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Asian Cardiovasc Thorac Ann 2000;8:67-69
© 2000 Asia Publishing EXchange Pte Ltd

CASE STUDY

Primary Pulmonary Leiomyosarcoma: A Rare Presentation

Naik Madhava Janardhan, FRCS, Lim Chong Hee, FRCS, Agasthian Thirugnanam, FRCS

Department of Cardiothoracic Surgery
National Heart Centre
Singapore, Republic of Singapore
For reprint information contact: Naik Madhava Janardhan, FRCS Tel: 65 321 4029 Fax: 65 224 3632 email: mnaik{at}pacific.net.sg Department of Cardiothoracic Surgery, National Heart Centre, 17 Third Hospital Avenue, Mistri Wing, Singapore 168752, Republic of Singapore.

   Abstract
TOP
 Abstract
Introduction
 Case Report
 Discussion
 References
 
An unusual case of primary leiomyosarcoma presenting as a cystic mass is reported. An 86-year-old man underwent resection of a large cystic mass measuring 15 cm in diameter, arising from the left lower lobe of the lung. Histology of the resected specimen showed primary pulmonary leiomyosarcoma. The patient made a good recovery.


   Introduction
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 Abstract
 Introduction
Case Report
 Discussion
 References
 
Primary leiomyosarcomas are extremely rare tumors of the lung. There are reports of individual cases but no large series from a single institution has been published. These tumors have been found incidentally on chest radiographs and they range in behavior from indolent to very aggressive, depending on the grade of the tumor. Diagnosis is difficult and management is complete resection of the tumor.


   Case Report
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 Abstract
 Introduction
 Case Report
Discussion
 References
 
An 86-year-old retired carpenter with a known history of bronchial asthma, hypertension, and ischemic heart disease presented with recent worsening of symptoms. He gave no history of hemoptysis, loss of weight, or loss of appetite. He was a chronic smoker but denied exposure to tuberculosis or organic dusts. On examination, he was in atrial fibrillation with bilateral pedal edema and a raised jugular venous pressure. Features of pleural effusion were noted on the left side. His hematological and biochemical investigations were normal. Chest radiography confirmed pleural effusion. A diagnostic pleural tap revealed bloodstained fluid that on microscopy showed mesothelial cells mixed with lymphocytes in a background of fibrinous material. No malignant cells were isolated. Zeil-Nielsen staining was negative for acid-fast bacilli. A computed tomography scan showed a cystic mass with pleural effusion (Figure 1Go). Bronchoscopy showed extrinsic compression of the left main bronchus. Fine needle aspiration cytology and a computed tomography-guided core biopsy were inconclusive.



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  		Figure 1. Computed tomography scan of the chest showing the cystic mass with bilateral pleural effusion.

 
At surgery, approximately 800 mL of bloodstained fluid was aspirated from the pleural cavity. There was a large cystic mass measuring approximately 15 cm in diameter (Figure 2Go) arising from the lower lobe of the lung with multiple vascular tributaries from the chest wall. Frozen section biopsy suggested a tumor of mesenchymal origin. As preliminary lung function tests precluded any radical surgery, the mass was excised along with a wedge of the lung. Talc pleurodesis was also performed concomitantly. Histology of the resected specimen showed primary pulmonary leiomyosarcoma (Figure 3Go). The patient made a slow but steady recovery and was discharged on the 11th postoperative day. He has been followed up regularly and was well at the last examination 9 months postoperatively.



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  		Figure 2. Specimen of the leiomyosarcoma measuring 15 cm excised along with a sliver of lung.

 


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  		Figure 3. (A) Photomicrograph showing cells with mitotic figures (hematoxylin and eosin stain, original magnification x40). (B) Immunohistochemistry slide showing positive reaction to smooth muscle actin stain (original magnification x40).

