Primary Pulmonary Leiomyosarcoma: Case Report and Review of Literature PAUL W. SCHANHER, Jr., M.D.
A case of primary leiomyosarcoma of the lung is added to the Springfield Community Hospital on March 15, 1970, with chief complaint literature. Previously, 66 cases were documented. This is a case of shortness of breath on any type of exertion. Walking up one flight of of a 55-year-old male, a pulmonary cripple for many years, with stairs required three or four resting pauses. During the past two weeks, the a collapsed left lower lobe found by roentgenogram, verified by symptoms had increased with associated wheezing and nonproductive bronchoscopy and bronchograms. Biopsy of lesion was reported cough. He had smoked since age nine and for many years consumed three as low grade leiomyosarcoma. Despite pulmonary function to four packs of cigarettes per day. He stopped cigarettes 1½ years prior to studies showing a vital capacity of 37% of predicted normal, a admission, but smoked 16-18 cigars daily until June 1969, when he left pneumonectomy was preformed. Postoperatively, the stopped completely. Laboratory studies showed a Hgb. 17.5 gm/100 ml with a Hct. of 51%. patient had a tachycardia which gradually decreased in the next several months. Two months following operation pulmonary WBC, differential, blood electrolytes and urine were essentially normal. function studies were essentially unchanged from those made Arterial blood gases: pH 7.384, pCO2 48.3 mm, PO2 75 mm, 02 saturation preoperatively. Arterial gas studies are presented pre and 94%. Roentgenogram of chest showed a collapsed left lower lobe with compensatory emphysema of left upper lobe. The right lung was clear and postoperatively.
overall the chest appeared somewhat emphysematous. Heart size was normal. Bronchoscopy revealed a polypoid type lesion occluding the left lower L EIOMYOSAIRCOMA of the lung has been considered bronchtus and a biopsy was taken. Because of his precarious respiratory balance, supravoltage radiation therapy was the only treatment considered an extremely rare tumor in the past. In 1952, Johnson initially the pathology report was returned as leiomyosarcoma of et al.6 documented the first case successfully treated by relativelytntil low grade malignancy. pneumonectomy. There has been a gradual increase in the Further evaluation was made. Pulmonary function stu'dies showed vital medical literature regarding this tumor since then. Havard capacity of 37% of predicted normal and on forced expiratory volume 1st and Hanburn5 found 28 previously reported cases in 1960 Sec. 60%, 2nd Sec. 78%, 3rd Sec. 85%, with the maximum voluntary ventilation 26%. This was interpreted as a severe increase in airway resistance and added an additional case. In 1965, Fadhli et al.' made a with severe loss of pulmonary compliance and an estimated 80% loss of statistical review of 38 cases from the literature plus a case pulmonary reserve. Bronchograms revealed an intraluminal lesion blocking report. Guccion and Rosen3 in 1972 reporting from the Arms the entire left lower lobe and extending into the left main stem bronchus Forces Institute of Pathology and the Veterans Administra- and over into the major portion of the left upper lobe bronchus with only a tion Central Laboratory in Washington, D.C., reviewed 19 very narrow irregular channel in the left upper lobe bronchus leading to size displaced bronchi distally (Fig. 1). cases, three of which were previously reported, thus adding normal of the favorable prognosis of the leiomyosarcoma of low grade Becatuse 16 cases. In all, 66 cases have been recorded to date. malignancy with surgical removal, an obvious trapped left lung and poor This case is reported because of its successful conclusion response of this type tumor to any type of radiation therapy, an exploratory in what was initially thought to be an irredeemable case stir- thoracotomy was done through a left posterior lateral incision. The left gically based on his history and pulmonary function studies. lower lobe was completely atelectatic with compensatory emphysema bullae of the upper lobe. There was a palpable mass in the inferior It represents the conversion of a "pulmonary cripple" to without hilar region extending down into the lower lobe adjacent to the left lower one capable of carrying on with everyday activities in a lobe bronchus. Blood arterial gases were taken from the left pulmonary limited fashion. artery and repeated following its occltusion, in approximately 5 minutes. Specimen 1: pH 7.18, pCO2 42 mm P02 45 mm. Specimen 11: pH 7.17, Case Report PCO2 50.5 mm., PO2 50 mm. A routine left pneumonectomy was carried with dissection of three mediastinal lymph nodes as a separate out C.S., a 55-year-old white married male with a long history of asthma, excised following bronchitis, emphysema and repeated bouts of pneumonia was admitted to specimen. An additional 1 cm of left stem bronchusofwas inspection of the resected lung due to the proximity the tumor to the cut edge. Frozen section of the separate bronchial specimen was negative for Stubmitted for puiblication April 19, 1974.
