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Esophagus

Carcinoma

Esophageal carcinoma-overview

Author: Elliot Weisenberg, M.D.

Last author update: 1 June 2013

Last staff update: 13 December 2023

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

PubMed Search: Carcinoma esophagus[TIAB]

Table of Contents

Cite this page: Weisenberg E. Esophageal carcinoma-overview. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/esophaguscarcinomageneral.html. Accessed January 10th, 2025.

Definition / general

< 1% of all cancers in US, ~6% of gastrointestinal cancers
Disproportionate numbers of deaths as most patients are asymptomatic during early stages and present with advanced or metastatic disease (Wikipedia: Esophageal Cancer [Accessed 25 February 2019], eMedicine: Esophageal Cancer [Accessed 25 February 2019])
Worldwide, 90% are squamous cell carcinoma
In US and Western Europe, has been a shift from squamous cell carcinoma to adenocarcinoma
Incidence of adenocarcinoma has been increasing, especially in white men and is now higher than squamous cell carcinoma in US (NIH: Esophageal Cancer [Accessed 25 February 2019]), France (Gastroenterol Clin Biol 2005;29:1258) and elsewhere
Estimated 17,990 new cases with 15,210 deaths in US in 2013 (CA Cancer J Clin 2013;63:11)

Risk factors

In developed world, smoking and drinking are most important risk factors
Other risk factors are diet, especially some hot liquids, Plummer-Vinson syndrome, achalasia, stricture, webs, rings, diverticula
Higher incidence among African Americans, city dwellers, poor
"Esophageal carcinoma belt" extends from Northeast China to Middle East, with high incidence of squamous cell carcinoma
Adenocarcinoma associated with Barrett esophagus

Sites

Squamous cell carcinoma: 20% upper, 50% middle, 30% lower esophagus
Adenocarcinoma mostly in distal esophagus
Adenocarcinoma rarely arises in heterotopic gastric mucosa or submucosal glands

Pathophysiology

Adenocarcinoma: dysplasia - carcinoma sequence occurs in Barrett mucosa with stepwise accumulation of genetic mutations, especially p53, also HER2 / cERB-B2, cyclin D1, cyclin E, RB, p16
Squamous cell carcinoma: genetic alterations include p53, p16^INK4a, amplification of cyclin D1, cMYC and EGFR; related to smoking, alcohol, diet, possible nutritional deficiency, genetic factors

Clinical features

Insidious onset, dysphagia to solids, followed by dysphagia to all food
Extreme weight loss due to loss of nutrition and tumor itself
Squamous cell carcinoma may erode through esophagus; invades respiratory tree with fistula formation and pneumonia; aorta with exsanguination; also invades mediastinum or pericardium
Metastasis generally occurs early even in superficial tumors due to extensive lymphatic network in esophagus that allows horizontal and longitudinal spread
Cancers of upper esophagus metastasize to cervical lymph nodes; of mid esophagus to mediastinal, paratracheal and tracheobronchial lymph nodes; of lower esophagus to gastric and celiac lymph nodes
Metastases to liver, lungs and pleura
Recurrences are common

Diagnosis

Endoscopic biopsy
Rarely identified during resection for achalasia or for lesions not amenable to biopsy

Radiology description

Stenosis or obstruction
Protruding mass may be detected

Overall 5 year survival

~ 14% (Oncologist 2005;10:590) but 47% after resection (J Am Coll Surg 2006;202:588, Dis Esophagus 2009;22:1)
Adenocarcinoma: < 20%
Squamous cell carcinoma: ~9%; early detection raises to 75%
~0% in stage IV disease
Presence of lymph node metastases at time of surgery greatly reduces survival

Case reports

47 year old man with esophageal adenocarcinoma arising from Barrett dysplasia (Clin Med Res 2006;4:184)
53 year old man with advanced middle esophageal cancer with multiple regional lymph node metastases (Jpn J Clin Onc 2002;32:310)

Treatment

Generally resection if possible
Sometimes adjuvant chemoradiation prior to resection
Chemoradiation may achieve results similar to surgery (Ann Thorac Cardiovasc Surg 2006;12:234)
Recommended to submit proximal margins for frozen section to rule out carcinoma underneath a normal appearing mucosa (Arch Pathol Lab Med 2005;129:1558)
Occasionally HER2+, although this is not related to survival (Mod Pathol 2007;20:120) and anti-HER2 therapy does not appear to be effective (Int J Radiat Oncol Biol Phys 2007;67:405)

Gross description

Adenocarcinoma is located in distal esophagus and may involve gastric cardia (note: tumors in the proximal 5 cm of the stomach are regarded as esophageal carcinomas in Edge: AJCC Cancer Staging Manual, 7th Edition, 2011)
Flat or raised patches of intact mucosa develop into nodular masses
Squamous cell carcinoma begins as an in situ process
Generally starts as plaque-like lesions which grow to eventually encircle the lumen
Mostly form protruding cauliflower-like lesions (60%), may be flat (15%) or ulcerated

Gross images

Contributed by Elliot Weisenberg, M.D.

Adenocarcinoma

Squamous cell carcinoma

Microscopic (histologic) description

Overwhelming majority of carcinomas of esophagus are adenocarcinoma and squamous cell carcinoma
Diagnosis usually simple
Generally mucin secreting adenocarcinomas, less often signet ring cell carcinoma
Usually foci of dysplastic mucosa adjacent to cancer
Squamous cells carcinoma tends to be well or moderately differentiated; histologic variants include verrucous, spindle cell or basaloid
Other carcinomas are adenoid cystic carcinoma, adenosquamous carcinoma, mucoepidermoid carcinoma
Special stains only rarely needed for diagnosis

Microscopic (histologic) images

Contributed by Elliot Weisenberg, M.D.

Adenocarcinoma

Adenocarcinoma in lymphatics

Squamous cell carcinoma in situ

Differential diagnosis

Chemoradiation induced atypia: enlarged hyperchromatic nuclei with prominent mitotic figures but relatively mild pleomorphism in inflammatory background
Reparative processes: atypical cells in granulation tissue but with fine chromatin, few mitotic figures; cells mature at deeper levels, keratin negative

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