Adenoid cystic carcinoma of trachea- a rare case with review of literature

A 54 years female presented with 6 months history of dyspnea and 2 months history of haemoptysis. She was evaluated with CT thorax which showed 22×16×15 mm sized hypodense soft tissue lobulated lesion in right lower trachea extending inferiorly into right side of carina, up to origin of right main bronchus. Brochoscopic guided biopsy was done which was S/O adenoid cystic carcinoma, grade 1. Patient was treated with definitive concurrent radiotherapy (IMRT 66Gy/33#/6.3 weeks) and weekly chemotherapy with Cisplatin. Post treatment CT thorax showed 16×15 mm sized heterogeneously enhancing soft tissue enhancing lesion at right side tracheal bifurcation. She was started on tab Gefitinib 250 mg once a day, tolerating it well since 19 months with minimal residual disease as per CT thorax. Tracheal tumors are very rare with an incidence less than 0.2 per 100,000 persons per year. Squamous cell carcinoma is most common followed by adenoid cystic carcinoma. It’s a very indolent tumor with prolonged clinical course with a tendency for local recurrence and late metastasis. Adenoid cystic carcinoma is not associated with smoking as causative factor unlike squamous cell carcinomas. It has got better prognosis than squamous cell carcinomas. Most of these tumors are detected in middle age in 4th and 5th decade and it has got almost equal distribution in both sexes. It is asymptomatic initially when the size is small, but as the size grows, it may cause hoarseness, cough, hemoptysis, wheezing, chest pain, dysphagia etc. Most of these patients are treated for bronchial asthma or bronchitis for a long time, before getting detected. There are 2 definitive treatment modalities, surgery and radiotherapy. Surgery is the treatment of choice with adjuvant radiotherapy in resectable tumors. Radiation is used in multiple indications like adjuvant after surgical procedure, definitive treatment of unresectable tumors and palliative treatment of tumor for symptomatic relief.