Definition/General
Introduction:
Cervical carcinosarcoma (malignant mixed Müllerian tumor, MMMT) is an extremely rare and highly aggressive biphasic malignant tumor containing both carcinomatous (epithelial) and sarcomatous (mesenchymal) components
It has very poor prognosis.
Origin:
Arises from pluripotent stem cells or through epithelial-mesenchymal transition
Contains both malignant epithelial and mesenchymal elements
May develop de novo or from pre-existing carcinoma.
Classification:
WHO Classification categorizes as malignant mixed Müllerian tumor
Subdivided into homologous (mesenchymal elements native to uterus) and heterologous (foreign mesenchymal elements).
Epidemiology:
Extremely rare, <1% of cervical malignancies
Peak incidence 60-80 years (older than typical cervical cancers)
Associated with radiation therapy history
Very poor prognosis.
Clinical Features
Presentation:
Postmenopausal bleeding
Large, polypoid cervical mass
Rapid growth
Advanced stage at presentation
May have history of prior radiation therapy.
Symptoms:
Abnormal vaginal bleeding (heavy)
Pelvic pain or pressure
Visible polypoid mass
Constitutional symptoms (weight loss, fatigue)
Urinary or bowel obstruction.
Risk Factors:
Advanced age (>60 years)
Prior pelvic radiation therapy
History of cervical dysplasia
Nulliparity
No clear HPV association.
Screening:
Standard screening less effective due to age and rapid growth
Advanced imaging shows large heterogeneous mass
Tissue diagnosis required.
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Gross Description
Appearance:
Large, polypoid, or lobulated mass with heterogeneous appearance
Areas of firm and soft consistency
Extensive hemorrhage and necrosis common.
Characteristics:
Size typically large (>5 cm)
Variable consistency (firm and soft areas)
Gray-white to tan coloration
Polypoid or sessile
Extensive necrosis typical.
Size Location:
Usually involves entire cervix
Often protrudes through external os
May extend to parametrium, vagina, or corpus.
Multifocality:
Usually single large mass
May have satellite nodules
Extensive local invasion typical
Metastatic deposits possible.
Microscopic Description
Histological Features:
Biphasic tumor with intimate admixture of malignant epithelial (carcinomatous) and mesenchymal (sarcomatous) elements
Both components show high-grade malignant features.
Cellular Characteristics:
Carcinomatous: squamous or glandular carcinoma cells
Sarcomatous: spindle cells, may include heterologous elements (cartilage, bone, striated muscle).
Architectural Patterns:
Intimate admixture of epithelial and mesenchymal components
May show transition between components
Extensive necrosis and hemorrhage.
Grading Criteria:
High-grade malignancy by definition
Both components show high-grade features
Classification based on mesenchymal elements (homologous vs
heterologous).
Immunohistochemistry
Positive Markers:
Epithelial component: cytokeratin, EMA
Mesenchymal component: vimentin, specific markers based on differentiation (desmin, S100, etc.).
Negative Markers:
Variable based on specific differentiation
Cross-reactivity between components possible.
Diagnostic Utility:
Cytokeratin and EMA confirm epithelial component
Vimentin and specific markers confirm mesenchymal differentiation
Essential for diagnosis.
Molecular Subtypes:
Homologous (endometrial stroma, smooth muscle) vs
heterologous (cartilage, bone, striated muscle, fat).
Molecular/Genetic
Genetic Mutations:
TP53 mutations common (>80%)
Complex karyotype with chromosomal instability
PIK3CA alterations
PTEN loss possible.
Molecular Markers:
Aberrant p53 expression
High Ki-67 in both components
Complex genomic alterations
Loss of tumor suppressor function.
Prognostic Significance:
Extremely poor prognosis
Median survival <2 years
Stage most important prognostic factor
Heterologous elements may worsen prognosis.
Therapeutic Targets:
Combination chemotherapy (carcinoma and sarcoma regimens)
Surgery when feasible
Radiation therapy
Limited targeted options.
Differential Diagnosis
Similar Entities:
Carcinoma with sarcomatoid features
Sarcoma with epithelial differentiation
Collision tumor
Adenosarcoma with malignant transformation.
Distinguishing Features:
Carcinosarcoma: intimate admixture, both components malignant
Sarcomatoid carcinoma: predominant epithelial
Adenosarcoma: benign epithelial.
Diagnostic Challenges:
Recognition of both components
Distinction from sarcomatoid carcinoma
Assessment of heterologous elements
Sampling adequacy.
Rare Variants:
Carcinosarcoma with neuroendocrine differentiation
Teratoid carcinosarcoma
Pure sarcomatous areas.
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]