Myofibroblastoma (MFB) of the breast is an uncommon but well described benign stromal neoplasm of the breast. MFB can have many different morphologic patterns (see variants below), but important defining characteristics include:
Mesenchymal tumor without epimyoepithelial elements
Clinically present with breast erythema and thickening (peau d’orange)
Dermal lymphatics filled with tumor
3-year survival rate 3-10%
Neuroendocrine Tumors
Well-differentiated Neuroendocrine Tumor
Small Cell Carcinoma
References
Kumar, Vinay, Abul K. Abbas, and Jon C. Aster. Robbins and Cotran Pathologic Basis of Disease. Ninth edition. Philadelphia, PA: Elsevier/Saunders, 2015.
Invasive breast carcinomas not segregated out as a special subtype are classified as invasive ductal carcinoma, no special type (NST). These tumors (and most of the special types) have prognosis and treatment plans based on the receptor status (and sometimes additional molecular profiling).
Receptor testing includes ER/PR/Her-2 and Ki-67 (proliferation marker)
Neoplastic proliferation resembling small ducts with expanded duct and acinar structures
Myoepithelial cell layer is intact surrounding the neoplastic proliferation (non-invasive tumor)
Typically express E-Cadherin
Bilateral in 10-20% of cases
Detected by mammography (not clinically evident)
Represents 15-30% of neoplasms identified in screening populations
Typically identified by abnormal calcification, sometimes as abnormal densities
Comedo DCIS
High grade pleomorphic nuclei
Central necrosis
Non-comedo DCIS
Lacks either high grade nuclei or central necrosis
Subtypes/patterns
Solid DCIS
Micropapillary DCIS
Cribiform DCIS
DCIS grading
Low-grade DCIS
1%/year risk of developing an invasive carcinoma
Intermediate-grade DCIS
High-grade DCIS
Paget Disease
Nipple manifestation of disease (1-4% of cases) – looks like eczema on the nipple
Tumor cells extend into the epidermis of the skin overlying the nipple from underlying DCIS within the ductal system of the breast.
50-60% of women will have an underlying palpable mass
Vast majority will have an invasive carcinoma (often ER neg./Her-2 pos.)
Women without a palpable mass will usually only have DCIS
Photomicrographs
References
Kumar, Vinay, Abul K. Abbas, and Jon C. Aster. Robbins and Cotran Pathologic Basis of Disease. Ninth edition. Philadelphia, PA: Elsevier/Saunders, 2015.
The Bloom-Richardson grading system was refined in the Nottingham/Tenovus Primary Breast Cancer Study (1974) into what is now referred to as the Nottingham grading system. This is a semi-quantitative evaluation with better inter-observer reproducibility and evaluation criteria.
For many years it was noted that tumor differentiation corresponded to prognosis. Bloom and Richardson in 1957 published the first widely known study, which showed that taking into account nuclear atypia, tubule formation, and mitotic activity of the tumor could result in a numeric grading system.
Atypical lobular hyperplasia is a proliferation of epithelial cells in the terminal duct-lobular unit that lacks expression of E-cadherin and fills the lobular unit (but does not expand). Some define ALH as filling or distending <50% of the acini within an affected lobule. There is some variation between experts as the exact differentiating line between ALH and lobular carcinoma in situ (LCIS). It is best to think of ALH and LCIS as representing a morphologic spectrum with filling and distention of lobular acini being the measured characteristic.
In some cases ALH/LCIS may appear similar to DCIS or merge with areas of DCIS. E-cadherin is a helpful marker to differentiate between these lesions. Clinically it is important to differentiate ALH/LCIS from DCIS because LCIS/ALH is an incidental finding on mammogram and represents a risk factor for bilateral breast carcinomas. Management is not through local excision, but surveillance and hormonal therapy dependent upon the type of lesion. DCIS is treated through local excision because these lesions tend to be localized/focal.
ALH carries an increased risk of developing an invasive breast carcinoma (4-5x relative risk, 13-17% lifetime risk), while LCIS carries an 8-10x increased relative risk, 25-30% lifetime risk). These are the same risks as ALH and DCIS, respectively. Although ALH/LCIS risk is bilateral, while ADH/DCIS tends to be more ipsilateral.
Kumar, Vinay, Abul K. Abbas, and Jon C. Aster. Robbins and Cotran Pathologic Basis of Disease. Ninth edition. Philadelphia, PA: Elsevier/Saunders, 2015. p. 1050-1051