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Background: Primary squamous cell carcinoma of the breast (PSCCB) is one of the rarest forms of breast cancer, accounting for less than 0.1% of all breast cancers. PSCCB can be aggressive with no typical radiologic findings on mammogram and a wide range of clinical presentations, often leading to delayed or missed diagnoses. Methods: We performed a retrospective review of patients diagnosed with PSCCB at our institution from 2007 to 2012. Inclusion criteria included females over the age of 18 years old with a primary tumor consisting of >90% malignant cells of squamous origin and excluded squamous cell cancer that had metastasized to the breast or lesions that were not independent of the overlying skin or nipple. In addition, we performed a literature review using the PubMed database. Results: We identified two patients diagnosed with PSCCB during that timeframe with ages ranging from 35-52 years old. The average size of the mass seen on mammogram was 2.1 cm (range of 1 cm to 3.6 cm). Histologically, all cancers were moderately to poorly differentiated with negative estrogen, progesterone, and HER2 receptor status and an elevated Ki-67. One patient underwent breast conservation with partial mastectomy and axillary sentinel lymph node biopsy with whole breast radiation. The second patient underwent a total mastectomy and axillary sentinel lymph node biopsy without radiation. Neither of the patients had evidence of regional nodal disease at time of surgery nor received any systemic therapy. Both patients were disease free at last follow up which was five to twenty eight months respectively. Conclusion: Because PSCCB is so rare, prognosis and optimal treatment are still controversial, making treatment options limited. More research is needed to further elucidate the biological behavior of this rare cancer as well prognostic factors that may allow more conservative treatment.

A Retrospective Case Series and Literature Review of Primary Squamous Cell Cancer of the Breast