|Year : 2012 | Volume
| Issue : 2 | Page : 323-325
Primary squamous cell carcinoma of the breast: Case report and management decisions
Nidhi Gupta1, Rajesh Vashisht1, Vinod Nimbran1, Rakesh Gupta1, Neerja Dhingra2, Anjali Bhutani2
1 Department of Radiation Oncology, Grecian Super Speciality Hospital, Sector 69, Mohali, Punjab, India
2 Department of Pathology, Grecian Super Speciality Hospital, Sector 69, Mohali, Punjab, India
|Date of Web Publication||26-Jul-2012|
G-427, GH-2, Shikhar Appartments, Mansa Devi Complex, Sector 5, Panchkula
Source of Support: None, Conflict of Interest: None
Though breast carcinoma is one of the most common malignancies worldwide, adequate knowledge of this disease is still very poor. Pure primary squamous cell carcinoma of breast is one such mystery, which is encountered very rarely. Its clinical behavior should not be correlated with the standard infiltrating duct carcinoma of breast. It follows an atypical presentation in terms of tumor size, lymph node and systemic spread. It is generally a triple negative tumor with an aggressive behavior and resistance to standard chemotherapeutic regimens. Management decisions need to be individualized with emphasis toward platinum-based chemotherapy and targeted therapy.
Keywords: Carcinoma breast, squamous cell, management
|How to cite this article:|
Gupta N, Vashisht R, Nimbran V, Gupta R, Dhingra N, Bhutani A. Primary squamous cell carcinoma of the breast: Case report and management decisions. J Can Res Ther 2012;8:323-5
|How to cite this URL:|
Gupta N, Vashisht R, Nimbran V, Gupta R, Dhingra N, Bhutani A. Primary squamous cell carcinoma of the breast: Case report and management decisions. J Can Res Ther [serial online] 2012 [cited 2019 Nov 19];8:323-5. Available from: http://www.cancerjournal.net/text.asp?2012/8/2/323/99006
| > Introduction|| |
Pure primary squamous cell carcinoma (SCC) of the breast is an extremely rare malignancy. It constitutes <0.1% of all primary invasive breast carcinomas.  Certain criteria need to be fulfilled before the tumor can be classified as a pure SCC of the breast. These are 1) greater than 90% of the malignant cells of squamous cell origin, 2) tumor independent of the overlying skin and nipple, 3) other sites of primary SCC excluded. , Clinical and radiographic characteristics are not specific for this tumor. In general, these are very aggressive, hormone receptor negative and treatment-refractory tumors with a poor prognosis.  We report a case of this rare breast malignancy and review the literature for appropriate management decisions.
| > Case Report|| |
A 63-year-old, postmenopausal women, noticed a lump in her right breast for the past one month, gradually progressive in size, not associated with nipple discharge, retraction or dimpling of skin. She was a multiparous lady who had breast fed all her three children and there was no family history of malignancy. Clinically she had a 6 × 3 cm lump in the retroareolar region with no palpable lymph nodes (cT3N0M0). Mammography as advised by the general physician was suggestive of a soft tissue lesion with spiculated margins in the retroareolar region of right breast -BIRADS 4(c), another well-defined cystic lesion seen in upper inner quadrant of left breast- BIRADS 2. FNAC of right breast lump was reported as fibrocystic breast disease. Thereafter, the patient underwent excision of the breast lump. Post operative histopathology of the breast lump showed infiltrating squamous cell carcinoma, and surgical margins were infiltrated by tumor. An extensive workup ruled out other primary sites or metastatic disease. Patient was then taken up for Rt modified radical mastectomy (MRM) with axillary lymph node dissection. Postoperative histopathology showed moderately differentiated squamous carcinoma of the breast, overlying skin and nipple was uninvolved [Figure 1]. Tumor cells were diffusely positive for cytokeratin 5 and 6 on immunohistochemistry. All the ten lymph nodes removed were negative for metastases (T3N0M0). Immunohistochemistry results showed a triple negative tumor (ER negative, PR negative, cerb B2 negative). Patient received six cycles of adjuvant chemotherapy with 5FU/epirubicin/cisplatin followed by postoperative radiotherapy to chest wall and drainage areas (50Gy/25#/5 weeks). One year post treatment, patient is locally and systemically free of disease.
