Intravascular leiomyomatosis: an exceptional entity

Area of Science:

• Gynecologic Pathology
• Vascular Medicine
• Surgical Oncology

Background:

• Intravenous leiomyomatosis (IVL) is a rare benign smooth muscle cell proliferation.
• Originating from uterine venous walls or myomas, it commonly affects pelvic vessels.
• Rarely, it can extend into the inferior vena cava and cardiac chambers.

Observation:

• Preoperative diagnosis of IVL is often difficult.
• Suspicion is warranted in women with prior hysterectomy or myomectomy presenting with pelvic or cardiac masses.
• Clinical presentation can mimic malignant conditions, complicating diagnosis.

Findings:

• Surgical management typically involves hysterectomy with bilateral oophorectomy.
• Complete mass or metastasis removal is crucial during surgery.
• Long-term follow-up with imaging (ultrasound, MRI) is essential for monitoring recurrence.

Implications:

• Adjuvant antiestrogenic therapy can aid disease control.
• This is particularly relevant when complete tumor resection or oophorectomy is not feasible.
• Understanding IVL's behavior is critical for effective patient management and prognosis.