1. Benign metastasizing leiomyomatosis

2. Benign metastasizing leiomyomatosis
Leiomyoma is the most common pelvic tumor in women.
In rare circumstances, uterine leiomyoma has been reported
to involve other organs or structures.
This is termed benign metastasizing leiomyoma (BML).

3. Benign metastasizing leiomyomatosis
Intravenous leiomyomatosis (IVL)
- leiomyoma-like lesions that extend from the uterus
into the pelvic blood vessel, and sometimes as far as the heart.
- with leiomyomas and significant dyspnea, an intracardiac mass,
or evidence of right heart failure.
Disseminated peritoneal leiomyomatosis (DPL)

4. Benign metastasizing leiomyomatosis
Pathology
Uterine leiomyomas
with less than 5 mitoses per 10 HPFs, (high power field)
with no cellular atypia or necrosis
leiomyosarcomas
a mitotic index greater than 10, marked cellular atypia,
and coagulative tumor cell necrosis characterizes.
For tumors with characteristics between these extremes, the term,
“leiomyoma of uncertain malignant potential”

5. Benign metastasizing leiomyomatosis
Predominantly in women during reproductive years
Steiner
- prevalence of about 50% above the age of 30 yr
The lung is the most common site of metastasis
- 0,085% of pulmonary benign tumors
- Hematogenous metastases
- Lymph nodes, the venous system, peritoneum or pericardium.
- The mechanism of the tumor spread is not certain.

6. Benign metastasizing leiomyomatosis
The mechanism
Intravascular tumor dissemination from surgical trauma
a commonly purposed mechanism pulmonary metastasis occur
in patients with a history of previous hysterectomy .
Others
from microscopic vascular invasion of the leiomyoma
into the venous system (intravenous leiomyomatosis).
the concurrence of pulmonary nodules and the
uterine tumor before surgery in some cases.

7. Benign metastasizing leiomyomatosis
On chest CT
- as multiple well circumscribed nodules
- ranging in size from a few millimetres to several centimetres
- not calcified
- cavitation or a military pattern uncommon
- no pleural effusion and
no mediastinal lymphadenopathy.
- Pericardial effusion

8. Benign metastasizing leiomyomatosis
Diagnosis
Percutaneous or open biopsy
Differential Diagnosis
appearance is non-specific.
malignant metastases , infectious inflammatory granulomas
sarcoidosis, rheumatoid nodules, amyloidosis,
and arteriovenous malformations.

9. Benign metastasizing leiomyomatosis
Treatment
Treatment options based on hormonal manipulation
through surgical or medical oophorectomy.
Most BMLs are ER positive
The estrogen and progesterone status
In post-menopausal women vs in pre-menopausal women

10. Benign metastasizing leiomyomatosis
Treatment
Hormonal manipulation - Antiestrogen
LHRH agonist
Aromatase inhibitor (Anastrozole)
decrease estrogen production from
extragonadal sources
SERMs
Selective Estrogen Receptor Modulators
(Raloxifene)
antagonize estrogen actions
in target tissues

11. Benign metastasizing leiomyomatosis
Treatment
Raloxifene, a synthetic nonsteroidal SERM
- as an estrogen agonist,
on the skeleton, cardiovascular system, and CNS
weak estrogenic antagonist effect on the breast and uterus.
- A randomized, placebo-controlled trial
the addition of raloxifene in patients treated with an LHRH agonist
induces a greater reduction of leiomyoma sizes.
- The addition of raloxifene prevents
LHRH-agonist treatment-related bone loss in premenopausal women
with uterine leiomyomas.

12. Summary of recent reports of benign metastasizing leiomyoma
Modern Pathology (2006) 19, 130–140

13. Benign metastasizing leiomyomatosis
Prognosis
The clinical course
- usually indolent
Abramson et al reviewed seven patients with BML in the lungs
- no significant morbidity or mortality on long term follow up.
- little correlation between the patient’s respiratory symptoms
and the radiologic disease extent.
BML should be regarded as a borderline tumor or
a tumor with low malignant potential.

14. Natural Decrease of Benign Metastasizing Leiomyoma
Chest 2000;117;
52 age Estrogen 20.4 µg/dL
Progesterone 1.5 ng/mL
53 age Estrogen 4.3 µg/dL
Progesterone 0.3 ng/mL

15. A woman with a pelvic mass and pulmonary nodules
The British Journal of Radiology, 77 (2004), 459–460
Department of Diagnostic Radiology, Tuen Mun Hospital, Hong Kong, China
47-year-old Chinese woman, Tamoxifen (anti-estrogen)

16. Hormonal Manipulation
The Journal of Clinical Endocrinology & Metabolism
(2004) 89(7):3183–3188
A 47-yr-old Caucasian woman
hysterectomy at age 36 yr for a 6-cm
uterine leiomyoma.
The long-acting LHRH agonist, leuprolide acetate,
anastrozole, an AI

17. Hormonal Manipulation
The Journal of Clinical Endocrinology
& Metabolism (2004) 89(7):3183–3188
A 37-yr-old Haitian woman
Respirology department for progressive
shortness of breath
age 34 yr. Cells from the 22 x 16 x 10 cm
Intramural leiomyoma
strongly positive for both ER and PR.
At age 47 yr,
ICU with acute respiratory failure
raloxifene 60mg and anastrozole 1 mg daily.
bilateral oophorectomy

18. Benign metastasizing leiomyomatosis
Benign Pulmonary Metastasizing Leiomyomatosis Kwon YI. Hanyang University College of Medicine, Seoul, Korea.
Korean J Intern Med Sep;21(3):
A Case of Benign Metastasizing Pulmonary Leiomyomatosis.
Kim YS. Dankook University College of Medicine
Tuberc Respir Dis Aug;53(2):
An Intravenous leiomyoma with cardiac involvement in a patient with uterine leiomyoma.  Kim JS. Sungkyunkwan University School of Medicine.
Korean J Intern Med May;66(5):
Two Cases of Intravenous Leiomyomatosis of the Uterus. 
Kim JH, Koh MW. Department of Obstetrics and Gynecology,
College of Medicine, Yeungnam University, Daegu, Korea.
Yeungnam Univ J Med Dec;23(2):