Aggressive angiomyxoma

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

Angiomyxoma is a myxoid tumor involving the blood vessels.

It can affect the vulva 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.

Genetics

• HMGA2 rearrangement
  • by translocation t(12;21)(q15;q21.1)
  • by translocation t(11;12)(q23;q15)
  • by translocation t(8;12)(p12;q15)
• t(5;8)(p15;q22)

Pathology

Microscopy

• Vascular appearance of tumor
• 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

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, but the term "angiomyxoma" dates back to at least 1952.

See also

• Myxoma
• Cutaneous myxoma (Superficial angiomyxoma)

External links

Classification