Definition/General
Introduction:
Cervical carcinoid (neuroendocrine tumor) is an extremely rare malignant neuroendocrine neoplasm arising in the cervix
It demonstrates neuroendocrine differentiation and has potential for hormone secretion.
Origin:
Arises from neuroendocrine cells within the cervical epithelium or from pluripotent stem cells that undergo neuroendocrine differentiation
May be primary or metastatic.
Classification:
WHO Classification categorizes as well-differentiated neuroendocrine tumor (Grade 1-2)
Distinguished from poorly differentiated neuroendocrine carcinoma by grade and behavior.
Epidemiology:
Extremely rare, <0.1% of cervical tumors
Peak incidence 40-60 years
No clear predisposing factors
May be associated with multiple endocrine neoplasia syndromes.
Clinical Features
Presentation:
Abnormal vaginal bleeding
Cervical mass or enlargement
Rarely carcinoid syndrome if functional
Often asymptomatic until advanced.
Symptoms:
Abnormal vaginal bleeding (most common)
Vaginal discharge
Pelvic pain
Carcinoid syndrome rare (flushing, diarrhea, bronchospasm).
Risk Factors:
No clear risk factors identified
Possible association with MEN syndromes
Not related to HPV infection unlike other cervical cancers.
Screening:
No specific screening
Imaging shows solid mass
Octreotide scan may be positive
Serum chromogranin A, synaptophysin levels.
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Gross Description
Appearance:
Well-circumscribed, firm, yellow to tan colored mass
Homogeneous cut surface
May have areas of hemorrhage but necrosis uncommon.
Characteristics:
Size variable (1-8 cm)
Firm consistency
Yellow to tan color
Well-demarcated borders
Homogeneous appearance.
Size Location:
Can arise from any part of cervix
Often involves full thickness of cervical wall
May extend to parametrium in advanced cases.
Multifocality:
Usually solitary lesion
May be part of multiple endocrine neoplasia syndrome with other neuroendocrine tumors.
Microscopic Description
Histological Features:
Uniform small to medium-sized cells arranged in nests, trabeculae, or ribbon-like patterns
Cells have round nuclei with stippled chromatin and moderate cytoplasm.
Cellular Characteristics:
Monotonous small to medium cells
Round to oval nuclei with salt-and-pepper chromatin
Moderate eosinophilic cytoplasm
Minimal pleomorphism.
Architectural Patterns:
Nested, trabecular, or ribbon-like growth patterns
May show insular or solid areas
Minimal stroma between cell nests.
Grading Criteria:
WHO grading based on mitotic count and Ki-67: Grade 1 (<2 mitoses/10 HPF, Ki-67 <3%), Grade 2 (2-20 mitoses/10 HPF, Ki-67 3-20%).
Immunohistochemistry
Positive Markers:
Chromogranin A positive
Synaptophysin positive
CD56 positive
Neuron-specific enolase (NSE) positive.
Negative Markers:
Cytokeratin may be focal positive
TTF-1 negative (unlike pulmonary carcinoids)
CDX2 negative (unlike GI carcinoids).
Diagnostic Utility:
Chromogranin A and synaptophysin confirm neuroendocrine differentiation
CD56 supports diagnosis
Essential for confirming neuroendocrine nature.
Molecular Subtypes:
No specific molecular subtypes
May express various hormones (serotonin, ACTH, gastrin) detectable by immunohistochemistry.
Molecular/Genetic
Genetic Mutations:
Limited data on cervical carcinoids
May have MEN1 gene mutations if associated with MEN syndrome
Different from pulmonary or GI carcinoids.
Molecular Markers:
Low to moderate Ki-67 proliferation index (<20%)
Normal p53 expression
Hormone receptors variable.
Prognostic Significance:
Generally better prognosis than other cervical cancers if low grade
Size, grade, and stage important prognostic factors.
Therapeutic Targets:
Surgery is primary treatment
Somatostatin analogues for functional tumors
Peptide receptor radionuclide therapy (PRRT) for advanced cases.
Differential Diagnosis
Similar Entities:
Poorly differentiated neuroendocrine carcinoma
Metastatic carcinoid from other sites
Small cell carcinoma
Paraganglioma
Adenocarcinoma with neuroendocrine features.
Distinguishing Features:
Well-differentiated carcinoid: low grade, uniform cells
Small cell carcinoma: high grade, high mitoses, necrosis
Metastatic: clinical history.
Diagnostic Challenges:
Distinction from metastatic carcinoid
Differentiation from other neuroendocrine tumors
Recognition of neuroendocrine differentiation.
Rare Variants:
Atypical carcinoid
Mixed neuroendocrine-adenocarcinoma
Goblet cell carcinoid.
Sample Pathology Report
Template Format
Sample Pathology Report
Complete Report: This is an example of how the final pathology report should be structured for this condition.
Specimen Information
[specimen type], measuring [size] cm in greatest dimension
Diagnosis
[diagnosis name]
Classification
Classification: [classification system] [grade/type]
Histological Features
Shows [architectural pattern] with [nuclear features] and [mitotic activity]
Size and Extent
Size: [X] cm, extent: [local/regional/metastatic]
Margins
Margins are [involved/uninvolved] with closest margin [X] mm
Lymphovascular Invasion
Lymphovascular invasion: [present/absent]
Lymph Node Status
Lymph nodes: [X] positive out of [X] examined
Special Studies
IHC: [marker]: [result]
Molecular: [test]: [result]
[other study]: [result]
Prognostic Factors
Prognostic factors: [list factors]
Final Diagnosis
Final diagnosis: [complete diagnosis]