Definition/General

Introduction:
-Primary squamous cell carcinoma (SCC) of the breast is an extremely rare and aggressive malignancy
-It is a type of metaplastic carcinoma where the entire tumor is composed of malignant squamous cells
-The diagnosis requires the exclusion of metastasis from another primary site.
Origin:
-The origin is debated, but it is thought to arise from the metaplastic transformation of breast ductal epithelium or from the epidermis of the nipple.
Classification:
-It is classified as a subtype of metaplastic breast carcinoma
-It can be keratinizing or non-keratinizing.
Epidemiology:
-Extremely rare, accounting for less than 0.1% of all invasive breast cancers
-It typically affects older women.

Clinical Features

Presentation:
-Presents as a large, rapidly growing, palpable breast mass
-The mass is often cystic and can be associated with skin ulceration or abscess formation.
Symptoms:
-A painless, large breast lump is the most common symptom
-Skin changes are common.
Risk Factors:
-No specific risk factors are well-established
-It can be associated with chronic inflammation or abscesses.
Screening:
-Usually presents as a palpable mass
-Mammographic findings are non-specific, often showing a complex cystic mass.

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Gross Description

Appearance:
-A large, often cystic mass with a thick, irregular wall
-The cyst may contain necrotic material
-Solid areas are firm and gray-white.
Characteristics:
-The tumor is often large at diagnosis
-Necrosis and cystic degeneration are common features.
Size Location: Can occur anywhere in the breast.
Multifocality: Rare.

Microscopic Description

Histological Features:
-The tumor is composed of sheets and nests of malignant squamous cells with intercellular bridges and keratinization
-A glandular component is absent.
Cellular Characteristics:
-The tumor cells are large and polygonal with abundant eosinophilic cytoplasm and large, hyperchromatic, irregular nuclei
-Mitotic activity is high.
Architectural Patterns:
-The growth pattern is infiltrative
-Keratin pearls are a characteristic feature of well-differentiated tumors.
Grading Criteria: These are considered high-grade tumors.

Immunohistochemistry

Positive Markers:
-The tumor cells are positive for squamous markers such as p63, CK5/6, and CK14
-They are also positive for broad-spectrum cytokeratins.
Negative Markers:
-Typically triple-negative (ER, PR, and HER2 negative)
-Negative for glandular markers like CK7 in most cases.
Diagnostic Utility: IHC is essential to confirm squamous differentiation and to exclude other tumor types.
Molecular Subtypes: Most are classified as triple-negative/basal-like breast cancer.

Molecular/Genetic

Genetic Mutations: The molecular genetics are not well characterized but often involve TP53 mutations.
Molecular Markers: No specific molecular markers are routinely used for diagnosis.
Prognostic Significance:
-The prognosis is generally poor, worse than that of conventional adenocarcinoma NST
-It has a high rate of local and distant recurrence.
Therapeutic Targets:
-Treatment is primarily surgical
-As a triple-negative cancer, it does not respond to hormonal or HER2-targeted therapy
-Platinum-based chemotherapy is often used.

Differential Diagnosis

Similar Entities:
-Metastatic SCC from another site (e.g., lung, cervix)
-Metaplastic carcinoma with squamous differentiation
-Adenosquamous carcinoma.
Distinguishing Features:
-Excluding a metastasis is crucial and requires a thorough clinical workup
-Metaplastic carcinoma with squamous differentiation and adenosquamous carcinoma will have a glandular component, which is absent in pure SCC.
Diagnostic Challenges:
-The main challenge is to confirm a primary breast origin
-The presence of an in situ squamous component would be definitive but is rarely seen.
Rare Variants: The entire entity is a rare variant.

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]