malignant fibrous histiocytoma ( mfh ) is a soft tissue neoplasm with poor prognosis , representing the most common soft tissue sarcoma of middle and late adulthood ( 1 , 2 ) . this tumor occurs most frequently in the lower and upper extremities and retroperitoneum , but the occurrence in the breast is very rare ( 1 ) . mfh is also known as malignant fibrous xanthoma , pleomorphic fibrous histiocytoma , and pleomorphic fibrous xanthoma ( 1 , 3 ) . it is one of the diverse group of benign and malignant tumors that o'brien and stout proposed first as having a common origin from the tissue histiocyte . the newer concepts of these disease entities have been accepted and refined ( 4 ) . it is possible that some of the tumors diagnosed in the past as mfh were actually pleomorphic variants of liposarcoma , rhabdomyosarcoma , and leiomyosarcoma , and conversely , some of the tumors diagnosed as the latter were mfh . recently , the diagnosis of mfh has become more accurate by the development of immunohistochemistry and electron microscopy ( 1 , 5 ) . in this case report , we present a 48-yr - old woman with mfh involving almost the entire left breast , of which the central portion has rotted due to extensive necrosis . a 48-yr - old woman presented with a huge necrotic mass in her left breast . she had first noticed the mass 1 yr before , since then it had rapidly grown and become necrotic . she was a mother of two children in good health , and had no family history of breast disease . on physical examination , the central portion of left breast including the nipple was rotted by severe necrosis , and the surrounding skin was erythematous . chest computed tomography ( ct ) demonstrated a hypodense mass , 108 cm in size , with central necrosis and numerous enlarged lymph nodes in the left axilla , but there was no abnormal finding in the lung fields . the tumor markers were within normal limits , including ca15 - 3 ( 5.0 u / ml ) and ca125 ( 7.2 u / ml ) . all laboratory findings , plain chest radiography film , and bone scintigraphy revealed no abnormalities . pseudomonas and candida were grown on a bacterial culture containing discharge from the necrotic tumor . although there were no symptoms or signs of generalized infection , systemic antibiotics were administered for fear of disseminating inflammation for several days before operation . at a later date , the patient underwent a radical mastectomy . in the operative field , numerous enlarged lymph nodes were observed in the left axilla . among these , the largest one was more than 2 cm in diameter , and diagnosed benign in the frozen section examination . because primary skin closure was impossible , the defect in the left anterior chest wall was covered with the myocutaneous flap using the left latissimus dorsi muscle . during the pathological examination , the breast was replaced by a 12.5115 cm gray , hard mass occupying almost the whole portion of the left breast , except pectoralis major muscle . on cut section , the mass showed areas of hemorrhage and necroses . the nipple and skin also showed necroses and involvement of a tumor component . in the microscopic examinations , the mass was composed of spindle - shaped tumor cells having polygonal nuclei and plump eosinophilic cytoplasm . the nuclei comprising giant cells were hyperchromatic and vesicular , and showed prominent nucleoli with frequent and abnormal mitoses ( fig . the cytoplasm of tumor cells were diffusely positive for vimentin and lysozyme , and negative for cytokeratin , epithelial membrane antigen , s-100 protein , and smooth muscle actin . the patient made an uneventful recovery . while the patient is hesitating to take postoperative adjuvant therapy , she had metastasis to lungs 2 months after the operation . primary malignancies of the breast stromal elements and sarcomas of mesenchymal origin comprise less than 1% of all breast neoplasms , and are a highly heterogeneous group of tumors . the majority of these are made up of malignant fibrous histiocytomas , fibrosarcomas , liposarcomas , and the less common angiosarcomas ; true " stromal sarcomas " of the breast are distinctly rare ( 6 ) . stromal sarcoma of the breast was initially defined as all sarcomas of the breast , excluding cystosarcoma phyllodes and angiosarcoma ( 7 ) ; but later it was redefined as rare sarcomas of a nonspecific type that arose in intralobular stroma ( 8) . since mfh and stromal sarcoma consist of spindle cells resembling fibroblasts , the only distinguishing feature are the primary involvement of extralobular stroma and intralobular stroma , respectively . jones et al . ( 9 ) proposed that mfh and fibrosarcoma have many features in common and thus , classification into one group or another can be arbitrary . therefore , it is reasonable to group both neoplasms together and divide them into low- and high - grade forms based on nuclear atypia and mitotic activity . mfh arises most frequently ( 58 - 75% ) from the deep fascia or skeletal muscle of the limbs , and then occurs