Esophagus
Carcinoma
Adenocarcinoma of gastroesophageal junction


Topic Completed: 1 June 2013

Revised: 25 February 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: Gastroesophageal junction adenocarcinoma[TI]

See also: Adenocarcinoma, Arising in ectopic gastric mucosa, Intramucosal carcinoma

Elliot Weisenberg, M.D.
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Cite this page: Weisenberg E. Adenocarcinoma of gastroesophageal junction. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagusadenocarcinomagastroesophageal.html. Accessed October 22nd, 2019.
Definition / general
  • Per WHO criteria published in 2010, adenocarcinomas that cross the gastroesophageal junction (GEJ) regardless of where the bulk of the tumor lies
  • Considered esophageal if entirely above GEJ; considered gastric if entirely below GEJ
  • Recommended to NOT use terminology "carcinoma of the gastric cardia"
  • Most literature describes GEJ as proximal extent of gastric folds
  • Edge: AJCC Cancer Staging Manual, 7th Edition, 2010 includes carcinomas of GEJ within the category "lower thoracic esophagus / esophagogastric junction" bordered superiorly by the inferior pulmonary veins and inferiorly by the stomach (Ann Surg Oncol 2010;17:1471)
  • Per AJCC criteria, cancers whose epicenter is in the lower thoracic esophagus, the GEJ or within the proximal 5 cm of the stomach are stage grouped as adenocarcinoma of esophagus
Terminology
  • Siewert classification is commonly used to characterized adenocarcinoma of the GEJ, especially in surgical literature where exact location of tumors of the GEJ may affect management
  • Type I arise in the distal esophagus, are associated with intestinal metaplasia and extend distally
  • Type II originates in the true GEJ
  • Type III is the from subcardial stomach and extends proximally
Epidemiology
  • Significant increase in incidence (similar to esophageal adenocarcinoma in general), although efforts to categorize tumors as either of gastric or esophageal origin hinder determination of exact incidence
  • This supports contention that pathophysiology of most adenocarcinomas of GEJ is similar to adenocarcinoma of esophagus
Pathophysiology
  • Most adenocarcinomas of GEJ arise in background of reflux esophagitis and follow Barrett metaplasia-dysplasia-carcinoma sequence
  • Small number occur in background of Helicobacter pylori gastritis with same presumed mechanisms as H. pylori associated gastric adenocarcinoma (Am J Surg Pathol 2007;31:569, Hum Pathol 2006;37:40)
  • Both mechanisms involve intestinal metaplasia
  • Very rarely, signet ring cell carcinoma occurs in GEJ; may have same underlying pathophysiology as gastric signet ring cell carcinoma
  • Classification as "collision tumor" based on histology is inaccurate - need molecular analysis (Am J Surg Pathol 2004;28:1492)
Clinical features
  • Dysphagia, pain, weight loss
  • Often symptoms of GERD, less often of peptic ulcer disease
Diagnosis
  • Endoscopic biopsy
Prognostic factors
  • Stage, especially presence of metastatic disease, completeness of resection, postsurgical complications
Case reports
Treatment
  • Surgical resection if possible, often with neoadjuvant therapy
  • Chemotherapy
Gross images

Contributed by Elliot Weisenberg, M.D.

Various images

Microscopic (histologic) description
  • Papillary, tubular, mucinous, signet ring cell, poorly cohesive without signet ring cells, mixed
  • Well to poorly differentiated
  • Greater tendency towards poorly differentiated tumors than esophageal adenocarcinoma
  • Often adjacent intestinal metaplasia / Barrett esophagus (Dis Esophagus 2007;20:36)
Microscopic (histologic) images

Contributed by Elliot Weisenberg, M.D.

Adenocarcinoma of GEJ



Contributed by Dr. Mark R. Wick

Signet ring cell

Positive stains
  • May be HER2 / neu positive, patients may be candidate for trastuzumab treatment
Molecular / cytogenetics description
  • p53 is most common mutation
  • Frequent deletions of 14q31-32.1 (Hum Pathol 2006;37:534)
  • Gains or losses of chromosomal loci have been reported involving 1q, 3p, 4q, 5q, 7p, 8q, 9q, 17q, 18q, 19q, 20pq, y
  • MYC, ERBB2, EGFR, APC, α catenin, SMAD4, DCC, APC, CDX2, RAS and Cdk6 may be affected
  • At present, molecular testing is NOT used to determine treatment
Differential diagnosis
  • Well differentiated adenocarcinoma may resemble hyperplastic or dysplastic lesions
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