Angiomyxoma
Synonyms: Aggressive angiomyxoma
Unique to the soft tissues of the lower genital tract, pelvis and perineum
Quick Facts
Behaviour
Intermediate
Category
Soft tissue
Synonyms
Aggressive angiomyxoma
Category
Soft tissue
Behaviour
Intermediate
Gender
Female (5:1)
Tissue of Origin
Uncertain
Epidemiology
- Unique to the soft tissues of the lower genital tract, pelvis and perineum
- Age range: 14 - 77 years (median ~ 40 years)
Clinical Features
- Ill defined, painless, slowly enlarging mass
- Symptoms of pelvic fullness or frequent urination
- Can clinically resemble a Bartholin cyst, Gartner duct cyst, lipoma or hernia
- May grow rapidly in pregnancy
Location
- Vulva
- Perineum
- Deep pelvic tissues
- Retroperitoneum
- Penis
Imaging
USS: local infiltration and rich vasculature
Pathology
Hypocellular lesion with myxoid stroma and abundant vasculature, infiltrating fibroadipose tissue
Treatment
Wide local excision with clear margins
Prognosis
- Positive margins increase recurrence risk
- Approximately 30% local recurrence
- Distant metastasis is exceptionally rare
Key Points
- Unique to the soft tissues of the lower genital tract, pelvis and perineum
- Local recurrence in ~ 30% but distant metastasis and death from disease are exceptionally rare
- Clinical follow-up recommended
Workup - Blood Tests
No blood tests required
Workup - Local Imaging
MRI with contrast of primary site
Workup - Biopsy
Core needle biopsy or excisional biopsy
Workup - Staging
CT chest - metastasis rare but should be excluded at staging
Workup - Other
MDT review with sarcoma specialist centre
Follow-up Summary
Soft Tissue Tumours - Localised Extremity - Low Grade
Routine MRI of primary site not indicated unless difficult to assess clinically
Medical disclaimer
The content on Sarcopedia is for educational and informational purposes only and does not constitute medical advice. It is not intended to be a substitute for professional medical diagnosis or treatment. Always consult with a qualified physician regarding any health concerns or before starting any new treatment. Reliance on any information provided on this site is solely at your own risk.