Cricotracheal Adenoid Cystic Carcinoma: Insights Into the Diagnosis and Management of an Uncommon Anatomic Variant

Adenoid cystic carcinoma (ACC) is the second most common malignant salivary gland tumor and accounts for 30% of minor salivary gland tumors. Its location in the larynx and trachea are rare. We present the case of a 45-year-old healthy male whose MRI revealed a posterior endoluminal tumor that invaded the posteroinferior perichondrium of the cricoid lamina and displaced the hypopharynx and esophagus. A left-limited cervical surgical exploration and an intraluminal incisional biopsy through the tracheostomy space were performed by another surgical team. The pathological study reported an ACC, T4aN0M0, stage IVA tumor. Then, a circular tracheal resection and an excision of the inferior part of the posterior cricoid lamina were carried out. The macroscopic study showed a lesion, 3cm long, 2.2cm wide, and 1cm thick, located at the posterior wall of the cricoid cartilage and proximal trachea. Only the upper margin was compromised. Microscopically, the tumor showed tubular, solid, cribriform, and trabecular patterns. One and a half years after surgery, the patient still has bilateral vocal cord mobility and normal speech. It is clear that a contrast-enhanced CT scan is useful to assess tumor extent and growth pattern in these rare variants. Among treatment alternatives, surgery sometimes complemented with radiotherapy is essential; constant follow-up is mandatory.