Esophagus
Carcinoma
Small cell neuroendocrine carcinoma
Author: Elliot Weisenberg, M.D.
Last author update: 1 February 2014
Last staff update: 14 June 2022
Copyright: 2003-2023, PathologyOutlines.com, Inc.
PubMed Search: Small cell neuroendocrine carcinoma[mh] esophagus[TI] full text[sb]
Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Pathophysiology | Clinical features | Diagnosis | Radiology description | Radiology images | Case reports | Treatment | Clinical images | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Electron microscopy images | Molecular / cytogenetics description | Differential diagnosisCite this page: Weisenberg E. Small cell neuroendocrine carcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/esophagussmallcell.html. 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
Terminology
- 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
Epidemiology
- 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
Sites
- More common in lower esophagus
Pathophysiology
- 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)
Clinical features
- Very aggressive (Dis Esophagus 2014;27:152, Hepatogastroenterology 2005;52:1738)
- Median survival 18 months (BMC Cancer 2007;7:38)
- 5 year survival 8% or less (Chin Med J (Engl) 2007;120:355, Ann Surg Oncol 2013;20:4239, Hum Pathol 1999;30:216)
Diagnosis
- Tissue biopsy
Radiology description
- FDG PET scans may determine extent of disease (Indian J Cancer 2005;42:60)
Radiology images
Images hosted on other servers:
Tumor of the lower esophagus
Prophylactic irradiation
Esophagogram before and after treatment
Case reports
- 63 year old woman with coexisting paraneoplastic neurological syndrome (Jpn J Clin Oncol 2006;36:109)
- 66 year old man with rare collision tumor of squamous carcinoma and small cell carcinoma in esophagus in separate lymph nodes (J Thorac Dis 2013;5:E203)
- 79 year old man (BMJ Case Rep 2013 Sep 3;2013)
Treatment
- Chemoradiation
- Resection if feasible, possibly endoscopic mucosal resection if superficial (Gan To Kagaku Ryoho 2007;34:81)
Clinical images
Images hosted on other servers:
Endoscopic picture before and after treatment
Gross images
AFIP images
Bulky, ulcerated, infiltrative lesion
Images hosted on other servers:
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
Images hosted on other servers:
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
- Synaptophysin (95%), CD56 (76%), TTF1 (71%)
- Neuron specific enolase (62%), chromogranin A (62%), EMA (62%), AE1 / AE3 (57%)
- Also p53 (81%), BCL2 (33%), S100 (19%)
Electron microscopy images
Images hosted on other servers:
Dense core neurosecretory granules
Molecular / cytogenetics description
- High incidence of PTEN mutations (BMC Cancer 2014;14:19)
Differential diagnosis
- Basaloid squamous cell carcinoma
- Carcinoid tumor: also called neuroendocrine tumor G1 (carcinoid), G2
- Lymphoma
- Metastatic lung carcinoma
