomyoma", see Angioleiomyoma.

Angiomyxoma
Synonyms
Pronounce
Field	vascular system
Symptoms
Complications
Onset
Duration
Types
Causes
Risks
Diagnosis
Differential diagnosis
Prevention
Treatment
Medication
Prognosis
Frequency
Deaths

Angiomyxoma is a myxoid tumor involving the blood vessels.

It can affect the vulva<ref name="pmid18603701">,

 Aggressive angiomyxoma of the vulva presenting as a pedunculated swelling, 
 Indian J Pathol Microbiol, 
 2008,
 Vol. 51(Issue: 2),
 pp. 259–60,
 DOI: 10.4103/0377-4929.41677,
 PMID: 18603701,</ref> 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.<ref name="pmid17568501">, 
 Aggressive angiomyxoma of the pelvis: a case report, 
 Can J Surg, 
 
 Vol. 50(Issue: 3),
 pp. 228–9,
 
 PMID: 17568501,
 PMC: 2384290,
 
 Full text,
 Accessed on: 2008-08-24.</ref>

Genetics

• HMGA2 rearrangement
  • by translocation t(12;21)(q15;q21.1)<ref>,

 Aggressive angiomyxoma with t(12;21) and HMGA2 rearrangement: Report of a case and review of the literature, 
 Cancer Genetics and Cytogenetics, 
 2008,
 Vol. 181(Issue: 2),
 pp. 119–24,
 DOI: 10.1016/j.cancergencyto.2007.11.008,
 PMID: 18295664,
 PMC: 2396496,</ref><ref>, 
 Frequency and characterization of HMGA2 and HMGA1 rearrangements in mesenchymal tumors of the lower genital tract, 
 Genes, Chromosomes and Cancer, 
 2007,
 Vol. 46(Issue: 11),
 pp. 981–90,
 DOI: 10.1002/gcc.20483,
 PMID: 17654722,</ref>  

• • by translocation t(11;12)(q23;q15)<ref>,

 Deregulation of HMGA2 in an aggressive angiomyxoma with t(11;12)(q23;q15), 
 Virchows Archiv, 
 2006,
 Vol. 448(Issue: 6),
 pp. 838–42,
 DOI: 10.1007/s00428-006-0186-5,
 PMID: 16568309,</ref>

• • by translocation t(8;12)(p12;q15)<ref>,

 Chromosomal translocation t(8;12) induces aberrant HMGIC expression in aggressive angiomyxoma of the vulva, 
 Genes, Chromosomes and Cancer, 
 2001,
 Vol. 32(Issue: 2),
 pp. 172–6,
 DOI: 10.1002/gcc.1179,
 PMID: 11550285,</ref>

• t(5;8)(p15;q22)<ref>,

 Chromosomal translocation t(8;12) induces aberrant HMGIC expression in aggressive angiomyxoma of the vulva, 
 Genes, Chromosomes and Cancer, 
 2001,
 Vol. 32(Issue: 2),
 pp. 172–6,
 DOI: 10.1002/gcc.1179,
 PMID: 11550285,</ref>

Pathology

Microscopy

• Vascular appearance of tumor<ref>,

 Aggressive angiomyxoma: An unusual presentation, 
 Korean Journal of Radiology, 
 2012,
 Vol. 13(Issue: 1),
 pp. 90–3,
 DOI: 10.3348/kjr.2012.13.1.90,
 PMID: 22247641,
 PMC: 3253408,</ref>

• 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.

Diagnosis

Differential diagnosis

• myxoid tumors
  • Angiomyofibroblastoma

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.

History

Aggressive angiomyxoma was originally described in 1983,<ref name="pmid16749950">,

 A solitary encapsulated pelvic aggressive angiomyxoma, 
 Ann R Coll Surg Engl, 
 
 Vol. 86(Issue: 6),
 pp. W1–3,
 DOI: 10.1308/14787080465,
 PMID: 16749950,
 PMC: 1964274,</ref> but the term "angiomyxoma" dates back to at least 1952.<ref name="pmid13011222">RAEBURN C, 
 The histogenesis of four cases of angiomyxoma of the auricle, 
 J. Clin. Pathol., 
 
 Vol. 5(Issue: 4),
 pp. 339–44,
 DOI: 10.1136/jcp.5.4.339,
 PMID: 13011222,
 PMC: 1023673,
 
 Full text,</ref>

See also

• Myxoma
• Cutaneous myxoma (Superficial angiomyxoma)