Breast Sarcoma in a 16 Year-Old Girl

Abstract

INTRODUCTION: Sarcomas are histologically heterogeneous nonepithelial tumors, which may develop in the connective tissue of the breast, only in around 4, 6 cases per million women/year. They represent less than 1% of all the breast cancers; however their grim prognosis implies an importance in the study of these tumors. Sarcomas usually occur in women over 45 years of age. Furthermore, these malignancies can be classified according to their histology, as fibrosarcoma, angiosarcoma, and pleomorphic sarcoma. The patient appears with a fast growing mass in her breast. The prognosis depends on stage and histological grade, and treatment is mainly surgery. This case report is of a teenager without any family history who developed this rare malignancy. CASE REPORT: APLV, female, 16 years old, white, natural from and resident in Rio de Janeiro, Brazil, student, single, was referred to the senology department for further investigation of breast mass. The patient denied any previous illnesses, contact with Tuberculosis or anything important in the family history. Her physical exam presented with a 20cm tumor occupying the whole left breast, stretching the skin but without infiltrating it, with a hard consistency, and an areas of fluctuation. This area was drained and the liquid was sent to pathology for research for neoplastic cells, Tuberculosis, and other pathogens. The axillary lymph nodes were not palpable. A core-biopsy of the tumor was performed for diagnosis, and the patient was submitted to a breast sonogram and mammography. The cytology showed no signs of disease. The result of the core- biopsy was malignant mesenchymal neoplasm. The sonogram and the mammography showed a large hypoechoic/hyperdense mass occupying all quadrants of the left breast, of about 14 cm, suggesting a solid lesion. The immunohistochemistry research showed fusiform cells neoplasm with intense pleomorphism and necrosis, positive for vimentine, and negative for CD31, CD34, estrogen receptor, progesterone receptor and CK5. The screening for metastatic disease was negative, and the patient was submitted to mastectomy with immediate reconstruction with skin graft. The final result from pathology of the left breast was a malignant mesenchymal neoplasm with necrosis, and pronounced nuclear pleomorphism and high mitotic index. Nipple, skin, and surgical margins free from neoplasm. The patient was followed up at the hospital for one year and she has had a favorable outcome, with good healing and no metastasis so far. DISCUSSION: The diagnosis of breast sarcoma is very rare, and it is usually followed by a poor prognosis for the patient. It is important to report the rare cases in order to further understand and to clarify the aspects of this disease. The patient in this case report, epidemiologically different from the usual breast sarcoma patient, shows that anything is possible in oncology.