Definition/General
Introduction:
Cervical micropapillary carcinoma is an extremely rare and aggressive variant of cervical adenocarcinoma characterized by small papillary clusters of malignant cells without fibrovascular cores.
Origin:
Arises from endocervical glandular epithelium showing micropapillary growth pattern
Represents a morphological variant associated with aggressive biological behavior.
Classification:
WHO Classification recognizes this as a rare variant of cervical adenocarcinoma
Defined by prominent micropapillary architecture (>50% of tumor).
Epidemiology:
Extremely rare variant of cervical adenocarcinoma
Peak incidence 40-60 years
Associated with particularly aggressive clinical behavior and poor prognosis.
Clinical Features
Presentation:
Abnormal vaginal bleeding
Advanced stage at presentation common
Cervical mass or enlargement
May present with metastatic disease.
Symptoms:
Abnormal vaginal bleeding (most common)
Vaginal discharge
Pelvic pain
Urinary or bowel symptoms
Weight loss
Abdominal distension if metastatic.
Risk Factors:
Similar to cervical adenocarcinoma: HPV infection, smoking, immunosuppression
May have additional unknown factors contributing to aggressive behavior.
Screening:
May be detected on Pap smear as atypical glandular cells
Often presents at advanced stage
Imaging shows extensive local or metastatic disease.
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Gross Description
Appearance:
Large, infiltrative mass with irregular borders
Cut surface shows solid, white-gray areas
Areas of necrosis and hemorrhage common.
Characteristics:
Size often large at presentation (>5 cm)
Firm consistency
White-gray color
Poorly circumscribed borders
Extensive local invasion common.
Size Location:
Often involves entire cervix at presentation
Frequent extension to parametrium, vagina, and corpus
Advanced local spread typical.
Multifocality:
Usually unifocal but may show extensive local spread
Satellite lesions possible
Metastatic deposits may be present.
Microscopic Description
Histological Features:
Small papillary clusters and micropapillary structures lacking fibrovascular cores
Cells show high-grade nuclear features with frequent mitoses.
Cellular Characteristics:
High-grade epithelial cells with enlarged, pleomorphic nuclei
Prominent nucleoli
Increased nuclear-to-cytoplasmic ratio
High mitotic activity.
Architectural Patterns:
Characteristic micropapillary pattern with small papillary clusters
Cells appear to float in clear spaces
May have associated conventional adenocarcinoma.
Grading Criteria:
High-grade adenocarcinoma by definition due to architectural pattern and nuclear features
Aggressive biological behavior.
Immunohistochemistry
Positive Markers:
CK7 positive
CEA positive
EMA positive (apical pattern characteristic)
p16 variable
PAX8 positive.
Negative Markers:
CK20 typically negative
TTF-1 negative
CDX2 negative
Mammaglobin negative.
Diagnostic Utility:
EMA shows characteristic apical/luminal staining pattern
CK7/CK20 profile supports gynecologic primary
PAX8 confirms müllerian origin.
Molecular Subtypes:
Limited molecular data available
HPV association variable and may differ from conventional cervical adenocarcinomas.
Molecular/Genetic
Genetic Mutations:
Limited data on molecular alterations
May have different molecular profile from conventional cervical adenocarcinomas
Complex genomic instability likely.
Molecular Markers:
High Ki-67 proliferation index (>50%)
p53 mutations possible
Variable p16 expression suggesting variable HPV association.
Prognostic Significance:
Extremely poor prognosis with high propensity for lymphovascular invasion and metastasis
Early distant spread common.
Therapeutic Targets:
Aggressive multimodal therapy
Standard cervical cancer protocols often inadequate
Systemic chemotherapy often required.
Differential Diagnosis
Similar Entities:
Metastatic micropapillary carcinoma (breast, lung, ovary)
Serous carcinoma
Conventional cervical adenocarcinoma with papillary features.
Distinguishing Features:
Primary cervical: clinical presentation, CK7+/CK20-
Metastatic breast: mammaglobin+, GATA3+
Serous: WT1+, p53 mutant pattern.
Diagnostic Challenges:
Distinction from metastatic micropapillary carcinoma crucial
Recognition of micropapillary pattern
Exclusion of other primary sites.
Rare Variants:
Mixed micropapillary and conventional adenocarcinoma
Micropapillary features in adenosquamous carcinoma.
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]