Aggressive angiomyxoma

Aggressive angiomyxoma
Micrograph of an aggressive angiomyxoma. Intermediate magnification.
Synonyms
Pronounce	N/A
Specialty	N/A
Symptoms	Pelvic mass, perineal mass, vulvar swelling
Complications	Local recurrence
Onset	Typically in adults
Duration	Long-term
Types	N/A
Causes	Unknown
Risks
Diagnosis	Histopathology
Differential diagnosis	Angiomyofibroblastoma, fibroepithelial stromal polyp, myxoid liposarcoma
Prevention
Treatment	Surgical excision
Medication
Prognosis	Generally good, but high risk of local recurrence
Frequency	Rare
Deaths	N/A

A rare, slow-growing soft tissue tumor

Aggressive angiomyxoma is a rare type of soft tissue tumor that primarily affects the pelvic and perineal regions. It is characterized by its locally aggressive behavior and tendency to recur after surgical removal. Despite its name, aggressive angiomyxoma is generally considered a benign tumor because it does not metastasize to distant sites.

Presentation

Aggressive angiomyxoma typically presents as a slow-growing mass in the pelvis or perineum. It is more common in women than in men, with a higher incidence in women of reproductive age. Patients may experience symptoms such as a palpable mass, discomfort, or pain in the affected area. In some cases, the tumor may cause urinary or bowel symptoms due to its location and size.

Pathology

The tumor is composed of myxoid stroma with scattered blood vessels and spindle-shaped cells. The histological appearance is characterized by a loose, myxoid matrix with stellate and spindle cells. The vascular component is prominent, with numerous thin-walled vessels. Immunohistochemical staining often shows positivity for estrogen and progesterone receptors, which suggests a hormonal influence on tumor growth.

Diagnosis

Diagnosis of aggressive angiomyxoma is typically made through a combination of imaging studies and biopsy. Magnetic resonance imaging (MRI) is the preferred imaging modality, as it provides detailed information about the extent and characteristics of the tumor. A biopsy is necessary to confirm the diagnosis and differentiate it from other soft tissue tumors.

Treatment

The primary treatment for aggressive angiomyxoma is surgical excision. Due to the tumor's infiltrative nature, achieving clear surgical margins can be challenging, and there is a high risk of local recurrence. In some cases, hormonal therapy with agents such as gonadotropin-releasing hormone (GnRH) analogs may be used to reduce tumor size or as an adjunct to surgery. Regular follow-up with imaging is essential to monitor for recurrence.

Prognosis

The prognosis for patients with aggressive angiomyxoma is generally good, as the tumor is benign and does not metastasize. However, the high rate of local recurrence necessitates long-term follow-up. Recurrences can occur many years after the initial treatment, so ongoing surveillance is important.

Related pages

• Soft tissue tumor
• Pelvis
• Perineum
• Myxoid stroma
• Estrogen receptor
• Progesterone receptor