Aggressive angiomyxoma

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
* by translocation t
* by translocation t
t

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

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, 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, and cytotoxic chemotherapy, as well as radiation therapy especially for recurrent disease.

History

Aggressive angiomyxoma was originally described in 1983, but the term angiomyxoma dates back to at least 1952.