Ann Thorac Surg 2009;87:613-614. doi:10.1016/j.athoracsur.2008.06.052
© 2009 The Society of Thoracic Surgeons
Case Reports
Consequences of a Wait-and-See Strategy for Benign Metastasizing Leiomyomatosis of the Lung
Konrad Hoetzenecker, MDa,
Hendrik J. Ankersmit, MDa,*,
Clemens Aigner, MDa,
Michael Lichtenauer, MDa,
Soeren Kreuzer, MDb,
Stefan Hacker, MDa,
Wolfram Hoetzenecker, MD, PhDc,
György Lang, MDa,
Walter Klepetko, MDa
a Department of Cardiothoracic Surgery, Medical University of Vienna, Vienna, Austria
b Department of Radiology, Medical University of Vienna, Vienna, Austria
c Department of Dermatology, Medical University of Tübingen, Germany
Accepted for publication June 3, 2008.
* Address correspondence to Dr Ankersmit, Department of Cardiothoracic Surgery, Medical University of Vienna, Währinger Gürtel 18-20, Vienna, 1090, Austria (Email: hendrik.ankersmit{at}meduniwien.ac.at).
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Abstract
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Pulmonary benign metastasizing leiomyomatosis (BML) is a rare smooth-muscle cell disorder of the lung. Most BML lesions stay constant in size for a long time. The prevailing treatment option is primary excision of the nodules or if unresectable, long-time hormone therapy. Herein, we present a case of BML in which a wait-and-see strategy after diagnosis was decided. Fourteen years later a routine chest roentgenogram revealed multiple bi-lobar BML lesions with a giant cyst filling the whole left lung cavity. We conclude that a wait-and-see procedure for BML is feasible, but primary resection of the BML tumor masses is preferable to avoid complications as described in our case.
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Introduction
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Benign metastasizing leiomyomatosis (BML) of the lung is a rare disease entity presenting with pulmonary smooth-muscle cell nodules originating from an uterus myomatosus [1]. Despite its metastasizing potential, BML is classified as a benign tumor due to the absence of anaplasia or prominent mitotic activity in histologic analysis. Lesions are seldom symptomatic and therefore, they are often discovered incidentally by routine chest roentgenogram. In rare cases, BML can present as cysts. Entrapment of mucus-secreting pulmonary epithelium by proliferating nodules is made responsible for this cyst formation [2]. There are only few cases of cystic BML reported in the literature. Cysts are normally small in size, but lesions can grow up to 20 cm in diameter [3].
In most cases, pulmonary nodules have been shown to stay constant for a long time [2]. Currently there are no guidelines for the treatment of BML. Older case reports suggest a hormone treatment as the option of choice; however, recently, hormone-therapy resistant BML have been described [4]. If resectable, a surgical excision of the nodules is recommended as a first-line therapy.
Herein, we present a case of BML diagnosed 14 years ago. At that time, an observational strategy with regular control chest roentgenograms was decided. Multiple bi-lobar lesions and a giant cyst developed in the patient 14 years later, completely filling out the left thoracic cavity and leading to a beginning mediastinal shift.
