breast sarcoma is an extremely rare and heterogeneous disease , which includes a group of several subtypes of cancer [ 1 , 2 , 3 , 4 , 5 , 6 , 7 ] . they can appear as primary forms or secondary to radiation therapy or chronic lymphedema [ 3 , 8 ] . primary breast sarcomas ( pbs ) are malignant tumors arising from the connective tissue within the breast and account for less than 1% of breast cancer and no more than 5% of all sarcomas [ 1 , 9 ] . the published literature regarding breast sarcomas is predominantly composed of small retrospective analyses and case reports . these studies include patients with a wide variety of histologic features , treated over many decades with great variability in surgical approaches , and inconsistencies on what concerns the effectiveness of adjuvant therapies . pbs is mostly diagnosed in middle - aged patients in their fifth or sixth decade of life [ 1 , 10 ] . the typical patient presents with a painless well - circumscribed and mobile unilateral mass , with variable size ( ranging from 1 to 40 cm ) and a faster growth rate compared to epithelial breast cancer [ 1 , 10 , 11 ] . the gross appearance is influenced by the specific histologic features [ 1 , 3 ] . unlike epithelial breast carcinoma , the most common mode of dissemination is hematogenous , typically to the lungs , bone marrow , and liver . lymphatic spread is uncommon and so axillary lymph node involvement is not a frequent finding [ 1 , 3 , 4 , 12 ] . the breast skin and the nipple areola complex are rarely involved in breast sarcomas . prognosis is based on tumor size , the presence of regional or distant metastases , and histological grade . there is no definitive consensus regarding the treatment of breast sarcomas . due to the rarity and heterogeneity of breast sarcomas , there are no guidelines or published randomized trials that validate any particular treatment strategy . however , there is general agreement that surgical excision with adequate margins free of cancer cells should always be the first modality of treatment [ 1 , 2 , 3 , 5 , 12 ] . radiation therapy and chemotherapy , although controversial , may be considered in patients with high risk of recurrence due to positive margin resection , tumors larger than 5 cm , or a high - grade sarcoma , since these lesions have a tendency to recur locally and can metastasize [ 1 , 2 , 3 , 4 ] . breast sarcomas usually have a poor prognosis , with high risk of recurrence , 80% developing during the first 2 years after diagnosis . we report a case of a previously healthy 52-year - old woman ( perimenopausal ) , 1 delivery at 25 years of age , with regular breast surveillance and a normal mammography 2 years before . she presented an extremely voluminous tumor in the left breast with rapid growth , which appeared 10 days before admission , associated with a very thin and preulcerative skin over the last 4 days . physical examination revealed quite a voluminous left breast , subverted by a very large tumor ( > 20 cm ) , with bosselated contours , hypervascularized , firm , with soft cystic and hemorrhagic areas ( fig 1 ) . hemoglobin dropped to 8.8 g / dl during this occurrence . in order to control bleeding , a total mastectomy and homolateral axillary sentinel node biopsy were performed as it was surgically achievable and no systemic dissemination was found . histopathology displayed a malignant phyllodes tumor , with 24 cm of longer axis , widely ulcerated , with focally tangential margins , and the malignant mesenchymal component matched a high - grade leiomyosarcoma , with marked mitotic activity and a diffusely infiltrative pattern . due to very fast development and growth as well as the aggressive histological characteristics , 10 cycles of adjuvant chemotherapy with doxorubicin and dacarbazine ( adic ) + ifosfamide ( ifx ) , followed by radiotherapy ( a total dose of 50 gy in 25 fractions over 5 weeks to the chest wall , plus a boost to the primary site of 20 gy in 10 fractions over 2 weeks ) were administered after informed consent , and it was well tolerated . clinical and ultrasound examination were performed every 6 months , and mammography every year for the surveillance of the contralateral breast . after 5 years of follow - up , no recurrence was found and the patient is alive and free of disease ( fig 3 ) . pbs is a rare and very aggressive entity , comparable to other soft tissue sarcomas and is totally independent