Esophagus
Carcinoma
Neuroendocrine tumor / carcinoma

Author: Feriyl Bhaijee, M.D. (see Authors page)
Editor: Israh Akhtar, M.D.

Revised: 23 January 2018, last major update July 2013

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Neuroendocrine (tumor[TI] OR carcinoma[TI]) esophagus

Cite this page: Bhaijee, F. Neuroendocrine tumor / carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagusneuroendocrine.html. Accessed May 25th, 2018.
Definition / general
Terminology
  • Synonyms:
    • Neuroendocrine tumor (low grade) = carcinoid tumor, atypical carcinoid tumor
    • Neuroendocrine carcinoma (high grade) = small cell carcinoma, large cell carcinoma
Epidemiology
  • Extremely rare in esophagus: about 100 reported cases (mostly high grade / small cell carcinomas)
  • M:F ratio = 3:1 (Am J Surg Pathol 2013;37:467)
  • Mean age: 62 years
Clinical features
  • Usually incidental / unexpected finding on radiologic studies or upper GI endoscopy
  • Dysphagia, weight loss, chest pain with high grade carcinoma (Int J Clin Exp Pathol 2013;6:485)
  • Typically diagnosed via biopsy or (less commonly) surgical resection
Grading
Prognostic factors
  • Mitotic rate and Ki67 index determine grade
  • Low grade lesions have favorable prognosis
  • High grade carcinomas are very aggressive, as in other body sites
Case reports
Treatment
Gross description
  • Polypoid or ulcerated mass on upper endoscopy
Microscopic (histologic) description
  • Well differentiated (low grade) tumors
    • Uniform, small, bland tumor cells in solid, trabecular, gyriform or glandular patterns
    • May have Paneth cell differentiation
  • Poorly differentiated (high grade) carcinomas
    • Large cell type: nests of pleomorphic, large cells with prominent nucleoli and a moderate amount of cytoplasm
    • Small cell type: sheets and nests of small cells with hyperchromatic nuclei and a minimal to moderate amount of cytoplasm; prominent crush artifact and Azzopardi phenomenon, as in small cell carcinomas at other sites
    • Necrosis
    • Increased mitotic activity
    • Angiolymphatic invasion common
  • Solid to cribriform growth
  • Usually in lamina propria
  • May be associated with heterotopic oxyntic mucosa or Barrett esophagus (large cell carcinoma)
  • Neuroendocrine carcinoma may have small component(s) of adenocarcinoma or squamous cell carcinoma differentiation
Microscopic (histologic) images

Images hosted on PathOut server:

Small cell carcinoma of esophagus



Images contributed by Dr. Mark R. Wick:

In muscularis



Images hosted on other servers:

Carcinoid

Large cell neuroendocrine carcinoma and adenocarcinoma

Large cell: rosette formation

Cytology description
  • Low grade tumors:
    • Flat sheets or loosely cohesive groups / cords of monotonously uniform plasmacytoid cells
    • Eccentric nuclei, coarsely stippled (salt and pepper) chromatin, finely granular cytoplasm
  • High grade carcinomas:
    • Obvious pleomorphism, marked nuclear molding, hyperchromatic nuclei, inconspicuous nucleoli
    • Numerous mitoses, crush artifact, necrosis
    • Apoptotic figures, blue bodies
Cytology images

Images hosted on PathOut server:

Images contributed by Dr. Mark R. Wick:


Images hosted on other servers:

Carcinoid tumor

Small cell carcinoma

Positive stains
Negative stains
Electron microscopy images

Images hosted on other servers:

Neurosecretory granules

Differential diagnosis