Unusual Intrapulmonary Tumor
A 31-year-old black nurse was most recently admitted for assessment of recurrent hemoptysis and severe bronchiectasis. The patient had been well to age 15, when she began suffering recurrent respiratory tract infections and fevers. At ages 20 and 22 years, partial right pneumonectomies were performed for recurring hemoptysis.
Chest radiographs demonstrated extensive bronchiectasis in the right lung associated with fibrosis and volume loss. Punctate calcifications were noted in the right suprahilar and paratracheal regions where there was severe fibrotic and architectural distortion. An air space opacity was identified in the lingula with possible underlying bronchiectasis. The remainder of the left lung appeared normal (Fig 1). Fiberoptic bronchoscopy revealed edematous granular mucosa with copious purulent secretions in all of the visualized right bronchial tree. No abnormality was identified in the left system.
Computed tomography (CT) of the chest confirmed extensive destructive and cystic disease in the right lung and bronchiectasis limited to the lingula in the left lung (Fig 2). A mass was demonstrated in the right upper hemithorax; it did not enhance after intravenous contrast was injected, but contained punctate calcifications and scattered areas of decreased CT density. The mass extended into the superior mediastinum surrounding the right brachiocephalic vein and engulfing the lower superior vena cava. A fine needle percutaneous aspiration biopsy was performed, and cytologic study revealed respiratory epithelium, squamous epithelium and mesothelium. No malignant cells were identified.
Figure 1 (top), 2 (center), 3 (bottom).
Right pneumonectomy was carried out. Surgery was complicated by the presence of dense fibrous and hyperemic scar tissue binding the entire lung to the chest wall. Despite maximal surgical attempts to stop hemorrhage, the patient continued to bleed and expired 48 hours following surgery.
Histologic examination of the resected specimen showed well differentiated tissues representing all three germinal layers (Fig 3). Organoid arrangements of skin appendages, brain, bone, fat, muscle and gastrointestinal tissue were present. Pancreatic islet and acinar tissue was prominent. There was direct continuity of tumor with the lung parenchyma, and its mediastinal aspect was covered by visceral pleura. The remaining lung was grossly bronchiectatic.