 

   Discussion
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 Abstract
 Introduction
 Case Report
 Discussion
References
 
Primary pulmonary sarcoma accounts for approximately 0.5% of all lung tumors.1,2 Very little is known about its clinicopathological variations. Leiomyosarcoma is the most common primary pulmonary sarcoma.3 Although more often seen in adults, it has also been described in children.1,4 Such tumors are more frequent in males and are usually found incidentally on routine chest radiographs as they seldom produce symptoms.4 They grow in the parenchyma of the lung and are seen as rounded and sharply defined opacities on chest radiographs.2,4 Rarely do they show cavitation to present as cystic masses. Unlike epithelial tumors, they do not exhibit any tendency towards exfoliation and hence bronchoscopy and washings are not good diagnostic tools.2,4 Cytology of the pleural fluid may not yield much information for the same reasons. Leiomyosarcomas metastasize via the blood stream and very infrequently through the lymphatics, making mediastinoscopy inappropriate for diagnosis. Percutaneous core biopsies are necessary for definitive diagnosis and sometimes mandate thoracoscopy or thoracotomy. A frozen section is of limited use because of the similarities with some other tumors.1,2 Other tumors to be considered in the differential diagnosis include undifferentiated carci-noma, biphasic tumors, carcinosarcoma, carcinoid tumor, intrapulmonary thymoma, and lymphoma. This makes the use of special techniques such as immunohisto-chemistry and electron microscopy important in the evaluation of such lesions.

Primary pulmonary sarcomas are classified on the basis of the cell of origin. Leiomyosarcomas are spindle cell tumors of the lung (Table 1Go).2 They arise from the hilum, originating from the peribronchial smooth muscle fibers.2,5 Unlike other sarcomas that invade the bronchial tree, these expand locally in the parenchyma. The microscopic features are a fascicular proliferation of spindle cells with scant fibrillary cytoplasm and elongated blunt-ended nuclei.2 Immunohistochemical studies show positive reactions mostly for alpha-smooth muscle actin or desmin.2,6 Keratin proliferation is also well described in leiomyosarcomas and confuses interpretation. Electron microscopy is useful as it can demonstrate the ultra-structural features of smooth muscle differentiation including subplasmalemmal densities, active pinocytosis, and cytoplasmic microfilaments with focal condensations.2


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  		Table 1. Classification of Primary Pulmonary Sarcomas
 
The best available treatment option is complete resection of the tumor, which is associated with a reported 5-year survival of 29% to 40%.2,5,7 However, only a third of such patients are amenable to surgery due to patient factors or tumor extent.2 As these tumors are resistant to both chemotherapy and radiotherapy, adjuvant therapies are generally not considered.3 The most consistent predictor of long-term survival is the grade of the tumor.1,3,6 The size of the tumor, stage at presentation, and completeness of resection are the other variables.


   Acknowledgments
 
We would like to thank the department of pathology for their cooperation and help in making the slides and the printing thereof.


   References
TOP
 Abstract
 Introduction
 Case Report
 Discussion
 References
 

  1. Attanoos RL, Appleton MA, Gibbs AR. Primary sarcomas of the lung: a clinico-pathological and immunohisto-chemical study of 14 cases. Histopathology l996;29:29–36.[Medline]

  2. Suster S. Primary sarcomas of the lung. Semin Diagn Pathol 1995;12:140–57.[Medline]

  3. Janssen JP, Mulder JJS, Wagenaar SS, Elbers HRJ, van den Bosch JMM. Primary sarcoma of the lung: a clinical study with long-term follow-up. Ann Thorac Surg 1994;58:1151–5.[Abstract]

  4. Yu H, Ren H, Miao Q, Cui Q, Zhang Z, Xu L. Primary leiomyosarcoma — a report of three cases. Chin Med Sci J 1996;11:191–4.[Medline]

  5. Moran CA, Suster S, Abbondanzo SL, Koss MN. Primary leiomyosarcomas of the lung — a clinicopathological and immunohistochemical study of eighteen cases. Mod Pathol 1997;10:121–8.[Medline]

  6. Miller DL, Allen MS. Rare pulmonary neoplasms. Mayo Clin Proc l993;68:492–8.[Medline]

  7. Travis WD, Travis LB, Deveser SS. Lung cancer. Cancer 1995:75(Suppl 1):191–202.[Medline]





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