Esophageal cancer

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 5 Oct 2024

Last updated: 22 Oct 2024

Esophageal cancer incidence is increasing across the developed world. Men are considerably more likely than women to develop the disease.

The two main histologic types are squamous cell carcinoma and adenocarcinoma. In the developed world, adenocarcinomas predominate.

Low socioeconomic status, smoking, excessive alcohol use, GERD, Barrett esophagus, and obesity are some of the main risk factors.

Tumors are often locally advanced at the time of diagnosis. Accurate staging is important for prognosis and treatment planning.

Superficial intramucosal well-differentiated esophageal cancer can be managed with an esophagus-sparing approach utilizing endoscopic resection and surveillance. Localized tumors that are not amenable to endoscopic resection, those with poor differentiation, lymphovascular invasion, or deeper submucosal invasion are often best treated by esophagectomy.

For locally advanced disease, combined modality therapy is considered the current standard. This involves chemotherapy or chemoradiation followed by surgery.

Patients without a complete pathologic response following resection may benefit from adjuvant immunotherapy.

Targeted therapies can be used in patients with metastatic esophageal and esophageal junction cancer.

Treatment decisions for patients with recurrent or refractory disease are informed by prior treatment history and multidisciplinary tumor board discussions.

Definition

Most esophageal cancers are neoplastic mucosal lesions that originate in the epithelial cells lining the esophagus.

Esophageal cancers are usually squamous cell carcinomas or adenocarcinomas.

Rarely, other cancers, such as melanoma, sarcoma, small cell carcinoma, or lymphoma, can occur in the esophagus.

History and exam

Key diagnostic factors

dysphagia
odynophagia
weight loss

Full details

Other diagnostic factors

hiccups
postprandial/paroxysmal cough

Full details

Risk factors

male sex
older age
tobacco use
excessive alcohol use (squamous cell carcinoma)
Barrett esophagus (adenocarcinoma)
GERD (adenocarcinoma)
hiatal hernia (adenocarcinoma)
family history of esophageal or other cancer (squamous cell carcinoma)
low socioeconomic status
nonwhite race (squamous cell carcinoma)
high-temperature beverages and foods (squamous cell carcinoma)
drinking maté (squamous cell carcinoma)
low intake of fresh fruit and vegetables
hereditary cancer syndromes
obesity (adenocarcinoma)
human papillomavirus (squamous cell carcinoma)
achalasia
vitamin and mineral deficiencies (squamous cell carcinoma)
poor oral hygiene (squamous cell carcinoma)

Full details

Diagnostic tests

1st tests to order

esophagogastroduodenoscopy (EGD) with biopsy
endoscopic ultrasound (EUS) ± fine needle aspiration (FNA)
CT thorax and abdomen
(18F)-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) scan
molecular and pathologic tests

Full details

Tests to consider

comprehensive metabolic panel
MRI thorax and abdomen
bronchoscopy ± fine needle aspiration (FNA)
thoracoscopy and laparoscopy
liquid biopsy
pulmonary function tests
cardiac stress test
echocardiogram

Full details

Treatment algorithm

ACUTE

limited disease (cT1, cN0, M0)

localized disease (cT2, cN0, M0): suitable for surgery

localized disease (cT2, cN0, M0): unsuitable for surgery

locally advanced disease (cT3-4, cN1-3, M0): suitable for surgery

locally advanced disease (cT3-4, cN1-3, M0): unsuitable for surgery

metastatic (M1) disease

ONGOING

recurrent disease

Contributors

Authors

Ravi Rajaram, MD MSc FACS

Assistant Professor

Thoracic and Cardiovascular Surgery

University of Texas MD Anderson Cancer Center

Houston

Disclosures

RR is a research consultant for Johnson & Johnson and Cook Medical.

Acknowledgements

Dr Ravi Rajaram would like to gratefully acknowledge Dr Naureen Starling, Dr Caroline Fong, Dr Mark J. Krasna, and Dr Ghulam Abbas, the previous contributors to this topic.

Disclosures

MJK is an author of several references cited in this topic. GA declares that he has no competing interests. NS has received research funding from AstraZeneca, BMS, and Pfizer; travel and accommodation funding from AstraZeneca, BMS, Eli Lilly, Merck, Roche, and MSD Oncology; honoraria from Eli Lilly, Merck Serono, MSD Oncology, Pierre Fabre, Servier, GSK, and Amgen. She has been on the advisory board for Pfizer, AstraZeneca, Servier, and MSD (Merck). NS is an Honorary Clinical Senior Lecturer within the Division of Clinical Studies at the Institute of Cancer Research and serves on the UK National Cancer Research Institute (NCRI) esophagogastric sub-group. NS has acted as a clinical expert in esophagogastric cancer for NICE (guideline committee and technology appraisal) and is an upper GI expert for International Cancer Benchmarking Partnership. She is a Trustee for Pancreatic Cancer UK and a member of the EORTC General Assembly representing The Royal Marsden, as well as a member of the European Society for Medical Oncology (ESMO) Gastrointestinal Faculty. Educational roles include the NIHR Training Lead for NIHR Biomedical Research Centre, member of the Cancer Research Centre of Excellence training committee, Deputy Training Program Director (one of three) for South London Medical Oncology Training, member of the pan-London specialist Medical oncology training committee, and member of the Medical Oncology National Recruitment steering committee. CF declares that she has received honoraria from Bristol Myers Squibb.

Peer reviewers

Peter McCulloch, MBChB, MA, MD, FRCS (Ed), FRCS (Glas)

Clinical Reader in Surgery

Nuffield Department of Surgery

University of Oxford

Oxford

Disclosures

PM declares that he has no competing interests.

Nikhil I. Khushalani, MD

Assistant Professor of Oncology

Roswell Park Cancer Institute

Buffalo

Disclosures

NIK has received funding for the conduction of clinical trials and associated translational studies from Merck, Pfizer, and Astra-Zeneca. NIK has a grant from the National Comprehensive Cancer Network (from research support by Roche).

Differentials
- Benign stricture
- Achalasia
- Barrett esophagus
More Differentials
Guidelines
- NCCN clinical practice guidelines in oncology: esophageal and esophagogastric junction cancers
- NCCN clinical practice guidelines in oncology: management of immunotherapy-related toxicities
More Guidelines
Patient information
Quitting smoking
Esophageal cancer
More Patient information
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Esophageal cancer

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic tests

1st tests to order

Tests to consider

Treatment algorithm

limited disease (cT1, cN0, M0)

localized disease (cT2, cN0, M0): suitable for surgery

localized disease (cT2, cN0, M0): unsuitable for surgery

locally advanced disease (cT3-4, cN1-3, M0): suitable for surgery

locally advanced disease (cT3-4, cN1-3, M0): unsuitable for surgery

metastatic (M1) disease

recurrent disease

Contributors

Authors

Ravi Rajaram, MD MSc FACS

Disclosures

Acknowledgements

Disclosures

Peer reviewers

Peter McCulloch, MBChB, MA, MD, FRCS (Ed), FRCS (Glas)

Disclosures

Nikhil I. Khushalani, MD

Disclosures

Differentials

Guidelines

Patient information

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