What is the prevalence of gastroesophageal (GE) cancer and what is its corresponding Orphanet (ORPHA) code?

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Last updated: May 27, 2025View editorial policy

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From the Guidelines

Gastroesophageal cancer is not considered rare, with esophageal cancer being a significant cause of cancer deaths worldwide, as stated in the 2020 study by the Journal of Clinical Oncology 1.

Definition and Classification

Gastroesophageal cancer refers to cancers that occur at the junction of the esophagus and stomach. According to the provided evidence, esophageal cancer is the sixth most common cancer worldwide, with an estimated 450,000 deaths per year 1. This suggests that gastroesophageal cancer is relatively common and not rare.

Orphanet Codes

Since gastroesophageal cancers are not classified as rare diseases, they do not have specific Orphanet (ORPHA) codes. ORPHA codes are specifically assigned to rare diseases, which are typically defined in Europe as conditions affecting fewer than 1 in 2,000 people.

Medical Coding

For medical coding purposes, you would instead use ICD-10 codes such as:

  • C16 for malignant neoplasm of stomach
  • C15 for malignant neoplasm of esophagus If you're specifically looking for the gastroesophageal junction cancer, you would use:
  • C16.0 (cardia of stomach)
  • C15.5 (lower third of esophagus) These standard diagnostic codes are used universally for insurance, research, and healthcare documentation purposes rather than ORPHA codes, which are reserved for truly rare conditions.

Prevalence and Incidence

The incidence of esophageal cancer represents one of the widest variations, with a 60-fold difference between high- and low-incidence regions, as reported in the 2015 study by the Journal of the National Comprehensive Cancer Network 1. High-prevalence areas include Asia, southern and eastern Africa, and northern France. In Western countries, the most common site of esophageal cancer is in the lower third of the esophagus, which often involves the gastroesophageal junction.

Key Points

  • Gastroesophageal cancer is not rare
  • Use ICD-10 codes for medical coding purposes
  • ORPHA codes are not applicable for gastroesophageal cancer
  • Esophageal cancer is a significant cause of cancer deaths worldwide, with varying incidence rates across different regions 1

From the Research

Gastroesophageal Cancer Incidence

  • Gastroesophageal cancer is not considered rare, with an estimated 16,910 new cases and 15,910 deaths projected in 2016 in the United States 2.
  • The incidence rate of adenocarcinoma of the esophagogastric junction is increasing in association with the epidemiologic rise in distal esophageal adenocarcinoma and gastric cardial tumors 3.
  • Esophageal cancer has a poor prognosis and high mortality rate, with squamous cell carcinoma and adenocarcinoma accounting for more than 95% of esophageal cancers 2.

Orpha Code

  • The Orpha code for gastroesophageal cancer is not explicitly mentioned in the provided studies.
  • However, the studies provide information on the classification, staging, and treatment of gastroesophageal cancer, which may be useful for determining the relevant Orpha code.

Epidemiology and Classification

  • Gastroesophageal cancer (GEC) comprises proximal esophagogastric junction (EGJ) and distal gastric cancer (GC), with a significant change in epidemiology over the past several decades, especially in developed countries 4.
  • The changing epidemiology is thought to be mainly due to changing trends of risk factors such as lower incidence of Helicobacter pylori infection and increasing incidence of obesity and gastroesophageal reflux 4.
  • Histologically, EGJ cancers are adenocarcinoma (AC), while distal esophagus may be squamous cell carcinoma (SCC) or AC 4.

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Esophageal Cancer.

American family physician, 2017

Research

Gastroesophageal junction adenocarcinoma.

Current treatment options in oncology, 2000

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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