Adenoid cystic carcinoma of the cardia: Report of a rare case and review of the Chinese literature

Abstract

Adenoid cystic carcinoma (ACC) is a relatively common head and neck tumor, however, is rare in the digestive tracts. There have been <100 cases of esophageal ACC reported to date and no cases of gastric ACC. The present study reports the exceptional case of a 53-year-old male with a primary ACC of the cardia. The patient underwent a radical total gastrectomy with D2 lymphadenectomy and Roux-en-Y esophagojejunal reconstruction. Immunohistochemical analysis identified a case of primary ACC that exhibited a positive expression for cytokeratin, calponin, cluster of differentiation 117, p63 and smooth muscle actin, with typical cribriform foci. No signs of recurrence have been detected during the 30-month follow-up. Thus, a precise diagnosis of ACC is primarily based on the results of immunohistochemical analysis and radical resection is considered to be the best treatment option for ACC of the digestive tracts. The current study also reviewed 17 cases of ACC of the esophagus reported in China, with special reference to the criteria for histological diagnosis and therapeutic options. The prognosis of esophageal ACC is poor due to early metastasis, mainly relying on the resectability of the tumor.