Definition/General
                                                                                Introduction: 
                                                                                An atypical intraductal papilloma is a benign papillary lesion of the breast that contains a focus of atypical ductal hyperplasia (ADH) or low-grade ductal carcinoma in situ (DCIS).                                    
                                
                                                                                Origin: 
                                                                                It arises from the ductal epithelium.                                    
                                
                                                                                Classification: 
                                                                                                                    
                                It is classified as a benign proliferative breast lesion with atypia
The presence of atypia within a papilloma increases the risk of subsequent breast cancer.
                                                                                Epidemiology: 
                                                                                                                    
                                They are uncommon lesions, found in a small percentage of breast biopsies.
Clinical Features
                                                                                        Presentation: 
                                                                                        Similar to benign papillomas, they can present with nipple discharge or as a palpable mass.                                        
                                    
                                                                                        Symptoms: 
                                                                                        Nipple discharge is the most common symptom.                                        
                                    
                                                                                        Risk Factors: 
                                                                                        There are no well-established risk factors.                                        
                                    
                                                                                        Screening: 
                                                                                        They can be associated with mammographic abnormalities, such as a mass or calcifications.                                        
                                    Master Atypical Papilloma Pathology with RxDx
Access 100+ pathology videos and expert guidance with the RxDx app
Gross Description
                                                                                        Appearance: 
                                                                                                                                
                                    A friable, polypoid mass within a dilated duct.
                                                                                        Characteristics: 
                                                                                        The gross appearance is indistinguishable from a benign papilloma.                                        
                                    
                                                                                        Size Location: 
                                                                                        Usually small and located in the large subareolar ducts.                                        
                                    
                                                                                        Multifocality: 
                                                                                        Can be multifocal.                                        
                                    Microscopic Description
                                                                                        Histological Features: 
                                                                                                                                
                                    The lesion is a papilloma with a focus of epithelial proliferation that meets the criteria for ADH or low-grade DCIS
The atypical proliferation is typically focal and involves the epithelial component of the papilloma.
                                                                                        Cellular Characteristics: 
                                                                                                                                
                                    The atypical cells are monotonous, with low-grade nuclear atypia, similar to that seen in ADH or low-grade DCIS.
                                                                                        Architectural Patterns: 
                                                                                                                                
                                    The atypical proliferation can have cribriform, micropapillary, or solid patterns.
                                                                                        Grading Criteria: 
                                                                                                                                
                                    This is a lesion with atypia
The key is to distinguish it from a papilloma completely involved by DCIS.
Immunohistochemistry
                                                                                        Positive Markers: 
                                                                                                                                
                                    The myoepithelial layer is preserved, which can be highlighted by myoepithelial markers
The atypical epithelial cells are ER-positive.
                                                                                        Negative Markers: 
                                                                                                                                
                                    The atypical cells are negative for high molecular weight cytokeratins (e.g., CK5/6).
                                                                                        Diagnostic Utility: 
                                                                                                                                
                                    IHC for myoepithelial markers is essential to distinguish from invasive papillary carcinoma
CK5/6 can help differentiate the atypical proliferation from usual ductal hyperplasia within a papilloma.
                                                                                        Molecular Subtypes: 
                                                                                        Molecular subtyping is not relevant for this pre-invasive lesion.                                        
                                    Molecular/Genetic
                                                                                        Genetic Mutations: 
                                                                                                                                
                                    The atypical component can show genetic alterations similar to low-grade DCIS, such as loss of heterozygosity at 16q.
                                                                                        Molecular Markers: 
                                                                                        No specific molecular markers are routinely used for diagnosis.                                        
                                    
                                                                                        Prognostic Significance: 
                                                                                                                                
                                    Atypical papillomas are associated with a significantly increased risk of subsequent breast cancer, higher than that of a benign papilloma or ADH alone.
                                                                                        Therapeutic Targets: 
                                                                                        Surgical excision is the standard treatment to ensure the entire lesion is removed and to rule out an associated invasive carcinoma.                                        
                                    Differential Diagnosis
                                                                                Similar Entities: 
                                                                                                                    
                                Benign papilloma with UDH
Papillary DCIS
Invasive papillary carcinoma.
                                                                                Distinguishing Features: 
                                                                                                                    
                                UDH within a papilloma has a heterogeneous cell population and mosaic CK5/6 staining
Papillary DCIS involves the entire papilloma and lacks a myoepithelial layer in the papillae
Invasive papillary carcinoma shows stromal invasion.
                                                                                Diagnostic Challenges: 
                                                                                                                    
                                The main challenge is distinguishing an atypical papilloma from papillary DCIS on a core needle biopsy
The distinction can be difficult and often requires excision for definitive diagnosis.
                                                                                Rare Variants: 
                                                                                There are no specific rare variants.                                    
                                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]
Final Diagnosis
Final diagnosis: [complete diagnosis]