E0215 - Take My Breath Away: A Rare Cause of Dysphagia

Natali M. Perez-Cruz, MD, Delva Rivera Chacon, MD, Zeyn Mirza, MD, Priscilla Magno, MD, MSc
VA Caribbean Health Care System, San Juan, Puerto Rico

Introduction: Esophageal adenoid cystic carcinoma (EACC) is an extremely rare malignancy, with very poor prognosis with distant metastases, accounting for only 0.1% of esophageal malignancies. It is most common in the parotid and salivary glands with low locoregional metastasis, hence dysphagia is rarely associated with EACC. Here we report a case that debuted with dysphagia.

Case Description/Methods: An 81-year-old man with a history of smoking, chronic obstructive pulmonary disease, and acute promyelocytic leukemia in remission presented with four years of intermittent dysphagia to solids that improved with drinking water. He denied weight loss, chest pain, vomiting, regurgitation of food contents, odynophagia or abdominal pain. Physical exam and laboratories were unremarkable. Barium swallow showed a corkscrew appearance suggestive of esophageal spasm. GI service was consulted and recommended an EGD for direct visualization as symptoms were not consistent with esophageal spams. A chest/neck CT was ordered by Pneumology service due to associated symptoms of persistent cough and dysphonia. The CT revealed a lesion in the subcarinal compartment measuring approximately 2.7×2.1cm proximal to the left mainstem bronchus, inseparable from the esophagus. An endobronchial ultrasound (EBUS) with fine-needle aspiration (FNA) was performed on the mediastinal mass, and pathology reported an adenoid cystic carcinoma. Positron emission tomography–computed tomography showed abnormal metabolic activity of posterior mediastinum retrocarinal space, with no lymphadenopathy or evidence of distant metastases. EGD with normal esophageal mucosa, fixed extramural compression against the esophageal wall. An endoscopic ultrasound (EUS) with FNA confirmed the presence of EACC. Pathology showed cohesive clusters and aggregates of atypical epithelial cells in a tubular and cribriform pattern.

Discussion: The most common endoscopic finding of EACC is an esophageal endoluminal mass. Histologic findings of this tumor may show three growth patterns: cribriform, tubular, or solid, the latter being associated with a worse prognosis. Treatment of choice for EACC is radical excision. Left main bronchus involvement require a tracheal and bronchus reconstruction. The reported 5-year survival rate of EACC is around 35%. This highlights the importance of expedited evaluation in cases of dysphagia and the awareness of rare causes such as EACC, as the diagnosis may be confused with other entities if direct visualization and biopsy are not performed.


Disclosures:


Natali M. Perez-Cruz, MD, Delva Rivera Chacon, MD, Zeyn Mirza, MD, Priscilla Magno, MD, MSc. E0215 - Take My Breath Away: A Rare Cause of Dysphagia, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.