metaplastic breast carcinoma ( mbc ) is a rare aggressive breast malignancy with poor prognosis . there are no specific recommendations for mbc management , the standard regimen being surgery combined with adjuvant therapy , although there is evidence to suggest that mbcs have lower response rates to conventional adjuvant chemotherapy and a worse clinical outcome than other forms of breast cancers negative for estrogen receptor , progesterone receptor and human epidermal growth factor receptor 2 ( triple negative ) . in younger women , we report a case of an 18-year - old african female patient with mbc , who was subjected to the standard of care , but had a progression - free survival ( pfs ) of 6 months and an overall survival ( os ) of 9 months , thus supporting the poor prognosis of mbc in young women . an 18-year - old african female patient with no relevant past or family history was admitted at our hospital due to a 34-cm tumor on the right breast ( fig .  1 ) . the patient , from cape verde , delayed her appointment with a doctor because she could not afford to miss a day at work . when she finally went to the doctor , due to rapid growth of the mass , the tumor was extremely large , and she was immediately evacuated to portugal . thorax , abdomen and pelvic ct showed axillary lymph node involvement , without other secondary foci . a multidisciplinary team decided that a modified right radical mastectomy should be performed ( fig . figure  2:mri t1f study after paramagnetic contrast showing a large mass occupying the entire right breast with extensive central necrosis . mri t1f study after paramagnetic contrast showing a large mass occupying the entire right breast with extensive central necrosis . pathology confirmed a widely necrotic tumor with 3500 g and 34 cm , replacing the whole breast . histology showed atypical spindle and some epitheliod cells , with high nuclear pleomorphism , organized in long fascicles with an interwoven pattern and in a hemangiopericytoid fashion ( fig . 4a and b ) ; the mitotic index was high , with > 20 mitosis/10 high - power fields . there was focal expression of high - molecular - weight keratins ck903 and ae1/3 ( fig . based on the unequivocal expression of high - molecular - weight keratins , a diagnosis of metaplastic spindle cell carcinoma was rendered . figure  4:(a ) spindle cell proliferation with a hemangiopericitoid growth pattern ( 4 ) . six months after surgery , the patient showed a hard and painful nodule adjacent to the suture and adherent to the chest wall . chest ct revealed right pleural effusion , pleural thickening and consolidation of the adjacent parenchyma . figure  5:ultrasound of the mastectomy bed showing a hypoechoic solid mass in subpectoral topography with signs of muscle invasion . ultrasound of the mastectomy bed showing a hypoechoic solid mass in subpectoral topography with signs of muscle invasion . given the chest wall involvement , the tumor was considered unresectable and the patient evolved with rapid systemic deterioration and multiple organ failure . young women are more likely to develop more aggressive subtypes of breast cancer with poor prognosis , probably due to multiple factors , including unfavorable disease biology , delay in diagnosis and age . on the other hand , mbcs are aggressive regardless of age , usually characterized by rapid progression , needing immediate surgical treatment and en bloc resection . mbcs account for 0.25% of all invasive breast cancers , which usually present as high - grade neoplasms with a large tumor size , less likely nodal involvement , triple negative , poorly differentiated or undifferentiated [ 1 , 4 , 5 ] and high ki67 . our patient presented with these characteristics , but was much younger than the reported mean age of 6163 years [ 1 , 4 , 6 ] . also , the tumor was much larger than the reported range of 3.413 cm [ 7 , 8 ] . although with the use of neoadjuvant therapy for downstaging and downsizing , together with currently available oncoplastic techniques , it is possible to perform conservative breast oncologic surgery in cases previously treated with radical mastectomy , our first surgical option was a modified right radical mastectomy given the exceptionally large tumor , followed by adjuvant chemotherapy . the use of adjuvant therapy has been the subject of some debate [ 1 , 4 , 68 ] . in our case , since there is no standard treatment regimen specific for mbc , we followed the clinical practice guidelines for invasive breast adenocarcinoma , and adjuvant chemotherapy was decided by our breast cancer multidisciplinary group , given it is used more often for patients with mbc . also , its use in breast cancer is based on disease stage , not histologic subtype , as the prognostic value of histological grading in mbcs is uncertain . owing to several clinical complications arising during the ct cycles , relapse and deterioration of the patient 's general health status , adjuvant radiotherapy could not be offered . despite the aggressive treatment , pfs and os of our patient were much lower than in other studies [ 1 , 5 , 8 ] . this dismal scenario was not surprising given the tumor presentation at hospital admittance and the patient 's age and ethnicity . not only breast cancer in young women has been associated with more aggressive , poorly differentiated , estrogen receptor ( er ) negative tumors , but also african - american women have an increased risk of breast cancer at younger ages and are more likely to have higher grade , er - negative cancers . presently , there are no validated prognostic markers for metaplastic carcinomas . it is of paramount importance to rapidly identify these tumors and treat them aggressively , which can only be achieved with a multidisciplinary approach . the case we report did not have a good outcome , for a number of reasons . although mbc is rare , there are still a significant number of patients who , due to limited access to primary health care , present with very advanced tumors at hospital admittance , and there is a need to know how to manage these patients , in order to achieve the best possible outcome . the procedures followed were in accordance with the ethical standards of the helsinki declaration of the world medical association . the work has been approved by the hospital professor doutor fernando da fonseca ethical committee .
metaplastic breast carcinomas ( mbcs ) are rare malignancies usually with poor prognosis . 
 we report a case of an 18-year - old african female patient who presented with a 34-cm tumor on the right breast . 
 biopsy showed an extensively necrotic mbc negative for estrogen receptor , progesterone receptor and human epidermal growth factor receptor 2 ( triple negative ) . 
 a modified right radical mastectomy was performed , followed by adjuvant chemotherapy . 
 histology confirmed a widely necrotic undifferentiated malignant tumor , with strong and diffuse expression of vimentin and b - cell lymphoma 2 , focal high - molecular - weight keratins and focal cd34 expression ; ki67 was > 90% . 
 there was no skin , deep margin or lymph node involvement . 
 six months after surgery , the patient showed a 9  7 cm nodule adjacent to the suture and adherent to the anterior chest wall . 
 the tumor was considered unresectable and the patient evolved with rapid systemic deterioration . 
 the patient had a progression - free survival of 6 months and overall survival of 9 months .