Large cell neuroendocrine carcinoma

Topic Completed: 1 October 2013

Revised: 12 September 2019

Copyright: 2003-2019,, Inc.

PubMed Search: Large cell neuroendocrine carcinoma esophagus

Elliot Weisenberg, M.D.
Page views in 2019: 541
Page views in 2020 to date: 101
Cite this page: Weisenberg E. Large cell neuroendocrine carcinoma. website. Accessed March 31st, 2020.
Definition / general
  • Poorly differentiated neoplasm composed of medium to large cells with neuroendocrine differentiation arising in esophagus
  • Per WHO criteria, at least 70% neuroendocrine tumor
  • Tumors with at least 30% adenocarcinoma (rarely squamous) and 30% neuroendocrine carcinoma are classified as Mixed AdenoNeuroEndocrine Carcinoma (MANEC)
  • Exceedingly rare
  • Strong male predominance
  • Cases reported 3 - 82 years but most 50 - 69 years old
  • Most in lower esophagus
  • MANEC may be associated with Barrett esophagus and adenocarcinoma (very rarely squamous cell carcinoma)
  • However, paucity of cases makes hypothesis of relationship only speculative
  • Arise from endocrine cells in esophagus (more common in distal esophagus)
Clinical features
  • Highly aggressive
  • Usually metastasis on presentation
  • Survival usually months
  • Endoscopic biopsy
Case reports
  • Surgical debulking, radiation, chemotherapy but response poor
Gross description
  • Large, exophytic ulcerated masses, usually > 5 cm
Microscopic (histologic) description
  • Solid nests or acinar structures
  • Cells medium to large size
  • Low N:C ratio, often abundant cytoplasm
  • Vesicular chromatin, visible nucleoli
  • Foci of necrosis
  • Many mitotic figures
Microscopic (histologic) images

Contributed by Dr. Mark R. Wick

In muscularis

Images hosted on other servers

Collision tumor

Rosette formation

Synaptophysin, CD57

Cytology description
  • Same as large cell neuroendocrine carcinoma at other sites
Cytology images

Large cell neuroendocrine carcinoma

Electron microscopy description
  • Membrane bound neurosecretory granules
Electron microscopy images

Images hosted on other servers:

Desmosomal intercellular junctions

Differential diagnosis
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