Correct!
4. 1 or 3
Adenoid cystic carcinoma (ACC) was previously called a cylindroma (1,2). It is a rare tumor which originates from submucosal glands and can arise where submucosal glands are present. When present in the lung ACC usually arises centrally within large airways as an intraluminal mass, rarely forming a peripheral nodule. It is a slow-growing low-grade malignancy. Symptoms depend on location, often causing cough, dyspnea, wheezing and stridor. Hemoptysis is rare.
The preferred treatment is surgical resection (2). However, our patient has severe oxygen-dependent COPD which precludes a pneumonectomy or sleeve resection. In the case of an unresectable tumor, debulking and radiation therapy are usually performed. Five-year survival ranges between approximately 50 and 80 % in patients with resectable disease, and 30 % for those with unresectable disease. Recurrences can occur as late as 10-15 years after surgical resection. Chemotherapy has not been demonstrated to increase survival.
Our patient underwent debulking initially with a snare to remove large segments and biopsy forceps by rigid bronchoscopy. KTP laser was used to remove fragments (Figure 5).
Figure 5. Initial KTP laser.
A second session was performed with the KTP laser 4 months later (Figure 6).
Figure 6. Bronchoscopy for second session of KTP laser therapy 4 months after initial session.
Subsequently image-guided radiotherapy and endobronchial brachytherapy. Were performed. A year later the patient was doing well. Repeat thoracic CT scan showed the left mainstem bronchus to be open (Figure 7).
Figure 7. Repeat thoracic CT scan 1 year after laser therapy. A-C: representative axial views in lung windows. D-F: representative coronal views in lung windows.
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