A 56-year-old woman was admitted to the Medical University of Vienna in 1993, because a routine chest roentgenogram had previously revealed multiple, bilateral suspect pulmonary tumor masses. She had given birth to 2 children and underwent a hysterectomy at the age of 41 because of an uterus myomatosus. A video-assisted thoracoscopy with biopsies of two pulmonary lesions was performed. Pathologic evaluation showed bundles of spindle-shaped, smooth-muscle cells (Fig 1) staining positive for desmin and actin. Based on these findings, pulmonary BML was diagnosed. The patient refused a surgical intervention, and hormone treatment was abandoned because of heavy menopausal disorders. As the patient was asymptomatic, and malign transformation of BML is uncommon, no further therapeutic actions were taken, and regular control chest roentgenograms were agreed on. However, the patient was lost to follow-up, until in 2007, 14 years later, she applied to a pulmonologist in private practice because of increasing mucus production and occasional expiratory rhonchus. A chest roentgenogram showed a fluid pneumothorax displacing nearly the whole left lung. The patient was transferred to the Department of Cardiothoracic Surgery, Medical University of Vienna. A thoracic computed tomographic scan revealed eleven (eight on the right side and three on the left side) intrapulmonary circumscribed circular foci (Fig 2A). In addition, the left lower third was filled up with a tumor mass. Originating from this tumor mass, a giant cyst with a diameter of 18 cm extended into the thoracic cave. The left inferior lobe was completely, the superior lobe partly depleted, and a beginning transposition of the mediastinum to the right was visible. Lung function tests showed a significantly reduced functional vital capacity of 1.64 L (62.4% of the reference value). Based on these findings, an excision of the nodules was decided. Through a muscle-sparing left lateral thoracotomy, the three left nodules, the huge basal tumor, and the cyst were removed, and a complete decortication was performed. Entire expansion of the remaining lung was achieved. The postoperative course was uneventful, and the patient could be discharged on postoperative day 7. To avoid further complications it was decided to electively excise the remaining nodules on the right side (Fig 2B) 2 months later. This second intervention was performed without complication, and 6 weeks later on a routine control, the patient had fully recovered and had no evidence of a remaining disease.
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Fig 1. A histopathologic evaluation of pulmonary benign metastasizing leiomyomatosis nodules. The lesions contain spindle-shaped smooth muscle cells, devoid of cytologic malignancy. (Hematoxylin-eosin stain: left panel, 100x; right panel, 200x.)
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Fig 2. (A) Computed tomographic image in a lung window setting of the patient. Multi-loculated, well-defined circular foci can be seen scattered in both lung lobes. The left thoracic cavity is filled up with a basal tumor mass and a giant cyst. (B) A three-dimensional reconstruction of the remaining benign metastasizing leiomyomatosis lesions after the first surgical intervention. The left lung lobe is now completely expanded, and the tumor masses in the right lobe are still present.
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Comment
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We believe that this is the first case in literature describing a wait-and-see strategy of 14 years for BML. Treatment option of choice is a primary surgical resection of the tumor masses. If lesions are not resectable, a hormone treatment should be considered instead. Tumors were shown to be sensitive to treatment with progestin (ie, a synthetic progesterone), goserelin, and ovarian ablation (for estrogen restriction). However, not all patients seem to respond to hormone treatment [4], and side effects including flushes, fatigue, and nausea can be aggravating. As our patient refused to undergo surgery and a hormone treatment was not an option due to menopausal disorders, a wait-and-see strategy was decided. However, 14 years after the initial BML diagnosis, the patient presented with a giant cyst filling up the whole left lung cavity.
The reason for tumor progression in BML is not known. Most BML lesions stay constant in size; however a small percentage is known to display an aggressive course [2]. As most BML stain positive for estrogen and progesterone receptors [4, 5], a hormone involvement in the progression of the lesions is likely.
Our case report indicates that a wait-and-see strategy is feasible but could lead to complications. Therefore, we conclude that a primary excision of BML tumor masses is preferable to avoid complications leading to more extended surgical interventions.
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References
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- Martin E. Leiomyomatous lung lesions: a proposed classification AJR Am J Roentgenol 1983;141:269-272.[Abstract/Free Full Text]
- Bachman D, Wolff M. Pulmonary metastases from benign-appearing smooth muscle tumors of the uterus AJR Am J Roentgenol 1976;127:441-446.[Abstract]
- Gotti G, Haid MM, Paladini P, Di Bisceglie M, Volterrani L, Sforza V. Pedunculated pulmonary leiomyoma with large cyst formation Ann Thorac Surg 1993;56:1178-1180.[Abstract/Free Full Text]
- Goyle KK, Moore Jr DF, Garrett C, Goyle V. Benign metastasizing leiomyomatosis: case report and review Am J Clin Oncol 2003;26:473-476.[Medline]
- Takemura G, Takatsu Y, Kaitani K, et al. Metastasizing uterine leiomyoma: a case with cardiac and pulmonary metastasis Pathol Res Pract 1996;192:622-630.[Medline]
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Abstract
Introduction
