Definition/General

Introduction:
-Malignant peritoneal effusion contains neoplastic cells in ascitic fluid
-Results from peritoneal carcinomatosis or primary peritoneal malignancy
-Represents advanced stage disease in most cases
-Poor prognostic indicator
-Requires immediate oncological management.
Origin:
-Results from hematogenous spread to peritoneum
-Direct extension from intra-abdominal organs
-Transcoelomic spread via peritoneal cavity
-Primary peritoneal carcinoma
-Lymphatic dissemination
-Implantation during surgery.
Classification:
-Classified as exudative ascites
-SAAG <1.1 g/dL
-High protein content >2.5 g/dL
-Elevated tumor markers
-Based on primary site: ovarian, gastric, pancreatic, colorectal
-Primary vs secondary peritoneal malignancy.
Epidemiology:
-Accounts for 10-15% of all ascites
-Most common in ovarian (60%) and GI malignancies (30%)
-Female predominance (ovarian primary)
-Peak incidence 50-70 years
-Poor prognosis with median survival 6-12 months.

Clinical Features

Presentation:
-Progressive abdominal distention
-Early satiety and bloating
-Abdominal pain or discomfort
-Nausea and vomiting
-Dyspnea due to diaphragmatic elevation
-Weight loss despite fluid accumulation.
Symptoms:
-Abdominal distention (90% cases)
-Early satiety (80% cases)
-Abdominal pain (70% cases)
-Nausea and vomiting (60% cases)
-Dyspnea (50% cases)
-Leg swelling (40% cases)
-Fatigue and weakness (85% cases).
Risk Factors:
-Previous malignancy history
-Family history of ovarian/breast cancer
-BRCA1/2 mutations
-Advanced age
-Nulliparity (ovarian cancer)
-Chronic inflammatory conditions.
Screening:
-Tumor markers: CA-125, CEA, CA 19-9
-CT abdomen and pelvis
-Ultrasound abdomen
-Cytological examination of ascitic fluid
-Image-guided biopsy if needed.

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Gross Description

Appearance:
-Malignant ascites is often hemorrhagic or serosanguineous
-Turbid to cloudy appearance
-May be yellow to brown
-Viscous consistency due to high protein
-Large volume typically present.
Characteristics:
-High protein content >2.5 g/dL
-Elevated LDH >200 U/L
-SAAG <1.1 g/dL
-Low glucose may be present
-Elevated tumor markers
-pH may be acidic.
Size Location:
-Massive ascites often present
-Loculated due to adhesions and tumor deposits
-Omental involvement common
-Peritoneal studding
-Pelvic masses may be palpable.
Multifocality:
-Widespread peritoneal involvement
-Omental caking
-Multiple peritoneal implants
-Retroperitoneal lymphadenopathy
-Liver surface involvement.

Microscopic Description

Histological Features:
-Malignant cells in clusters, papillary fragments, and single cells
-High cellularity >1000 cells/μL
-Pleomorphic malignant cells
-High nuclear-cytoplasmic ratio
-Mitotic activity
-Necrotic background.
Cellular Characteristics:
-Large pleomorphic cells
-Hyperchromatic nuclei with irregular contours
-Prominent nucleoli
-Increased nuclear-cytoplasmic ratio
-Cytoplasmic vacuoles (adenocarcinoma)
-Signet ring cells (gastric primary).
Architectural Patterns:
-Three-dimensional cell clusters
-Papillary architecture (ovarian/peritoneal)
-Acinar formations (adenocarcinoma)
-Single cell pattern (lobular breast cancer)
-Sheet-like arrangement
-Psammoma bodies (serous carcinoma).
Grading Criteria:
-Nuclear grading: Grade 1 (mild atypia)
-Grade 2 (moderate atypia)
-Grade 3 (severe atypia)
-Mitotic count
-Architectural complexity
-Necrosis presence.

Immunohistochemistry

Positive Markers:
-Primary site-specific markers
-Ovarian: WT1, PAX8, CA-125
-Breast: GATA3, mammaglobin, GCDFP-15
-GI tract: CDX2, CK20
-Pancreas: CA 19-9, DPC4 loss
-Lung: TTF-1, napsin A.
Negative Markers:
-Calretinin, WT1 (to exclude reactive mesothelial)
-Site-specific negatives
-CK5/6 usually negative in adenocarcinoma
-p63 negative in most adenocarcinomas.
Diagnostic Utility:
-Essential for primary site identification
-Adenocarcinoma vs mesothelioma differentiation
-Panel approach recommended
-CK7/CK20 pattern helpful
-ER/PR for breast/gynecologic primaries.
Molecular Subtypes:
-Based on primary site and histology
-High-grade serous (ovarian)
-Mucinous adenocarcinoma
-Signet ring cell carcinoma
-Clear cell carcinoma
-Neuroendocrine carcinoma.

Molecular/Genetic

Genetic Mutations:
-TP53 mutations (high-grade serous)
-KRAS mutations (pancreatic, colorectal)
-BRCA1/2 mutations (ovarian, breast)
-PIK3CA mutations
-ARID1A mutations (clear cell, endometrioid).
Molecular Markers:
-Tumor markers elevated: CA-125, CEA, CA 19-9
-AFP (hepatocellular, germ cell)
-Beta-hCG (choriocarcinoma)
-Inhibin (granulosa cell tumor)
-Chromogranin, synaptophysin (neuroendocrine).
Prognostic Significance:
-Stage IV disease indicator
-Poor prognosis overall
-Primary site affects survival
-Response to chemotherapy variable
-Platinum sensitivity (ovarian)
-Targeted therapy options emerging.
Therapeutic Targets:
-Chemotherapy based on primary site
-Targeted therapy: HER2, EGFR, VEGF
-PARP inhibitors (BRCA-mutated)
-Immunotherapy (MSI-high tumors)
-Palliative paracentesis.

Differential Diagnosis

Similar Entities:
-Reactive mesothelial proliferation
-Malignant mesothelioma
-Lymphoma (primary effusion)
-Metastatic carcinoma from different primaries
-Primary peritoneal carcinoma.
Distinguishing Features:
-Malignancy: Cytological atypia
-Malignancy: Architectural complexity
-Reactive: Bland nuclear features
-Mesothelioma: Calretinin positive
-Lymphoma: Lymphoid markers positive
-Primary site: Specific IHC patterns.
Diagnostic Challenges:
-Distinguishing from reactive mesothelial proliferation
-Primary site identification
-Low-grade malignancies may be subtle
-Processing artifacts
-Sampling adequacy.
Rare Variants:
-Primary peritoneal carcinoma
-Mesothelioma
-Lymphomatosis
-Germ cell tumors
-Sarcomatous malignancies
-Neuroendocrine 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

Peritoneal fluid, volume [X] mL, [appearance - hemorrhagic/turbid/cloudy]

Adequacy

Adequate for cytological evaluation

Cellularity

High cellularity with [X] cells/μL

Malignant Cells

POSITIVE for malignant cells

Morphological Features

Malignant cells present in [clusters/single cells/papillary fragments]. Cells show [nuclear features] with [cytoplasmic features]. [Architectural pattern] noted.

Cell Type

Morphology consistent with [adenocarcinoma/other carcinoma type]

Immunohistochemistry

IHC panel: [List positive and negative markers]

Primary Site Assessment

IHC pattern suggests [primary site/unknown primary]

Final Diagnosis

MALIGNANT peritoneal effusion - [Carcinoma type] consistent with [primary site/unknown primary]

Comment

Findings indicate peritoneal carcinomatosis. Clinical correlation with imaging and tumor markers recommended. Oncology consultation advised.