Well differentiated neuroendocrine tumor

Last author update: 1 July 2013
Last staff update: 5 May 2022 (update in progress)

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PubMed Search: Neuroendocrine carcinoid tumor esophagus

Feriyl Bhaijee, M.D.
Israh Akhtar, M.D.
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Cite this page: Bhaijee F, Akhtar I. Well differentiated neuroendocrine tumor. website. Accessed February 7th, 2023.
Definition / general
  • Well differentiated epithelial neoplasm with prominent neuroendocrine differentiation
  • Pathologic spectrum from low grade neuroendocrine tumor (carcinoid, atypical carcinoid) to high grade neuroendocrine carcinoma (small or large cell carcinoma)
  • Synonym: neuroendocrine tumor (low grade) = carcinoid tumor, atypical carcinoid tumor
  • Extremely rare in esophagus: < 50 reported cases
  • 0.002% of all carcinoid tumors
  • Wide age range: 30 - 82 years, average age 60 years
  • M:F = 6:1
  • Distal esophagus
Clinical features
  • Usually incidental / unexpected finding on radiologic studies or upper GI endoscopy
  • Dysphagia is most common symptom
  • Carcinoid syndrome is rare
  • Endoscopic biopsy
Prognostic factors
  • Mitotic rate and Ki67 index determine grade
  • Low grade lesions have favorable prognosis
Case reports
  • Surgical resection
Gross description
  • Polypoid or ulcerated mass on upper endoscopy
Microscopic (histologic) description
  • Well differentiated (low grade) tumor
    • Uniform, small, bland tumor cells in solid, trabecular, gyriform or glandular pattern
    • May have Paneth cell differentiation
  • Solid to cribriform growth
  • Usually in lamina propria
Microscopic (histologic) images

Images hosted on other servers:

Small bowel carcinoid

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
Cytology images

Images hosted on other servers:

Carcinoid tumor

Positive stains
Negative stains
Electron microscopy images

Images hosted on other servers:

Neurosecretory granules

Differential diagnosis
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