| > Discussion|| |
Pure SCC of the breast is a rare and generally aggressive malignancy constituting < 0.1% of invasive breast cancers. The standard and leading text books of pathology and oncology do not mention pure SCC in their classification of malignant breast tumor. The origin of SCC in the breast is uncertain and its exact histogenesis remains obscure. It may arise directly from the epithelium of the mammary ducts or alternately it may arise from the foci of squamous metaplasia within a pre-existing adenocarcinoma. Malignant transformation of a deep-seated epidermal cyst can also be hypothesized. 
Radiologically, no typical mammographic appearances are seen except for lack of microcalcifications. Cystic lesions are characteristic presentations of SCC and more than 50% cases show cystic appearance.  Our patient typically had cystic lesions in the breast. Clinically they are indistinguishable from other breast malignancies and present as a usual hard breast lump. There are anecdotal reports of pyogenic abscess with underlying squamous cell malignancy.  Breast SCC are generally large (usually > 4 cm) at presentation, likewise our case was clinically T3 at presentation.
Contrary to the large primary tumor size, there is lower rate of lymph node metastases at presentation compared with similar lesions of infiltrating duct carcinoma (22% vs 40-60% IDC).  Similarly our patient had N0 disease in spite of extensive lymph node sampling. Unlike IDC, there is significant incidence of distant metastasis even without lymph node involvement in primary SCC of breast. These tumors are usually hormone receptor (ER/PR) and HER2/neu- negative while EGFR is frequently overexpressed.  Our patient was also triple negative on immunohistochemistry.
In the absence of adequate and accurate data, appropriate management remains controversial. Initial treatment is surgical by mastectomy with axillary clearance. Breast conservative surgeries in these patients are not usually possible because most patients present with locally advanced disease.  SCC of the breast is reported to be resistant to standard chemotherapy, for IDC (cyclophosphamide, methotrexate, 5-FU and adriamycin) as reported in studies using standard agents for neoadjuvant treatment to downstage the disease. Dejager et al. reported that a cisplatinum-based chemotherapeutic regimen commonly used for SCC of primary organs other than the breast was effective for SCC of the breast.  Radiotherapy has an important role in management in view of locally advanced disease at presentation. There is insufficient evidence about the activity of endocrine therapy for SCC, but it is reasonable to use endocrine treatment for patients with ER- and/or PR-positive SCC. The frequent expression of epidermal growth factor receptor (EGFR) in this disease may constitute a potential therapeutic target to be exploited, possibly in association with platinum-based treatment. 
Prognosis is controversial though majority studies suggest that it is an aggressive disease associated with frequent locoregional and distant relapses and resultant deaths. SCC of the breast is regarded as aggressive as grade 3 poorly differentiated, hormone receptor-negative adenocarcinoma.  In a series of 32 patients with breast SCC, Hennessy et al, reported 26% relapse free survival rate at five5 years with localized disease. The median overall survival was 37 months (range 12-108 months) with 40% surviving at five years. Median survival from the time recurrent disease was recognized was 14 months (range 2-86 months). 
| > Conclusion|| |
Pure primary SCC of the breast is a very rare malignancy. Clinical and radiographic characteristics are not specific except for the presence of cystic lesions, and usually present as large primary tumors with low LN involvement and are hormone receptor negative. In general, these are very aggressive, treatment-refractory tumors, with a poor prognosis. Use of anti-EGFR therapy, together with synergistic cytotoxics such as platinums and taxanes, should be explored in a clinical trial. Biologic studies to determine the reasons for chemotherapy resistance and to find other treatment targets can be undertaken.
| > References|| |
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