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Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Prognostic factors | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Differential diagnosis | Additional referencesCite this page: Neuroendocrine carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/colontumorneuroendocrine.html. Accessed July 14th, 2017.
Definition / general
- Rare, high grade neuroendocrine malignancy of the colorectum with poor prognosis (Am J Surg Pathol 2008;32:719)
- Excludes well differentiated neuroendocrine tumors (also known as carcinoid tumors)
Terminology
- Two types, based on morphology: large cell neuroendocrine carcinoma (discussed herein) and small cell carcinoma (discussed here)
Epidemiology
- Disease typically presents at advanced stage, in patients often younger than those with colorectal adenocarcinoma (Dis Colon Rectum 2004;47:163)
Sites
- Can arise anywhere along the colon or rectum
Prognostic factors
- Good prognostic factors include peritumoral lymphocytes, microsatellite instability and pure large cell features; poor prognostic factors include CD117 positivity and vascular invasion (Am J Surg Pathol 2012;36:601)
Clinical images
Images hosted on other servers:
Rectal mass
Noncontrast CT scans of abdomen
Microscopic (histologic) description
- Organoid arrangement of cells larger than those in small cell carcinoma, with nuclear pleomorphism and hyperchromasia, prominent nucleoli, numerous mitoses and tumor necrosis
Microscopic (histologic) images
Images hosted on other servers:
H&E, synaptophysin, chromogranin, Ki67
Large cell neuroendocrine carcinoma
ChromograninA, CK20
Ampulla of vater
Cervix
Positive stains
- Cytokeratin, synaptophysin, chromogranin; high Ki67 index
- CD117 in 23%, though not associated with activating mutations (Am J Surg Pathol 2003;27:1551)
Differential diagnosis
- Mixed adenoneuroendocrine carcinoma (MANEC): tumors with neuroendocrine and adenocarcinoma components of at least 30% each (Cancers (Basel) 2012;4:11)
- Adenocarcinoma with scattered neuroendocrine cells: common; histology resembles traditional colorectal adenocarcinoma (Arch Pathol Lab Med 1998;122:912)
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