British Journal of Radiology (2004) 77, 459-460
© 2004 British Institute of Radiology
doi: 10.1259/bjr/22684696
A woman with a pelvic mass and pulmonary nodules
C Y Chan, FRCR, FHKCR
Department of Diagnostic Radiology, Tuen Mun Hospital, Hong Kong, China
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Introduction
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A 47-year-old Chinese woman presented with a 6-month history of progressive abdominal distension. There were no other symptoms. Physical examination demonstrated a large solid and non-tender mass in the lower abdomen. Serum tumour markers including the CA125, alpha-fetoprotein, carcinoembryonic antigen and beta-human chorionic gonadotrophic hormone were all normal. Transvaginal ultrasound showed a large pelvic mass displacing the uterus to the right. There was no free pelvic fluid.
A percutaneous biopsy revealed normal smooth muscle and fibrous tissue with no evidence of malignancy. CT scan of the thorax, abdomen and pelvis was performed (Figures 1 and 2
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Figure 1. (a) Axial post-contrast CT scan through the lower abdomen shows the trilobed appearance of the solid and enhancing tumour. There is no ascites or lymphadenopathy. (b) A more caudal section demonstrates the pelvic origin of the tumour. The mass is inseparable from the left lateral uterine wall.
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What is the diagnosis?
CT images of the abdomen, pelvis and chest (Figures 1 and 2
) are shown. There was a large predominantly solid non-calcified heterogeneously enhancing mass arising from the pelvis. There was no ascites or lymphadenopathy. CT of the chest showed multiple well-circumscribed pulmonary nodules.
At operation, a 40 x 30 x 30 cm solid, reddish mass with lobulated surface was found arising from the left lateral uterine wall. It weighed 5250 g. The ovaries, the fallopian tubes and rest of the abdominal viscera were normal. Total hysterectomy and bilateral salpingo-oophorectomy were performed. The final histological diagnosis was uterine leiomyoma. The tumour was strongly positive for the oestrogen and progestogen receptors. The post-operative course of the patient was uneventful. She was put on Tamoxifen (anti-oestrogen) to shrink the pulmonary metastases. Follow-up CT 1 year later showed resolution of some of the pulmonary leiomyoma (Figure 3). She has remained asymptomatic.
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Figure 3. CT of the lower chest, at same level as in Figure 2, 1 year later shows some of the nodules in the right lower lobe are smaller or become inconspicuous.
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Discussion
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Leiomyoma is the most common pelvic tumour in women. In rare circumstances, uterine leiomyoma has been reported to involve other organs or structures. This is termed benign metastasizing leiomyoma (BML). The lung is the most common site of metastasis but spread to lymph nodes, the venous system, peritoneum or pericardium have also been reported [1].
In the past, these lesions are thought to have represented metachronous lesions from multifocal independent proliferation of smooth muscle tissue. Most people now believe these are haematogenous metastases from a morphologically benign uterine tumour [1, 2]. However, the mechanism of the tumour spread is not certain. Intravascular tumour dissemination from surgical trauma is a commonly purposed mechanism as many of the reported cases of pulmonary metastasis occur in patients with a history of previous hysterectomy [2, 3]. Others believe the pulmonary metastases are from microscopic vascular invasion of the leiomyoma into the venous system (intravenous leiomyomatosis). This could explain the concurrence of pulmonary nodules and the uterine tumour before surgery in some cases, including ours [1, 4].
Most of the patients with pulmonary BML are asymptomatic and the pulmonary nodules are found incidentally [1, 3]. A few patients present with chest pain, non-productive cough or dyspnoea. Rarely, BML can present acutely with shortness of breath resulting in respiratory failure shortly after uterine surgery [2].
On chest CT, BML typically manifests as multiple well-circumscribed nodules, ranging in size from a few millimetres to several centimetres and without zonal preponderant. These nodules are not calcified [1]. Cavitation or a military pattern has been described but is uncommon [5, 6]. There is no associated pleural effusion and no mediastinal lymphadenopathy. Pericardial effusion, however, can occur if the tumour has spread to the heart [1]. The appearance is non-specific and has a broad differential diagnosis, including malignant metastases and infectious inflammatory granulomas. Other possibilities include sarcoidosis, rheumatoid nodules, amyloidosis, and arteriovenous malformations. Percutaneous or open biopsy can be used to confirm the diagnosis.
The clinical course of the patients with pulmonary BML is usually indolent. Abramson et al reviewed seven patients with BML in the lungs and found no significant morbidity or mortality on long term follow up. They also found little correlation between the patient's respiratory symptoms and the radiologic disease extent [1]. The disease course also depends on the oestrogen and progesterone status of the patients. In post-menopausal women, most of the pulmonary nodules will either regress or remains static, whereas in pre-menopausal women, disease progression has been reported.
As oestrogen and progesterone receptors have been identified in the lung nodules. This has led to treatment options based on hormonal manipulation through surgical or medical oophorectomy [2, 3]. However, hormonal therapy may not always be indicated and its effect is difficult to analysis as literature reports are limited to case reports or small case series.
Received for publication January 5, 2004.
Accepted for publication February 11, 2004.
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References
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- Abramson S, Gilkeson RC, Goldstein JD, Woodard PK, Eisenberg R, Abramson N. Benign metastasizing leiomyoma: clinical, imaging, and pathologic correlation. AJR Am J Roentgenol 2001;176:1409–13.[Abstract/Free Full Text]
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- Abu-Rustum NR, Curtin JP, Burt M, Jones WB. Regression of uterine low-grade smooth-muscle tumors metastatic to the lung after oophorectomy. Obstet Gynecol 1997;89:850–2.[CrossRef][Medline]
- Canzonieri V, D'Amore ES, Bartoloni G, Piazza M, Blandamura S, Carbone A. Leiomyomatosis with vascular invasion. A unified pathogenesis regarding leiomyoma with vascular microinvasion, benign metastasizing leiomyoma and intravascular leiomyomatosis. Virchows Arch 1994;425:541–5.[Medline]
- Shin MS, Fulmer JD, Ho KJ. Unusual computed tomographic manifestations of benign metastasizing leiomyomas as cavitary nodular lesions or interstitial lung disease. Clin Imaging 1996;20:45–9.[CrossRef][Medline]
- Lipton JH, Fong TC, Burgess KR. Miliary pattern as presentation of leiomyomatosis of the lung. Chest 1987;91:781–2.[Abstract]
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Introduction
Discussion
