Definition/General

Introduction:
-Atypical lobular hyperplasia (ALH) is a proliferative breast lesion characterized by the proliferation of small, discohesive cells within the breast lobules, but not meeting the criteria for lobular carcinoma in situ (LCIS)
-It is a risk factor for developing invasive breast cancer.
Origin:
-ALH arises from the terminal duct-lobular unit (TDLU)
-It is part of the spectrum of lobular neoplasia and is characterized by the loss of E-cadherin.
Classification:
-ALH is a benign proliferative breast lesion with atypia
-The distinction from LCIS is quantitative: ALH is diagnosed when the characteristic cells fill less than 50% of the acini within a lobule.
Epidemiology:
-ALH is often an incidental finding on breast biopsies
-It is most common in premenopausal women.

Clinical Features

Presentation: ALH is typically asymptomatic and is usually found incidentally on a biopsy performed for other reasons.
Symptoms: Asymptomatic.
Risk Factors: The risk factors are similar to those for breast cancer in general.
Screening:
-ALH does not typically produce mammographic findings
-It is an incidental microscopic finding.

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Gross Description

Appearance: There are no specific gross findings for ALH.
Characteristics: Gross findings are not specific for this microscopic diagnosis.
Size Location: Gross findings are not specific for this microscopic diagnosis.
Multifocality: ALH can be multifocal and bilateral.

Microscopic Description

Histological Features:
-ALH is characterized by a proliferation of small, uniform, discohesive cells within the acini of a lobule
-The cells are identical to those of LCIS, but the proliferation involves less than 50% of the acini.
Cellular Characteristics:
-The cells are small and monotonous with scant cytoplasm and round, uniform nuclei
-Intracytoplasmic mucin vacuoles can be seen.
Architectural Patterns:
-The acini are partially filled with the discohesive cells
-The underlying lobular architecture is preserved.
Grading Criteria: ALH is a low-grade lesion.

Immunohistochemistry

Positive Markers:
-The cells are positive for ER and PR
-They are positive for cytokeratins.
Negative Markers:
-The hallmark is the loss of E-cadherin expression
-HER2 is negative.
Diagnostic Utility: IHC for E-cadherin is essential for diagnosis and to differentiate ALH from atypical ductal hyperplasia (ADH).
Molecular Subtypes: Molecular subtyping is not relevant for this pre-invasive lesion.

Molecular/Genetic

Genetic Mutations: Loss of the CDH1 gene is the key genetic event.
Molecular Markers: Loss of E-cadherin is the key molecular marker.
Prognostic Significance:
-ALH is a risk factor for developing invasive breast cancer, with a 4-5 fold increased risk
-The risk applies to both breasts.
Therapeutic Targets:
-Management typically involves clinical follow-up
-Risk-reducing medication (e.g., tamoxifen) may be considered.

Differential Diagnosis

Similar Entities:
-Lobular carcinoma in situ (LCIS)
-Atypical ductal hyperplasia (ADH).
Distinguishing Features:
-The distinction between ALH and LCIS is quantitative, based on the extent of acinar involvement
-ADH is E-cadherin positive and has different cytological and architectural features.
Diagnostic Challenges: The main challenge is the quantitative distinction from LCIS, which can be subjective.
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]