Esophagus
Carcinoma
Neuroendocrine tumor / carcinoma


Topic Completed: 1 July 2013

Revised: 27 February 2019

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

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


Feriyl Bhaijee, M.D.
Israh Akhtar, M.D.
Page views in 2018: 3,450
Page views in 2019 to date: 5,152
Cite this page: Bhaijee F, Akhtar I. Neuroendocrine tumor / carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagusneuroendocrine.html. Accessed December 5th, 2019.
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

AFIP images

Small cell carcinoma of esophagus

 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

Contributed by Dr. Mark R. Wick

Neuroendocrine tumor



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
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