Tracheal tumors are exceedingly rare; the incidence rate of primary tracheal tumors is less than 0.2 per 100,000 people [1]. As opposed to benign conditions, maximum tracheal cancers are malignant [2]. Around two-thirds of the primary airway neoplasm’s are squamous cell carcinoma and adenoid cystic carcinoma (ACC) [3]. ACC of the trachea, also known as “cylindroma” and “adenoid cystic carcinoma,” was first described in 1859 by Billroth [4]. Billroth also described its clinical and pathological characteristics. The prevalence of ACC is equal in both sexes and tends to be higher in younger people [5]. ACC doesn’t seem to be associated with smoking. Most ACC patients present with symptoms that frequently resemble those of chronic bronchitis or bronchial asthma. ACC is a low-grade malignancy with slow growth and a prolonged clinical course. Complete surgical removal of the primary tumor is the main treatment for tracheal ACC. However, resectability frequently becomes challenging if the disease involves nearby vital tissues, particularly in individuals with distal tracheal involvement [6, 7] or large tumors. In this instance, radiotherapy is utilized either as a primary treatment option for irresectable tumors or as an adjuvant care method after surgery. We present three cases of tracheal ACC: A 49-year-old man who had a tracheal ACC underwent surgery, then radiation. Another 65-year- old female patient with a metastatic posterior thoracic wall tumor that had returned was removed surgically and received adjuvant radiotherapy, and a 38-year-old man with a polypoidal growth in the tracheal lumen. Following the removal of the tumor, the patient had adjuvant radiation.
A 49-year-old male chronic smoker presented with symptoms like asthma in emergency and received supportive treatment but was not got relieved on further investigations a non-tracer avid cyst was found in the paratracheal region. Bronchoscopy showed a pedunculated tracheal mass and biopsy of the mass was suggestive of adenoid cystic carcinoma. The patient underwent mass excision. Post-operative imaging was suggestive of residual mass. Further the patient received adjuvant radiotherapy (Figure 1).
The patient at first follow-up after 1 month of follow-up was clinically asymptomatic and his CT scan showed no significant abnormality or disease.
A 65-year-old female presented with a mass on the posterior chest wall which was excised and the biopsy of which was suggestive of malignant skin adnexal neoplasm which on further IHC was found to be ACC. After 2 months patient again presented with scar hypertrophy at the same site, and radiological imaging also showed few ipsilateral axillary lymph nodes (Figure 2).
FNAC from the node was suggestive of metastases from primary chest wall disease. The patient again underwent local resection of the hypertrophied scar with axillary lymphadenectomy. Scar mass biopsy showed no evidence of recurrent disease but one lymph node was found to be positive for malignancy. Further, the patient received adjuvant radiotherapy to the post-operative tumor bed and axillary region. On the first follow-up after one month patient was asymptomatic. After three months a PET CT was advised showing no abnormal uptake.
38-year male, non-smoker, with occasional history of alcohol intake presented with a swelling in the neck which was associated with dry cough, mild breathlessness, chest pain, and hemoptysis for 2 years. His contrast-enhanced computed tomography (CECT) scan of the thorax was suggestive of a heterogeneously enhancing mass measuring 34×22mm, arising from the left and posterior side of the upper trachea causing severe airway narrowing (Figure 3).
Barium swallow showed a filling defect with an irregular outline in the anteromedial border of the upper part of the esophagus. A large polypoidal growth obstructing more than 2/3 rd of tracheal lumen 2 cm below the inferior vena cava was seen on bronchoscopy. Biopsy from the tracheal growth was reported as polymorphous low-grade adenocarcinoma while immuno-histochemistry (IHC) was positive for CD117, P53, SMA, and EMA and negative for S- 100 which concluded it to be a malignant salivary gland-like neoplasm with characteristics of ACC. After evaluation of the patient by a multidisciplinary team including surgeons and a radiation oncologist’s patient was planned for tracheal mass excision followed by radiotherapy. After completion of radiotherapy patient came for first follow-up after one month. A CT scan was advised to patient at third month of follow-up which was found to be normal after which patient was lost to follow-up. A tele-follow-up was done at one year period after radiotherapy on which patient confirmed that he is asymptomatic and doing well.