of epithelial breast cancers [ 9 , 11 ] . due to its rarity the optimal treatment for breast sarcoma involves a multidisciplinary working team , and patients should be treated according to the guidelines referring to soft tissue sarcomas . excisional biopsy should not be performed unless previous attempts at diagnosis were unsuccessful [ 3 , 10 , 11 ] . the most commonly used staging system for breast sarcomas is the american joint committee on cancer staging system for soft tissue sarcoma , which includes histological grade , tumor size , lymphatic involvement , and distant metastization . the treatment regimen should be individualized , and a multidisciplinary approach is mandatory [ 8 , 11 ] . the first - line treatment is complete surgical excision of the tumor with adequate margins , regardless of histological subtype , based on the evidence that local recurrence is more frequent in patients with surgical margins less than 12 cm . negative surgical margins are more effective markers for local recurrence and overall survival than the extent of surgical resection . lymph node involvement is rare and may occur in the carcinosarcoma subtype or when the disease is already disseminated . thus , axillary lymph node dissection is only recommended in case of enlarged lymph nodes , suspicious lymph nodes on breast imaging studies , or in case of the carcinosarcoma subtype . sentinel lymph node biopsy was performed in this case considering the very large and aggressive tumor that could comprise areas of carcinosarcoma . adjuvant radiotherapy and chemotherapy for pbs is not consensual and depends mainly on the risk of tumor recurrence . there is evidence of benefit with adjuvant radiotherapy for large ( > 5 cm ) , high - grade tumors or in patients with positive surgical margins in reducing rates of locoregional recurrence ; the impact on overall survival remains uncertain . soft tissue sarcomas are often minimally chemosensitive , with response rates ranging from 20 to 40% [ 1 , 14 ] . adjuvant chemotherapy is a reasonable option for patients who have a good functional status and a high - risk primary sarcoma or recurrent sarcoma . size seems to be the most frequently reliable prognostic factor in many series , with 5 cm serving as a valuable cut point . studies have demonstrated a 5-year disease - free survival ranging from 44 to 66% and a 5-year overall survival rate ranging from 49 to 67% . most detrimental events , such as local or distant recurrence and death , occur most frequently during the first years after diagnosis [ 1 , 3 , 11 ] . although rare , pbs is a very aggressive disease , with rapid growth , distinct from breast carcinoma . dissemination is primarily hematogenous , typically to the lungs , bone marrow , and liver . adjuvant therapy , although controversial , may be considered in tumors with high risk of recurrence . the prognosis is poor and detrimental events occur most frequently during the first years of follow - up . written informed consent was obtained from the patient for publication of this case report and the accompanying images .
primary breast sarcoma , arising from connective tissue within the breast , is extremely rare , accounting for less than 1% of all primary breast malignancies and no more than 5% of all sarcomas . 
 the rarity of this pathology limits most studies to case reports and small retrospective studies , which has led to a lack of consensus on the clinical management . 
 we report a clinical case of a 52-year - old woman , perimenopausal , previously healthy , with regular breast surveillance , who presented with a large ( > 20 cm ) and rapidly expanding hypervascularized tumor of the left breast developed over 10 days , with a very thin preulcerative skin over the last 4 days . 
 there was no systemic dissemination . 
 the patient was submitted to total mastectomy and excision of axillary adenopathy . 
 the tumor was diagnosed histologically as malignant phyllodes tumor associated with areas of high - grade sarcoma . due to rapid growth and aggressive histological characteristics , adjuvant 
 chemotherapy and radiotherapy were performed . 
 there is a lot of evidence that tumors larger than 5 cm are associated with a poor prognosis . despite the poor prognosis associated with this aggressive entity , the patient had no recurrence during 5 years of follow - up . 
 we review the relevant literature about primary breast sarcomas .