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Obstructing endobronchial fibrous histiocytoma: potential for lung salvage
Insights
Central endobronchial fibrous histiocytomas can cause complete lung collapse in children. Prompt diagnosis and a combined approach of tumor resection, lavage, and selective ventilation may offer an alternative to pneumonectomy.
Area of Science:
- Pediatric Pulmonology
- Thoracic Oncology
- Pediatric Surgery
Background:
- Central endobronchial fibrous histiocytomas are rare tumors.
- These tumors can lead to significant airway obstruction and lung collapse in children.
Observation:
- Two pediatric cases of central endobronchial fibrous histiocytoma presented with prolonged cough, dyspnea, and fever.
- Radiological and bronchoscopic findings revealed complete unilateral lung collapse due to endobronchial tumor obstruction.
- Computed tomography ruled out extrinsic causes, and pulmonary function tests indicated moderate to severe restrictive changes.
Findings:
- One child underwent left pneumonectomy, remaining well after 10 years.
- The second child, deemed unsuitable for endoscopic resection, underwent thoracotomy with tumor removal, lavage, and selective ventilation, achieving partial lung expansion and remaining well for 1.5 years.
- This case highlights the potential for lung salvage even in seemingly non-viable lungs.
Implications:
- Central endobronchial fibrous histiocytomas require prompt recognition and management.
- A multimodal approach including tumor resection, lavage, and selective ventilation may be a viable alternative to pneumonectomy.
- This strategy can potentially preserve lung function and improve outcomes in pediatric patients.
Abstract:
In the past 10 years two children, ages 3 1/2 and 7 years, have been seen with central endobronchial fibrous histiocytoma causing complete atelectasis. Each child had a 4- to 6-month history of cough, dyspnea and fever. After antibiotic failure, x-rays demonstrated complete unilateral lung collapse. Bronchoscopy in case 1 demonstrated total occlusion of the left mainstem bronchus with 80% stenosis of the right. Left pneumonectomy was ultimately performed with removal of tumor frm the right bronchus. Ten years later the patient is well. Case 2 had an extrinsic etiology ruled out by computed tomography scan. Ventilation/perfusion scan showed no ventilation and only faint perfusion of the involved lung. Pulmonary function testing showed moderate to severe restrictive changes with air trapping. Total obliteration of the left mainstem with tumor projecting into the carina was observed on bronchoscopy. Endoscopic resection was not felt to be safe. Thoracotomy showed a totally atelectatic, edematous lung filled with pus. The left main bronchus was opened, the tumor removed, and the distal bronchus lavaged and selectively ventilated. Partial lung expansion was obtained. The child is well 1 1/2 years later with a normal chest radiograph, pulmonary function tests, and bronchoscopy. Fibrous histiocytomas of the lung, if in a central endobronchial location, can cause complete lung collapse. A combination of tumor resection, lavage, and selective ventilation should be attempted as an alternative to pneumonectomy, even if the lung appears to be nonsalvageable.