Definition/General

Introduction:
-Adrenocortical carcinoma (ACC) is a rare, aggressive malignancy arising from the adrenal cortex
-It represents <1% of all malignancies
-It has bimodal age distribution
-FNAC shows malignant cortical cells with pleomorphic features.
Origin:
-Arises from adrenocortical cells in any cortical zone
-Shows loss of normal architecture
-Demonstrates invasive growth pattern
-May retain steroidogenic function
-Often shows dedifferentiation with loss of cortical markers.
Classification:
-Classified according to WHO 2017 classification
-Functional ACC (hormone-producing)
-Non-functional ACC (no hormone excess)
-Weiss criteria used for histological diagnosis
-Ki-67 >10% indicates high proliferative activity.
Epidemiology:
-Bimodal distribution: Children <5 years and adults 40-50 years
-Female predominance (1.5:1)
-Li-Fraumeni syndrome association
-Beckwith-Wiedemann syndrome in children
-Poor prognosis overall (5-year survival 20-35%).

Clinical Features

Presentation:
-Hormonal excess symptoms (60% functional)
-Abdominal mass (large tumors)
-Abdominal pain
-Weight loss
-Rapid onset symptoms
-Metastatic disease at presentation (30-40%)
-Constitutional symptoms common.

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Gross Description

Appearance:
-Large, irregular mass (typically >6 cm)
-Variegated cut surface with areas of necrosis and hemorrhage
-Golden-yellow areas (residual cortical differentiation)
-Gray-white areas (dedifferentiated regions)
-Soft to firm consistency.
Characteristics:
-Invasion of capsule and adjacent structures
-Central necrosis common
-Hemorrhagic areas frequent
-Calcifications possible
-Cystic degeneration
-Loss of normal architecture.

Microscopic Description

Immunohistochemistry

Positive Markers:
-Inhibin alpha (may be focal or lost)
-SF-1 (steroidogenic factor, often retained)
-Melan-A (variable, may be lost)
-Synaptophysin (variable)
-Calretinin (variable)
-D2-40 (lymphatic invasion).
Negative Markers:
-Chromogranin (usually negative)
-Cytokeratins (negative)
-EMA (negative)
-PAX8 (negative)
-TTF-1 (negative)
-S-100 (negative)
-Hepatocyte marker (negative).
Diagnostic Utility:
-SF-1 and Inhibin confirm adrenocortical origin (may be lost in high-grade)
-High Ki-67 supports malignancy
-p53 and beta-catenin alterations indicate aggressive behavior
-Loss of cortical markers with dedifferentiation.

Molecular/Genetic

Differential Diagnosis

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.

Clinical Correlation

Patient with [large adrenal mass] measuring [size] cm. [Functional/Non-functional] status. [Local invasion/Metastases] noted on imaging

Specimen Information

FNAC from [adrenal mass], [side], performed under [guidance method]

Specimen Adequacy

[Adequate/Inadequate] for cytological interpretation

Cytological Findings

Cellular smears showing [pleomorphic cortical cells] with [marked nuclear atypia]. [High mitotic activity] and [necrotic background] noted

Malignant Features

[Nuclear pleomorphism/Prominent nucleoli/High mitotic rate/Atypical mitoses/Necrosis] observed

Background

Background shows [necrosis/hemorrhage/inflammatory infiltrate]

Cytological Diagnosis

[Malignant] - Features consistent with adrenocortical carcinoma

Prognostic Factors

[Large size/High grade nuclear features/Necrosis/High proliferative activity] indicate aggressive behavior

Staging Implications

Findings consistent with [locally advanced/metastatic] disease based on clinical and imaging correlation

Recommendations

URGENT multidisciplinary team discussion. [Staging studies/Surgical evaluation/Medical oncology consultation] recommended

Note

Adrenocortical carcinoma is aggressive malignancy requiring immediate multidisciplinary management