Bilateral Primary Squamous Cell Carcinoma of Breast in a Pre Menopausal Woman.
Primary squamous cell carcinoma of breast is an extremely rare histological variant and constitutes about less than 1% of breast cancers. All published cases of primary squamous cell carcinoma of breast are unilateral. Bilateral involvement of breast is not reported in the literature. We present a case of bilateral primary squamous cell carcinoma of breast in a pre menopausal woman presenting as fungating breast mass. This report discusses the clinical presentation, radiological and histology findings along with review of the literature.
Key words: Breast cancer, squamous cell carcinoma, hormonal receptors, chemotherapy, radiotherapy, breast surgery.
Breast cancer is the commonest cancer in women in developed countries1 and also in Malaysia. The annual incidence of breast cancer is about 31% as reported in the National Cancer Registry of Malaysia in 20032. Primary squamous cell carcinoma of the breast is extremely rare with specific pathological and radiological criteria. The criteria used are: 1. the tumor should not originate from the skin overlying the breast or nipple or skin adenexal elements, 2. greater than 90% of the tumor must be squamous, 3. absence of neoplastic, ductal or mesenchymal elements on thorough sampling and 4. exclusion of metastasis from non- mammary sites3,4. Only 47 such cases have been reported in the literature as per laid down criteria5,6 Literature review shows only single sided lesions without lymph node involvement. We present a case of primary squamous cell carcinoma of breast which occurred bilaterally with lymph node involvement along with review of literature and management.
Two enlarged lymph nodes measuring about 4 cm. and 2 cm. respectively were felt in the right axilla. An initial diagnosis of carcinoma of both breasts, T4N2Mx on the right side and T4NxMx on the left, was made. Fine needle aspiration cytology of the right breast mass and the enlarged axillary lymph node was done. Wedge biopsies from both the right and the left breast were also taken.
Fine needle aspiration cytology from breast and lymph node, showed pleomorphic polygonal to spindle epithelial cells having large nuclei with coarse chromatin clumping, and prominent nucleoli. Occasional keratinisation was seen in the malignant cells and tumor diathesis was present in the background. Wedge biopsy from both breasts showed partly ulcerated skin and underlying markedly necrotic squamous cell carcinoma with moderate to poor differentiation (Figure-2). Intercellular bridges and single cell keratinisation were present at some foci. No breast lobules, ducts or glandular lumina were identified. No focus of adenocarcioma was detected and stains for mucin was negative. Immunohistochemical markers for estrogen and progesterone were negative. Stain for CerbB-2 marker was positive in both the tumors (Figure-3). Diagnosis of squamous cell carcinoma of both breasts with moderate differentiation was made.
Primary squamous cell carcinoma of the breast is a rare disease with an incidence ranging from 0.06 to 2% in various studies7,8. The origin of the squamous cells within the breast is unknown. There is no agreement among the pathologists about the tumor histogenesis with some proposing the theory of squamous metaplasia within breast adenocarcinoma9. This is supported by Stevenson et al10 who described primary squamous cell carcinoma identified on light microscopy and subjected to ultrastructural analysis. He showed presence of either separate squamous or glandular cells or both components coexisting in the same cell or squamous metaplasia associated with chronic inflammation11, as seen in conditions like chronic osteomyelitis, hidradentis and Marjolins ulcers in burns. Others suggest that it may originate from chronic abscess or keratinous cysts of breast12,13. Literature search shows primary squamous cell carcinoma involving single breast only.
However, in our case both the breasts were involved in an advanc d stage. The history given by the patient that the tumors appeared within a short period of each other and that no nodule was seen on the skin of both the breasts, supports the theory of two independent tumors arising in the two breasts. Primary squamous cell carcinoma of breast has an incidence of lymph node metastasis in 30% of the cases. In the present case, the right axillary lymph node involvement was proven on Fine needle aspiration cytology. On the contralateral side no lymph node was palpable. As in other cases, our patient also did not show estrogen or progesterone receptors14,15. Positive CerbB-2 status, as seen in this case, was not reported in the available literature. Fine needle aspiration from both tumors revealed only squamous elements and no adenocarcinoma. Due to the absence of precancerous etiology in our case, we strongly believe the origin of this cancer in both the breasts is de novo.
The transcutaneous spread is not possible as both the breast had tumor masses at the same time.
Wedge biopsy, though not very deep, showed only squamous carcinoma and no involvement of the skin and no adenocarcinoma. Extensive sampling of the tumors and surrounding breast tissue after mastectomy would have given the definite answer regarding the presence or absence of metaplastic squamous epithelium in the ducts or any other associated pathology. However, patient died of septicemia before she could be taken up for palliative mastectomy.
Fine needle aspiration cytology is useful for diagnosis even though some difficulties may be experienced in well differentiated tumors with necrosis or inflammation5. A biopsy gives a definitive diagnosis and also sufficient sample for immunohistochemical stains for the receptor status. Specific mammography findings have not been described for this tumor due to its rare presentation6. Literature mostly shows pooled data of different cases after reviewing the slides retrospectively12,16,17.
As in any other breast cancer, simple mastectomy with axillary clearance or segmental resection depending on the tumor size is the treatment of choice. There is no consensus among the surgeons and oncologists regarding adjuvant treatment in the form of radiotherapy or chemotherapy6,14. Chemotherapy regimes used cisplatinum along with 5 Flurouracil with or with out adriamycin3,10,15,18. Radiotherapy is given in the postoperative period to minimize the loco-regional recurrences19,20.
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Department of Nuclear Medicine, Radiotherapy and Oncology, Hospital University Sains Malaysia, Kubang Kerian, Kelantan.
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|Author:||Pasha, Mehboob Alam; Krishnabhavaraju, Venkata Murali; Hamzah, Azhar bin Amir; Meng, Leow Voon|
|Publication:||Pakistan Journal of Medical Research|
|Date:||Dec 31, 2010|
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