frequently in the retroperitoneum and abdominal cavity with a frequency of 9 - 16% . however its primary development in the breast , not in combination with any other malignancies such as cystosarcoma phyllodes , or secondary to radiation therapy , is extremely rare ; and to our knowledge , only 25 cases have been reported in medical journals written in english ( 1 , 2 , 10 , 11 ) . histologically , the tumor is a pleomorphic sarcoma that contains two cell types : fibroblast - like cells and histiocyte - like cells . each tumor contains varying proportions of the two cell types so that a wide range of histologic features is possible ( 2 ) . as a result , mfhs have been characterized into the following subtypes : storiform - pleomorphic , myxoid , giant cell , and inflammatory ( 12 ) . most reported cases of primary mfh of the breast were of the storiform - pleomorphic variant ( 13 ) . recently , ajisaka et al . ( 2 ) reported the first case of the myxoid variant occurring as a primary tumor in the breast . to our knowledge , the giant cell variant has not yet been reported as a primary tumor like the myxoid variant . for the proper management and prediction of prognosis , differentiating malignant fibrous histiocytoma from other carcinoma or sarcoma is very important . differential diagnosis of mfh includes spindle cell ( metaplastic ) carcinoma , pleomorphic rhabdomyosarcoma , and malignant phyllodes tumor ( cystosarcoma phyllodes ) . spindle cell carcinoma , the more frequent form of breast carcinoma characterized by a sarcomatous metaplasia of invasive ductal carcinoma , is characterized by positive immunohistochemical staining for cytokeratin , epithelial membrane antigen , and er ( 14 ) while mfh is positive for vimentin and lysozyme . in our case , although the normal ducts were positive , tumor cells were exclusively negative for cytokeratin and epithelial membrane antigen . as mfh can also occurs as a component of cystosarcoma phyllodes , it is very important to differentiate them . to rule out the possibility of cystosarcoma phyllodes completely , many tissue sections for microscopic examination are recommended and immunoreactivity for alph-1-antitrypsin , s-100 protein , and lysozyme should be evaluated . metastases of mfh are mainly hematogenous , and lymph node involvement is very unusual . when the involvement of lymph node is present , it is considered as a sign of a widely disseminated disease ( 12 ) . for this reason , axillary node dissection has not generally been recommended in the treatment of this tumor ( 3 ) . however , clarifying lymph node status by dissection is useful for predicting the prognosis , and helping to determine the indications for the postoperative adjuvant therapy . ( 15 ) , who for the first time found metastasis in the axillary lymph nodes , asserted lymphadenectomy to be mandatory . moreover , axillary lymph node dissection is easy to perform without significant morbidity . consequently , it will be up to the surgeon in case by case matter to decide whether axillary dissection is necessary or not . our patient was treated by total mastectomy , including resection of the pectoral muscles and lymphadenectomy was performed to level i. there has been no evidence of metastasis to the lymph nodes during the final pathologic examination . in terms of the postoperative adjuvant therapy , radiation therapy seems to play an important role ( 16 ) . the effect of chemotherapy , however , is still uncertain , although clamon et al . ( 17 ) reported a remission of more than 42 months by using aggressive combination chemotherapy . , the metastases of the lungs were found 2 months during which the patient had hesitated to take the adjuvant therapy , and she died 4 months later . in spite of its rarity , surgeons should be alert to the existence of this type of soft tissue tumors in the breast . more information about the biological and pathological behavior of these tumors is necessary in order to expand and improve our ability to treat these patients . so far , surgery is the first step in the treatment . although it remains doubtful whether radical or modified radical mastectomy is superior to wide local excision , the important point is to secure safe surgical margins of more than 3 cm ( 5 ) , in which ever procedure that would be carried out .
a case of primary malignant fibrous histiocytoma of the breast is reported . 
 the patient was a 48-yr - old woman with a huge tumor involving almost the entire left breast . 
 the central portion of her left breast was already rotted by extensive necrosis and inflammation . 
 she was treated by radical mastectomy and axillary lymphadenectomy to level i. pathologic examination supported by an immunohistochemical staining confirmed the tumor as malignant fibrous histiocytoma of giant cell type . 
 axillary lymph nodes were free from tumor metastasis . 
 she had not taken any postoperative adjuvant therapy . 
 the metastasis to lungs was found 2 months after the operation , and she died within 6 months .