Definition/General

Introduction:
-Cervical inflammatory carcinoma is a rare and aggressive clinical variant of cervical carcinoma characterized by rapid onset, diffuse cervical enlargement, and extensive lymphovascular invasion
-It mimics inflammatory conditions clinically.
Origin: May arise from any histological type of cervical carcinoma (squamous cell carcinoma or adenocarcinoma) but shows characteristic pattern of extensive lymphovascular invasion.
Classification: Classified based on underlying histological type (squamous or glandular) but designated as inflammatory variant due to clinical presentation and extensive vascular involvement.
Epidemiology:
-Extremely rare representing <1% of cervical carcinomas
-Peak incidence 35-55 years
-Associated with HPV infection
-Highly aggressive with poor prognosis.

Clinical Features

Presentation:
-Rapid onset cervical enlargement
-Diffuse cervical induration
-Erythema and edema of cervix
-May mimic acute cervicitis or pelvic inflammatory disease.
Symptoms:
-Profuse vaginal bleeding
-Foul-smelling discharge
-Pelvic pain
-Fever (may be present)
-Rapid clinical deterioration
-Urinary retention.
Risk Factors:
-HPV infection (high-risk types)
-Previous cervical dysplasia
-Immunosuppression
-Smoking
-Multiple sexual partners
-Poor access to screening.
Screening:
-Often advanced at presentation
-Pap smear may show malignant cells
-Clinical examination reveals indurated, enlarged cervix
-Biopsy mandatory for diagnosis.

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Gross Description

Appearance:
-Diffusely enlarged, indurated cervix
-Surface may be ulcerated or necrotic
-Edematous and hemorrhagic appearance
-Loss of normal cervical architecture.
Characteristics:
-Cervix typically >5 cm
-Hard, woody consistency
-Red to purple discoloration
-Extensive necrosis may be present
-Friable tissue.
Size Location:
-Involves entire cervix diffusely
-Extension to parametrium common
-Vaginal involvement frequent
-May involve corpus uteri.
Multifocality:
-Diffuse involvement by definition
-Multiple areas of vascular invasion
-Early metastatic spread common
-Widespread disease typical.

Microscopic Description

Histological Features:
-Extensive lymphovascular invasion is the defining feature
-Underlying carcinoma may be squamous or adenocarcinoma
-Dermal lymphatic-like pattern of invasion.
Cellular Characteristics:
-Cellular features depend on underlying carcinoma type
-High-grade nuclear features typical
-Abundant mitotic activity
-Necrosis common.
Architectural Patterns:
-Characteristic extensive lymphovascular invasion
-Tumor emboli in numerous vessels
-Underlying invasive carcinoma
-Stromal edema and inflammation.
Grading Criteria:
-Usually high-grade by definition due to aggressive behavior
-Grading based on underlying carcinoma type
-Extensive vascular invasion noted.

Immunohistochemistry

Positive Markers:
-Markers depend on underlying carcinoma
-Squamous: p63+, CK5/6+
-Adenocarcinoma: CK7+, CEA+
-Vascular markers highlight invasion (CD31, CD34).
Negative Markers:
-Markers negative based on underlying type
-Lymphoid markers negative
-Melanoma markers negative.
Diagnostic Utility:
-Vascular markers (CD31, CD34) highlight extensive lymphovascular invasion
-p16 may be positive if HPV-associated
-Confirms carcinomatous nature.
Molecular Subtypes:
-HPV-associated in most cases
-Molecular features similar to underlying carcinoma type
-Complex genetic alterations likely present.

Molecular/Genetic

Genetic Mutations:
-HPV integration common
-p53 mutations frequent
-PIK3CA alterations
-KRAS mutations
-Extensive chromosomal instability typical.
Molecular Markers:
-HPV DNA detectable
-p16 overexpression
-Loss of p53 function
-Very high Ki-67 proliferation index (>70%)
-Angiogenic markers elevated.
Prognostic Significance:
-Extremely poor prognosis
-Median survival <12 months
-Extensive vascular invasion predicts rapid dissemination
-Stage at presentation crucial.
Therapeutic Targets:
-Limited effective therapies
-Anti-angiogenic agents theoretical target
-Immune checkpoint inhibitors under investigation
-Aggressive multimodal therapy required.

Differential Diagnosis

Similar Entities:
-Acute cervicitis
-Pelvic inflammatory disease
-Cervical abscess
-Conventional cervical carcinoma with infection
-Lymphoma
-Metastatic carcinoma.
Distinguishing Features:
-Inflammatory carcinoma: Malignant cells in vessels, rapid progression
-Cervicitis: Inflammatory cells only, responds to antibiotics
-Lymphoma: Lymphoid markers positive.
Diagnostic Challenges:
-Clinical mimicry of infectious process
-May be misdiagnosed as PID initially
-Requires high index of suspicion
-Extensive sampling needed.
Rare Variants:
-Squamous cell type most common
-Adenocarcinoma type less common
-Mixed histologies possible
-Secondary inflammatory changes.

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] showing diffuse enlargement, measuring [size] cm

Diagnosis

Cervical inflammatory carcinoma, [underlying type]

Classification

Classification: [Squamous cell carcinoma/Adenocarcinoma], inflammatory variant

Histological Features

Shows [underlying carcinoma] with extensive lymphovascular invasion

Size and Extent

Size: [X] cm, extent: diffuse cervical involvement

Margins

Margins: [typically involved due to extensive disease]

Lymphovascular Invasion

Lymphovascular invasion: Extensive (defining feature)

Lymph Node Status

Lymph nodes: [X] positive out of [X] examined

Special Studies

IHC: [markers confirming carcinoma type]

Vascular markers: Highlight extensive invasion

[other study]: [result]

Prognostic Factors

Prognostic factors: Poor prognosis due to extensive vascular invasion

Final Diagnosis

Final diagnosis: Cervical inflammatory carcinoma, [type], extensive lymphovascular invasion