Small cell neuroendocrine carcinoma

Last author update: 1 February 2014
Last staff update: 14 June 2022

Copyright: 2003-2023,, Inc.

PubMed Search: Small cell neuroendocrine carcinoma[mh] esophagus[TI] full text[sb]

Elliot Weisenberg, M.D.
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Cite this page: Weisenberg E. Small cell neuroendocrine carcinoma. website. Accessed September 30th, 2023.
Definition / general
  • Small cell neuroendocrine carcinoma is analogous to pulmonary small cell carcinoma
  • WHO definition is:
    • Malignant epithelial tumor consisting or small cells with scant cytoplasm, ill defined cell borders, finely granular nuclear chromatin and absent or inconspicuous nucleoi
    • Cells are round, oval and spindle shaped
    • Nuclear molding is prominent
    • Necrosis is typically extensive and the mitotic count is high
  • May be called small cell endocrine carcinoma or high grade endocrine carcinoma (small cell type)
  • Terms oat cell carcinoma, small cell anaplastic carcinoma and undifferentiated small cell carcinoma are generally considered obsolete
  • Uncommon, about 1% of esophageal malignancies but most common site of small cell carcinoma outside the lung
  • Patients usually over 50, male predominance, approximately 2:1
  • Associated with heavy cigarette smoking
  • More common in lower esophagus
  • Likely origin from pleuripotent stem cells
  • Admixed squamous cell carcinoma is common (Hum Pathol 1984;15:460); evidence of squamous or glandular differentiation in half and squamous cell carcinoma in 1/3 (Hum Pathol 1999;30:216)
  • Tissue biopsy
Radiology description
Radiology images

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Tumor of the lower esophagus

Prophylactic irradiation

Esophagogram before and after treatment

Case reports
Clinical images

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Endoscopic picture before and after treatment

Gross images

AFIP images

Bulky, ulcerated, infiltrative lesion

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Small cell
carcinoma (upper),
squamous cell
carcinoma (lower)

Microscopic (histologic) description
  • Identical to pulmonary counterpart (see definition above), may have admixed squamous cell carcinoma, adenocarcinoma or mucoepidermoid carcinoma
  • May have rosettes, carcinoid type features and focal mucin production, encrustation of vessel walls by tumor DNA (Azzopardi phenomenon), crush artifact
Microscopic (histologic) images

AFIP images

Diffusely infiltrating sheets of small cells

In situ component

Squamous cell differentiation

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Ribbons and rosettes

Ribbons of cells with angular hyperchromatic nuclei

Spindle cells with
scant cytoplasm,
salt and pepper nuclei
with indistinct nucleoli

Nested or organoid growth pattern most common

With squamous cell carcinoma

Paraesophageal lymph nodes

Immunostains - CD56, Syn, CK and EMA

Cytology description
  • Small malignant cells with scant cytoplasm and nuclear molding
Positive stains
Electron microscopy images

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Dense core neurosecretory granules

Molecular / cytogenetics description
Differential diagnosis
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