IntermediateSoft tissue
Deep Agressive Angiomyxoma
Synonyms: Aggressive angiomyxoma
Large infiltrative pelvic mass in reproductive-age women
Quick Facts
Behaviour
Intermediate
Category
Soft tissue
Synonyms
Aggressive angiomyxoma
Category
Soft tissue
Behaviour
Intermediate
Gender
Female (6:1)
Tissue of Origin
Myxoid
Epidemiology
- Rare locally aggressive myxoid tumour
- Almost exclusively in women of reproductive age
- Pelvic and perineal region
Clinical Features
- Slowly growing pelvic or perineal mass
- Often very large at presentation
- May cause urinary or bowel symptoms
- Commonly misdiagnosed as Bartholin cyst
Location
- Vulva
- Perineum
- Pelvis
- Vagina
- Scrotum or inguinal region in men
Imaging
- MRI: large myxoid mass with 'swirling' pattern
- Extensive infiltration of pelvic structures
- Characteristic laminated appearance
Pathology
- Hypocellular myxoid stroma with scattered spindle cells
- Prominent thick-walled vessels
- Infiltrative growth without encapsulation
Genetics
- HMGA2 rearrangements
- 12q13-15 region
Treatment
- Wide surgical excision - complete resection challenging due to size and infiltration
- Hormonal therapy (GnRH agonists) may reduce size preoperatively
- High recurrence rate
Prognosis
- No metastatic potential
- High local recurrence rate (up to 70%)
- Long surveillance required
Key Points
- Large infiltrative pelvic mass in reproductive-age women
- Swirling MRI pattern characteristic
- High local recurrence - long-term surveillance essential
- No metastatic potential
Workup - Blood Tests
No blood tests required
Workup - Local Imaging
MRI with contrast of primary site
Workup - Biopsy
Core needle biopsy or incisional biopsy
Workup - Staging
- MRI pelvis
- CT chest/abdomen for large tumours
Follow-up Summary
Medical disclaimer
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