Intravascular Leiomyomatosis
Elizabeth A. Montgomery, MD
Key Facts
Terminology
Rare condition featuring growth of nodules of benign smooth muscle in veins of myometrium, some with extension into extrauterine veins
Clinical Issues
Most patients cured by hysterectomy
Overall good prognosis
About 30% experience persistence of disease
Occasional cases fatal
Extension into hepatic veins, heart, or lungs
Can result in presentation as “benign metastasizing leiomyomas”
Microscopic Pathology
Intravascular proliferation of lesion, showing features of a gynecologic leiomyoma
No atypia or necrosis
ER(+) and PR(+)
 Hematoxylin & eosin from a hysterectomy specimen shows intravascular growth of the lesion, which is more cellular than the investing vessels. |
 Hematoxylin & eosin shows the intravascular process at high magnification. Note the delicate longitudinal striations of the spindle cells  . |
TERMINOLOGY
Synonyms
Intravenous leiomyomatosis
Definitions
Rare condition featuring growth of nodules of benign smooth muscle in veins of myometrium, some with extension into extrauterine veins
CLINICAL ISSUES
Presentation
Not applicable
Usually premenopausal women
Median age: Early 40s
Abnormal vaginal bleeding
Pelvic pain
Enlarged uterus
Some patients (10-20%) have tumor extending into vena cava or heart
Present with cardiac symptoms
Prognosis
Overall good
Most patients cured by hysterectomy
About 30% experience persistence of disease
Occasional cases fatal
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