Submucosal gland neoplasms of the esophagus: an update and review
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REVIEW ARTICLE
Submucosal gland neoplasms of the esophagus: an update and review Ling Nie1 · Wei Li1 · Liyan Xue2 · Lin Wang3 · Yonghua Shen4 · Xiangshan Fan1 Received: 11 March 2020 / Accepted: 25 June 2020 © The Author(s) 2020
Abstract Submucosal glands (SMGs) present throughout human esophagus with clusters at either the upper third or lower third of the organ. SMGs tend to atrophy with age, and neoplasms arising in these glands are rare. In order to bring convenience to diagnosis, we summarize the histopathologic characteristics of all esophageal submucosal gland tumors (SGTs). Due to the morphological similarity, the nomenclature of salivary tumors is adopted for SGTs. However, there is great confusion about the definition and histogenesis of these tumors, especially the malignant subtypes. In the literature, esophageal mucoepidermoid carcinoma and adenoid cystic carcinoma usually adjoin the surface squamous epithelium and coexist with intraepithelial neoplasia or invasive squamous cell carcinoma (SCC). In addition, the typical gene alterations of salivary tumors have not been reported in these SGTs. Therefore, we propose to apply stringent diagnostic criteria to esophageal SGTs so as to exclude mimickers that are SCCs with various degree of SMG differentiation. Keywords Esophagus · Submucosal glands · Tumor · Classification
Introduction Esophageal submucosal gland (SMG) is located underneath the esophageal muscularis mucosa. It consists of mucous cells with or without a minor serous component and produces acid mucins and bicarbonate [1]. The duct penetrates through the mucosa to open into the esophageal lumen. Esophageal neoplasms arising in the SMG are rare. Due to the morphological similarity, the nomenclature of salivary tumors is adopted. The main types are mucoepidermoid * Xiangshan Fan [email protected] 1
Department of Pathology, The Affiliated Drum Tower Hospital, Nanjing University Medical School, Jiangsu Province 210008 Nanjing, People’s Republic of China
2
Department of Pathology, Cancer Institute and Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 100021 Beijing, People’s Republic of China
3
Department of Pathology, Shanxi Bethune Hospital, Shanxi Province 030032 Taiyuan, People’s Republic of China
4
Department of Gastroenterology, The Affiliated Drum Tower Hospital, Nanjing University Medical School, Jiangsu Province 210008 Nanjing, People’s Republic of China
carcinoma and adenoid cystic carcinoma [2, 3]. Over time, new entities have been discovered and characterized, and the spectrum of esophageal submucosal gland tumors (SGTs) has been expanded. However, most reported SGTs differ from salivary gland primaries. Awareness of the discrepancy is necessary to correct diagnosis and patient management. This review summarizes reported esophageal SGTs (Table 1) with particular emphasis on controversies with regard to histology and classification. Attention is also paid to immunohistochemical markers and molecular alterations that can aid in the
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