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PRIMARY PULMONAFLY LEIOMYOSARCOMA
tilation was 24% of predicted normal. This was interpreted as being essentially uinchanged from preoperative studies and an estimated 50% reduction in breathing reserve. Arterial gas studies were normal with the pH 7.406, pCO2 41.9 mm, P02 83.2 mm and 02 saturation 95.3%. Clinically, the patient has continued to do well, feeling better than he has for many years and capable of activities which were previotusly prohibitive. His pulse rate remains normal. He continues to take medication for his sinusitis and slight asthma plus an expectorant.
References 1. Fadhli, H. A., Harrison, A. W. and Shaddock, S. H.: Primary
Pulmonary Leiomyosarcoma. Dis. Chest, 48:431, 1965.
tracheostomy was preformed at the conclusion of the procedure with the patient tolerating the operation well throughout. Pathology report was leiomyosarcoma, moderately well differentiated arising from the lower lobe bronchus with metastases to the nearest peribronchial lymph node. The three mediastinal lymph nodes were negative for metastases. Postoperatively, the patient did well, but because he developed an AV block and tachycardia, the digoxin dosage which he had been taking preoperatively was increased and then subsequently discontinued when it was felt that this might be a factor in the continuation of the tachycardia. He was placed on potassium which resulted in improvement. On the 9th postoperative day arterial gases were pH 7.472, pCO2 34.5 mm PO2 67.2 saturation 93.5%. Improvement was gradual and he was dismm and charged on the 21st post-operative day. Two months following operation pulmonary function studies showed vital capacity of 40% of predicted normal. On forced expiratory volume 1st Sec. 60%, 2nd Sec. 73% and 3rd Sec. 86%. The maximum voluntary ventumor. A
2. Glenn, F. and Okinada, A. J.: The Lung Tumor of a Virologist; A Short Case Report of a Rare Tumor and a Bit of Medical History. J. Thoracic Cardiovasc. Surg., 51:455, 1966. 3. Guccion, J. G. and Rosen, S. H.: Bronchopulmonary Leiomyosarcoma and Fibrosarcoma. Cancer, 30:836, 1972. 4. Gtiillan, R. A., Wilen, C. J. W. and Zelman, S.: Primary Leiomyosarcoma of the Luing. Dis. Chest, 56:452, 1969. 5. Harvard, C. W. H. and Hanburn, W. J.: Leiomyosarcoma of the Lung. Lancet, 1:902, 1960. 6. Johnson, E. K., Mangiardi, J. L. and Jacobs, J. B.: Primary Leiomyosarcoma of the Lung Treated by Pneumonectomy. Surgery, 32:1010, 1952. 7. Lawson, E. E. and Goldstein, I. R.: Bronchial Leiomyosarcoma in a Child. J. Pediatr. Surg., 6:179, 1971. 8. Mason, M. K. and Azeem, P. S.: Primary Leiomyosarcoma of the Lung. Thorax, 20:13, 1965. 9. McNamara, J. J., Paulson, D. L., Kingsley, W. B., et al.: Primary Leiomyosarcoma of the Lung. J. Thorac. Cardiovasc. Surg., 57:635, 1969. 10. Randall, W. S. and Blades, B.: Primary Bronchiogenic Leiomyosarcoma. Arch. Pathol., 42:543, 1946. 11. Shaw, R. R., Paulson, D. L., Kee, J. L., and Lovett, V. F.: Primary Pulmonary Leiomyosarcomas. J. Thorac. Cardiovasc. Surg., 41:430, 1961. 12. Watson, W. L. and Anlyan, A. J.: Primary Leiomyosarcoma of the Lung. Cancer, 7:250, 1954. 13. Yacoubian, H., Connolly, J. E. and Wylie, R. H.: Leiomyosarcoma of the Lung. Ann. Surg., 147:116, 1958.