MALIGNANT PERIPHERAL NEURAL SHEATH TUMOR IN BREAST

Darley de L.F.Filho (Chefe do serviço de Mastologia e Reconstrução Mamária do Hospital Barão de Lucena (HBL) e Hospital da Polícia Militar de Pernambuco (HPMPE), Nancy Ferreira (Supervisora da Residência Médica de Mastologia do HBL)

Introduction: Malignant peripheral neural sheath tumor is a rare sarcoma subtype of ectomesenquimal origin. It accounts for 12% of soft tissue sarcomas and 0.65% of nerve sheath tumors originating from the center of the nervous system2, they can arise in irradiated fields, in patients with neurofibromatosis type 1 (NF1) or sporadic sites3. The most common site of involvement is deeper soft tissue, in the vicinity of a nerve trunk, the breast is extremely rare and, when it occurs, it presents as a nodule1. Because it is a rare tumor, there is controversy regarding its diagnosis and management because of inconsistent documentation. An unresolved question is whether NF1-related and sporadic forms would have a different prognosis and, therefore, should be managed differently3. Some authors affirm that total resection of the tumor would be the most indicated treatment, since it would have a reliable prognosis. It usually has aggressive behavior and somber prognosis2.Case report: AEMFA, 50 years old, Caucasian, was diagnosed with a 2.0 cm tumor in the upper right quadrant of the right breast through mammography and ultrasonography, and was first submitted to corebiopsy and then to a lumpectomy on 03/27/2015 at the Hospital of the Military Police of a nodule of benign characteristic, whose result of the histopathological was of Fibroadenoma .After 8 months, a new tumor appeared in the same place, where in the image examination it confirmed a solid tumor and irregular contours of 3.0 cm. She was submitted to a new surgery on 01/14/2016 at Barao de Lucena Hospital, where a segmental resection was performed in the upper inner quadrant, whose biopsy was Tumor Filodes Bordelaine. However, in a short interval, the tumor rebounded very rapidly, where a subcutaneous mastectomy was indicated with immediate breast reconstruction with a breast implant on 10/03/16. In the patient’s postoperative period, the patient continued with a lot of pain in the breast. Site of surgery and a tumor was observed growing at the height of the large pectoral muscle. A mammary gland exam was performed, which confirmed several tumors in the pectoral muscle area. It was discussed with the patient about the need for a new surgery, with the proposal of simple mastectomy and the use of an autologous tissue (large dorsal muscle) in immediate reconstruction, which was performed on 12/12/2016. The result of this biopsy was of a malignant phylum tumor with differential diagnosis of metaplastic carcinoma. In the immunohistochemical examination, the diagnosis was of a peripheral neural sheath tumor. At the moment the patient is undergoing chest MRI with several tumor foci in the right lung. He was referred for adjuvant therapy, and discussed the possibility of doing chemotherapy and radiotherapyConclusion: It is a rare tumor and, in addition to having aggressive behavior, presents a somber prognosis, which may vary according to its etiology. Therefore, more consistent information is needed, since there are few reported cases, which causes controversy in the management, in order to provide a more favorable prognosis.