Definition/General
Introduction:
Cervical lymphoma refers to primary lymphomatous involvement of the cervix, most commonly diffuse large B-cell lymphoma
It represents an extremely rare form of extranodal lymphoma affecting the female genital tract.
Origin:
Arises from lymphoid cells within cervical tissue, either from pre-existing mucosa-associated lymphoid tissue (MALT) or de novo lymphocyte transformation.
Classification:
WHO Classification includes various subtypes: diffuse large B-cell lymphoma (most common), MALT lymphoma, Burkitt lymphoma, and T-cell lymphomas.
Epidemiology:
Extremely rare, <0.1% of cervical malignancies
Peak incidence 40-60 years
May be associated with immunosuppression, HIV infection, or autoimmune conditions.
Clinical Features
Presentation:
Abnormal vaginal bleeding
Cervical enlargement or mass
Pelvic pain
B-symptoms (fever, night sweats, weight loss) in advanced cases.
Symptoms:
Abnormal vaginal bleeding (most common)
Vaginal discharge
Pelvic pain or pressure
Cervical mass on examination
Systemic B-symptoms possible.
Risk Factors:
Immunosuppression (HIV, organ transplant)
Autoimmune disorders
Epstein-Barr virus infection
Previous radiation therapy
Advanced age.
Screening:
No specific screening
Imaging may show soft tissue mass
Staging with CT/PET-CT
Bone marrow biopsy for staging.
Master Cervical Lymphoma Pathology with RxDx
Access 100+ pathology videos and expert guidance with the RxDx app
Gross Description
Appearance:
Enlarged, firm, gray-white cervical mass
Homogeneous cut surface without necrosis typically
May be polypoid or infiltrative.
Characteristics:
Size variable (2-10 cm)
Firm consistency
Gray-white to tan color
Homogeneous appearance
Usually non-necrotic.
Size Location:
Can involve entire cervix or be localized
May extend to parametrium, vagina, or corpus
Often presents as bulky disease.
Multifocality:
May be multifocal within cervix
Part of systemic disease in advanced cases
Local extension common.
Microscopic Description
Histological Features:
Diffuse infiltrate of large atypical lymphoid cells effacing normal cervical architecture
Cells show vesicular nuclei, prominent nucleoli, and moderate cytoplasm.
Cellular Characteristics:
Large lymphoid cells (>2x normal lymphocyte)
Vesicular nuclei with prominent nucleoli
Moderate to abundant cytoplasm
High mitotic activity.
Architectural Patterns:
Diffuse growth pattern effacing normal tissue
May show cohesive sheets or single-cell infiltration
Starry-sky pattern possible.
Grading Criteria:
High-grade malignancy
Classification based on WHO criteria and immunophenotype rather than morphologic grading.
Immunohistochemistry
Positive Markers:
CD20 positive (B-cell lymphomas)
CD79a positive
PAX5 positive
CD3 positive (T-cell lymphomas)
Ki-67 high (>40%).
Negative Markers:
Cytokeratin negative
EMA negative
S100 negative
Melanoma markers negative.
Diagnostic Utility:
CD20, CD79a, PAX5 confirm B-cell origin
CD3, CD5 for T-cell lymphomas
Ki-67 indicates proliferation rate
Essential for diagnosis.
Molecular Subtypes:
B-cell lymphomas (90%): DLBCL, MALT, Burkitt
T-cell lymphomas (10%): PTCL, ALCL
Each has specific immunoprofile.
Molecular/Genetic
Genetic Mutations:
Variable depending on subtype
DLBCL: MYC, BCL2, BCL6 rearrangements
MALT: t(11;18), t(1;14)
Burkitt: MYC translocations.
Molecular Markers:
High Ki-67 proliferation index
Clonal immunoglobulin gene rearrangements
Specific translocations by subtype.
Prognostic Significance:
Prognosis depends on subtype, stage, and patient factors
DLBCL has variable prognosis based on molecular subtypes.
Therapeutic Targets:
Chemotherapy (R-CHOP for DLBCL)
Radiation therapy for localized disease
Rituximab for B-cell lymphomas
Targeted therapies evolving.
Differential Diagnosis
Similar Entities:
Poorly differentiated carcinoma
Melanoma
Sarcoma
Plasmacytoma
Chronic inflammation with reactive lymphoid hyperplasia.
Distinguishing Features:
Lymphoma: CD45+, lymphoid markers+
Carcinoma: cytokeratin+
Melanoma: S100+, HMB-45+
Reactive: polyclonal, preserved architecture.
Diagnostic Challenges:
Distinction from other small round cell tumors
Recognition of lymphoid nature
Subtype classification requires comprehensive immunophenotyping.
Rare Variants:
MALT lymphoma
Burkitt lymphoma
T-cell lymphomas
Primary CNS-type lymphoma
Plasmablastic lymphoma.
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]