A 14-year-old female lifelong non-smoker presented with recurrent chest and right shoulder pain over a period of 6 weeks. She had no cough, haemoptysis and shortness of breath, upper airway symptoms or weight loss. She had no preceding illnesses. She played hockey without any problems up until the day of her presentation. Cervical lymphadenopathy was not noticed. Respiratory examination was normal.
Full blood count and blood biochemistry were normal. Chest x-ray revealed a large right middle and upper zone soft tissue mass. Obliteration of inferior aspect of right paratracheal line and right hilar structures indicated anterior and middle mediastinal involvement (). CT chest demonstrated a 10.7×12.9 cm mass with pleural involvement but no visible hilar or subcarinal lymph nodes. Major vascular structures were normal. Several small pleural nodules were identified representing pleural-based metastases (). Primary lung tumour, thymoma, teratoma and lymphoma were the important differentials.
Plain x-ray films showing a large mass extending the anterior and middle mediastinum.
CT scan of chest demonstrating a 10.7×12.9 cm mass and pleural involvement.
CT-guided needle biopsies using Temno Evolution18 (Carefusion, Voisins le BretonneuxFrance) gauge coaxial biopsy system were obtained. The positron emission tomography (PET) scan with 18F-fluorodeoxyglucose showed high metabolic activity in the tumour and pleural deposits but the disease extension was not noted in any further areas of the body (). Histopathological analysis revealed a cribriform and punched-out pattern with glandular spaces, typical picture of ACC () showing strong positive stain for actin and myosin but negative for carcinoembryonic antigen, neuroendocrine marker CD56 and chromogranin. Further immunoperoxidase studies for epithelial markers AE1/AE3 and CAM 5.2 showed strong positivity. This was in keeping with the diagnosis of ACC.
18F-fluorodeoxyglucose (FDG) positron emission tomography CT illustrated high metabolic activity in the tumour and pleural deposits.
Histology typical of adenoid cystic carcinoma showing a cribriform, punched-out pattern with glandular spaces. Magnification ×200.
Bronchoscopy showed no visible endobronchial mass lesions. External auditory canals and tympanic membranes were normal. Nasendoscopy showed normal nasal cavity and postnasal space. Oral cavity, pharynx and larynx, submandibular and parotid glands were normal. Flexible larnygopharyngoscopy was unremarkable.
After multidisciplinary lung cancer workup, right pneumonectomy and patch repair of the superior vena cava along with pleural resection for metastatic disease was carried out. Postsurgical resection sample confirmed ACC of bronchial origin infiltrating the pleural surface with pretracheal and tracheobronchial lymph node invasion. Thymic tissue did not show any signs of malignancy. Overall staging after surgery was pT3N2. After further consultation and careful consideration by the multidisciplinary lung cancer team, she received postoperative adjuvant radiotherapy to a dose of 60 Gy in 30 fractions over the period of 6 weeks using a conformal three-dimensional CT planning technique with a boost of a further 6 Gy to PET positive areas. She tolerated radiotherapy with very minimal toxicity. The patient remained clinically stable at clinic 18 months after the initial presentation and follow-up PET scan shows significant response to radiotherapy with ongoing clinical surveillance.