Intravenous leiomyomatosis: Case series and review of the literature

Abstract

Introduction Intravenous leiomyomatosis (ILV) is a rare pathology, part of leiomyoma beyond the uterus (LBU), characterized by benign smooth muscle cell tumor outside of the uterus and mainly affecting premenopausal woman with a medical history of leiomyoma or gynecologic surgical treatment. The treatment depends on the localization of the tumor, age of the patient, initial size, symptoms and the suitability for surgery but should always aims in toto surgical resection. Case presentation Retrospective case series and review of literature. Clinical discussion Symptoms presented by the patient were aspecific and only localized in the pelvic area. All cases were fortuitous histopathological diagnosis. No relapse was. Two out of 5 patients have pulmonary nodules, only one was biopsied and diagnosed with PBML (pulmonary benign metastasizing leiomyoma). Conclusion IVL and BML are rare disease that can co-exist. Because of tumoral hormonal receptors, hormonotherapy could be an optional treatment but to date no clear efficacy is demonstrated. In case of high recurrence risk such as voluminous initial mass, impairment of broad ligament, failure of total surgical resection, adjuvant hormonotherapy could be useful. Recurrence rate is about 16.6-30% and can occur even dozen years later and even after radical surgery, justifying a regular follow up.

Intravascular leiomyomatosis with extension to the inferior vena cava

“…IVL most commonly affects premenopausal women in the fifth decade of life,4 often with a history of uterine leiomyomas or hysterectomy 2 5. Its incidence in women with uterine leiomyomas is 0.25%–0.4% 6. IVL has been diagnosed up to 47 years after hysterectomy 4…”

“…There are two pathological theories as to how IVL occurs; first that leiomyomatosis cells arising from the myometrium invade the intima of myometrial sinuses, and second that the smooth muscles cells comprising the tumour arise directly from the venous walls of uterine or pelvic veins 1 2 5 6. Overall, the first theory is favoured in the literature, but rare cases of IVL with a normal uterus supports the second theory 4.…”

“…Overall, the first theory is favoured in the literature, but rare cases of IVL with a normal uterus supports the second theory 4. Two paths of tumour extension have been described in the literature; most commonly the tumour extends from the internal iliac vein to the common iliac vein and IVC, but it may also extend from the ovarian vein to the IVC 6 7. A staging system has been proposed where stage I IVL is confined to the pelvic cavity, stage II IVL extends into the abdominal cavity without reaching the renal vein, stage III IVL extends to the renal vein, IVC or right atrium and stage IV IVL encompasses invasion of the pulmonary arteries or metastases 6…”

“…Extra-pelvic extension of IVL is often asymptomatic until intracardiac extension occurs 1 3. Symptoms, including dyspnoea, orthopnoea, lower limb oedema, pleuritic chest pain, heart palpitations and syncopal episodes may occur with intracardiac extension of IVL 1 2 5 6. Other rare presentations such as Budd-Chiari syndrome have also been reported 5.…”

“…The key histological feature of IVL is intravenous growth of smooth muscle tumour in the absence of or beyond the limits of uterine leiomyoma 7. Most cases of IVL have no or minimal nuclear atypia and low mitotic index, however moderate to marked nuclear atypia and 5–10 mitotic figures per 10 high powered fields has been described 6 7. The degree of cytologic atypia does not appear to correlate with the clinical behaviour of the tumour, and its significance remains unknown 7.…”