Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Primary endobronchial leiomyosarcoma
- Radiologic Findings
- On pre contrast and (Fig.1) and post contrast images (Fig 2,3) an irregular well enhancing mass in right main bronchus is noted. Right main bronchus is completely obstructed by endobronchial mass. Atelectasis of right lung with right pleural effusion are also noted.
On chest PA view, total atelectasis of right lung is noted and an obstructing mass shadow is observed in right main bronchus.
- Brief Review
- Diagnosis was confirmed by bronchoscopic biopsy.
Primary endobronchial leiomyosarcoma is an uncommon malignancy of the lung. Leiomyosarcoma is somewhat more common than leiomyoma and is more common in men than women with a ratio of 2.5 to 1. Leiomyosarcomas are thought to arise from smooth muscles tissues in bronchus and pulmonary vascular structures. Endobronchial leiomyosarcomas are often symptomatic, complaining cough, dyspnea, hemoptysis or chest pain. Unlike other carcinoma of the lung, leiomyosarcoma is not associated with cigarette smoking or other potential inhalant carcinogens.
Radiographic findings consists of sharply defined lobulated pulmonary mass or atelectasis due to bronchial obstruction. Endobronchial mass may be observed on CT scans but definitive diagnosis can only be made by excision biopsy.
Prognosis is not well known because of the rarity of this neoplasm. According to reports, children and adolescents show more favorable prognosis than adults. Some reports describe that endobronchial tumors are less aggressive than parenchymal neoplasm, largely because the former tend to be smaller and diagnosed earlier. Surgical resection is the treatment of choice. Pulmonary leiomyosarcomas appears relatively resistant to adjunctive therapy, such as chemotherapy or radiotherapy. In a review of literatures, radical surgical resection produce a 5 year survival rate of 45~50%.
- References
- 1. Leslie KO, Wick MR. Practical pulmonary pathology: A diagnostic approach. 2nd ed.Elsevier Saunders 2011: 457-460
2. Takeda F, Yamagiwa I, Ohizumi H, Shiono S. Leiomyosarcoma of the main bronchus in a girl: a long-time survivor with multiple lung metastases. Pediatr Pulmonol 2004;37(4):368-74
3. Lee MC, Hsu CP, Hsia JY. Surgical treatment of endobronchial leiomyosarcoma with right main bronchus total obstruction: A case report. Ann Thorac Cardiovasc Surg. 2008;14(2):105-8
- Keywords
- bronchus, malignant tumor,