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Table of Contents
Definition / general | Epidemiology | Sites | Pathophysiology | Clinical features | Diagnosis | Radiology description | Prognostic factors | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy images | Differential diagnosis | Additional referencesCite this page: Weisenberg, E. Adenocarcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagusadenocarcinoma.html. Accessed July 22nd, 2018.
Definition / general
- Malignant gland forming tumor of esophagus
Epidemiology
- In developed world, incidence has increased, while incidence of squamous cell carcinoma has remained constant (Ann Oncol 2012;23:3155, J Natl Compr Canc Netw 2011;9:830)
- 50 - 70% of esophageal carcinomas are adenocarcinoma
- In 2013, American Cancer Society estimates 17,990 new cases of esophageal carcinoma in US with 15,210 deaths
- Mean age is ~60 years old; cases before age 50 are uncommon
- ~80% men
- While the most substantial increase has been among white men, incidence is increasing in women and men of other ethnicities
- Obesity and high BMI are strong risk factors, likely related to increased GERD and Barrett esophagus
- Barrett esophagus is most important risk factor
- Tobacco is moderate risk factor
- Infection with cagA+ Helicobacter pylori appears to be protective against reflux esophagitis, the initial step in development of adenocarcinoma (pathway is reflux to Barrett esophagus to dysplasia to adenocarcinoma); increase in adenocarcinoma may be due, in part, to decline in prevalence of Helicobacter pylori (Cancer Res 1998;58:588, Cancer Prev Res (Phila) 2008;1:329)
- H. pylori infection may cause atrophic gastritis, decreasing acid production and thus decreasing reflux disease
Sites
- Generally in distal esophagus
- Rarely may arise in submucosal glands or ectopic gastric mucosa
Pathophysiology
- Dysplasia - carcinoma sequence in Barrett mucosa with stepwise accumulation of genetic mutations, especially p53 gene; additional changes involve HER2 / c-ERBB2, cyclin D1, cyclin E genes, RB, p16 genes
Clinical features
- Insidious onset, dysphagia to solids, followed by dysphagia to all food
- Extreme weight loss due to loss of nutrition and the tumor itself
- Metastasis generally occurs early even in superficial tumors, due to extensive lymphatic network in esophagus that allows horizontal and longitudinal spread
- Adenocarcinoma occurs in lower esophagus and lymph node metastases involve gastric and celiac lymph nodes
- Visceral metastases to liver, lungs, pleura
- Recurrences are common
Diagnosis
- Overwhelming majority diagnosed by endoscopic biopsy
Radiology description
- Imaging reveals mass in distal esophagus
Prognostic factors
- Lymph node metastases, extracapsular lymph node involvement (Am J Surg Pathol 2006;30:171), depth of invasion (but see J Clin Oncol 2007;25:507), status of resection margins; possibly endoglin / CD105 (Hum Pathol 2005;36:955)
- Postchemoradiation therapy prognostic factors in resection specimens:
- Extent of residual carcinoma predicts survival based on 3 groups:
- P0 (0% residual carcinoma)
- P1 (1 - 50% residual carcinoma)
- P2 (> 50% residual carcinoma, Am J Surg Pathol 2007;31:58)
- Prominent mucin pools in patients with Barrett associated adenocarcinoma are associated with mucinous tumors but acellular mucin pools are NOT associated with poor survival, even if at radial margin (Am J Surg Pathol 2006;30:28)
- Extent of residual carcinoma predicts survival based on 3 groups:
Case reports
- 51 year old man with collision tumor with papillary adenocarcinoma and neuroendocrine carcinoma (Arch Pathol Lab Med 2000;124:411)
Treatment
- 5 year survival for nonresectable tumors is rare
- Resection if primary treatment modality (if possible); may be preceded by neoadjuvant therapy - report Barrett metaplasia on proximal margin, if present
- Adjuvant chemoradiation often given
Gross description
- Usually distal esophageal tumor with invasion of gastric cardia; appears as flat patches to nodular masses; may have adjacent Barrett mucosa
Gross images
Images hosted on PathOut server:
Adenocarcinoma in Barrett esophagus
Images contributed by Dr. Mark R. Wick:
In Barrett metaplasia
Images hosted on other servers:
Polypoid tumor
Images contributed by Dr. Elliot Weisenberg:
Microscopic (histologic) description
- Usually moderate or well differentiated, usually mucin producing (intestinal type mucosa), may have foci of squamous or endocrine differentiation
- Usually has adjacent Barrett mucosa with high grade dysplasia (may be displaced by adenocarcinoma)
- Rarely signet ring cells, papillary structures, Paneth cells, endocrine cells, pagetoid spread of tumor cells
Microscopic (histologic) images
Images hosted on PathOut server:
Arising from Barrett epithelium:
Various images
Images contributed by Dr. Mark R. Wick:
Metaplasia
Images hosted on other servers:
Arising from
Barrett epithelium:
moderately
differentiated
Adenocarcinoma / neuroendocrine carcinoma collision tumor
Images contributed by Dr. Elliot Weisenberg:
In lymphatics
Positive stains
- Rarely needed for diagnosis
- PAS, Alcian blue and mucicarmine (for mucin), usually CK7 and CK19 (Am J Surg Pathol 2002;26:1213), AMACR (Hum Pathol 2006;37:1601); focal positivity is common for somatostatin and serotonin (Histopathology 1987;11:53)
Negative stains
Electron microscopy images
Images hosted on other servers:
Adenocarcinoma (site unspecified)
Differential diagnosis
- High grade dysplasia (no single cell invasion, no infiltration of submucosa or muscularis mucosae), metastatic or adjacent gastric carcinoma (signet ring cells may be present; often CK20+ in background of intestinal metaplasia)
- Note per AJCC criteria, cancers of the gastroesophageal junction are staged as esophageal carcinomas
Additional references
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