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
Highlights
Adenoid cystic carcinoma (ACC) is an uncommon form of malignant neoplasm that arises most commonly in the major and minor salivary glands of the head and neck [1], but rare in the esophagus
As the invasive site of ACC is close to the esophagus and cardia, and the biological and pathological characteristics are similar, the current study presents a rare case of cardial ACC and a review of 17 other cases of ACC of the esophagus that have been reported in China so as to provide more information regarding the clinic manifestations and operable treatments for ACC of the upper digestive tract
Cancers of the adenoid cystic type commonly originate in the major salivary glands and account for 10‐25% of all salivary gland tumors
Summary
Adenoid cystic carcinoma (ACC) is an uncommon form of malignant neoplasm that arises most commonly in the major and minor salivary glands of the head and neck [1], but rare in the esophagus. The enhanced computed tomography (CT) scan showed that the stomach wall of the cardia was abnormally thickened and the lymph nodes around the lesser curvature were enlarged (Fig. 2). A local expanded resection of the liver nodule was performed to ascertain its pathological type and the frozen section results indicated that the nodule was an adenomatous hyperplasia. Microscopic examination of the lesion demonstrated an infiltrative malignant neoplasm invading serosa without metastases to the lymph nodes (pT4aN0M0, stage IIB according to the tumor‐node‐metastasis; TNM staging system). These findings were consistent with an ACC developing from the cardia. The patient was discharged from hospital seven days following surgery and no signs of recurrence have been detected during the 30 months of follow‐up
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