angiosarcoma ( as ) , also known as malignant hemangioendothelioma , is a rare and aggressive malignant tumor of endothelial cells of vascular or lymphatic origin . these tumors typically have a poor prognosis , and they represent less than 1% of all malignant tumors1 and between 1% to 2% of soft tissue sarcomas234 . about 50% to 52% of all ass occur in the head and neck35 and are found predominantly on the scalp and face of elderly men25 . one study showed that ass represented 11.2% of all sarcomas in the the head and neck region2 . as is extremely rare in the oral cavity and salivary glands , accounting for 1% of all ass reported in the literature14 . although the tumor can occur in any location , the most common sites are soft tissue , skin6 , trunk , and limbs7 . the tumor type is represented equally between sexes37 ; however , fanburg - smith et al.8 suggest a mild male predominance ( ratio 1.07:1 ) . there is inconsistency in the data on age - related tumor involvement because some authors postulate that older patients have a greater propensity for as , while others suggest that as favors those of a younger age7 . in general , all age groups can be affected , though it is prevalent among older white men and often presents as a cutaneous lesions1 . the median age of those who develop as , according to yang et al.3 , is 60 to 70 years , and the mean age , according to fanburg - smith et al.8 , is 55 years . ass are highly aggressive and can arise in association with some recognized clinical conditions , such as chronic lymphedema and previous radiotherapy , or they can arise de novo2 . as is initially inconspicuous and is often diagnosed as a simple bruise . it expands with gradual centrifugal infiltration , eventually resulting in nodule formation and skin ulceration2 . as presents as an extremely aggressive lesion with propensity for local recurrence and distant metastasis , leading to poor prognosis3 . the overall prognosis of as is poor , even compared with that of other head and neck sarcomas2 . the five - year survival in the head and neck is 10% to 35%34 , and approximately half of as patients die within 15 months of diagnosis4 . we present a case of a 35-year - old male with primary as of the cheek initially misdiagnosed as sclerozing hemangioma and a review of relevant literature . a 35-year - old male was seen at the maxillofacial surgery clinic of our hospital with a four - year history of left facial swelling of insidious growth . he reported a history of assault by armed robbers who hit him with a gun on the left cheek , where he developed swelling eight months later . three months prior to his presentation at our center , he presented at a peripheral hospital where he underwent a surgical procedure and received a histopathological report of sclerosing hemangioma . at presentation , the left cheek mass was covered with a dressing ( fig . 1 ) , which on exposure revealed an ulcerated mass with no discharge or bleeding . the mass measured 10 cm by 7 cm and was firm to the touch . it was attached to underlying structures and protruded intraorally with indentation of the overlying mucosa . a computerized tomographic angiogram of the cheek ( fig . 2 ) revealed a rounded , isodense and brightly enhancing mass over the body and anterior to the ramus of the left hemimandible . the enhancement was the same density as the adjacent left facial artery , which coursed through the medial aspect of the mass . other tests such as full blood chemistry , electrolyte count , creatinine , and random blood glucose were within the normal range for his age and sex . he was prepared for surgery , and on the operating table , the feeder vessel was ligated ( fig . the mass was surgically removed via an extraoral approach under general anesthesia . the whole specimen ( fig . 4 ) was sent to the histopathology laboratory of our hospital and was reported as as.(fig . 5 ) he had an uneventful recovery and was transferred to the oncology team for further management on the seventh postoperative day . his management was interrupted by a health worker strike , and he was discharged home . no obvious or residual tumour was seen , and he declined further medical intervention , including chemotherapy and radiotherapy . the tumor specimen was in the form of two irregular shaped tissues , one of which was covered with skin and the other ulcerated , measuring 87.53 cm and 996 cm , respectively , together weighing 179 g. the cut section revealed grey , white , solid , and hemorrhagic nodular surfaces . histology sections showed keratinized pigmented skin overlying a fibrocollagenized dermis and a malignant spindle cell tumor growing in sheets , with anastomosing dilated vascular channels . the cells comprising the tumor were plump and round to spindle - shaped with hyperchromatic to vesicular nuclei , prominent nucleoli , and scanty cytoplasm . as is said to account for about 2% of all soft tissue sarcomas , while oral as is very rare349 . the etiology of as is not fully understood2 , although the sun has been implicated as a causative agent in caucasian patients5 . chronic lymphedema and irradiation have been described as the most important etiological factors in the pathogenesis of as3 . only about 10% of ass are associated with chronic lymphedema , even though it is the most widely accepted sign of as in skin and soft tissue lesions3 . radiation - induced as usually occurs in the irradiated fields many years after radiotherapy , with a median time of 8.6 years having been reported3 . as is also said to occur in pre - existing or intermediate vascular lesions . several cases of as arising spontaneously from hemangiomas or vascular malformation have been reported , but the mechanism is not clear3 . even though most authors think trauma can only alter an already existing lesion , it has been reported as an etiological factor3 . fanburg - smith et al.8 reported 29 cases with 22 primary as lesions in the tongue ( n=9 ) , parotid gland ( n=4 ) , lip ( n=4 ) , submandibular gland ( n=3 ) , soft palate ( n=1 ) , and hard palate ( n=1 ) and seven secondary lesions in the gingivae ( n=4 ) and parotid gland ( n=3 ) . our case occurred in the cheek . as can arise from either a vascular or lymphatic origin , and predisposing factors to its development include irradiation , trauma , and advanced age . our patient reported a definite history of trauma , although the etiology and triggering factors are largely unknown in many cases . as has been categorized into five broad groups based on a wide range of associated clinical scenarios : lymphedemaassociated as , radiation - induced as , post - breast cancer as , soft tissue as , and cutaneous as . as of the oral cavity can involve oral soft tissue , minor salivary glands , or bones10 . our patient 's as affected the oral soft tissue of the cheek . several reports of primary as involving the oral soft tissue , lip , palate , tongue , parotid gland , and submandibular glands are also documented . the clinical presentation is often a mass and/or bleeding , pain , bruising , persistent hematoma , edema and ulceration , or round or ovoid nodules that appear red to bluish in color3468 . the clinical differential diagnoses include pyogenic granuloma , hemangioma , hemagioendothelioma , kaposi 's sarcoma , and ulcerative squamous cell carcinoma34 . our working diagnosis in this case was sclerosing hemangioma based on the histology report and the computed tomography ( ct ) scan the patient brought from another center . our case was initially reported as sclerosing hemangioma , and while a ct scan supported a facial artery aneurysm , other missed diagnoses include pyogenic granuloma1 . delayed or missed diagnosis is associated with poor treatment outcome1 . according to nagata et al.4 , as can show a wide spectrum of histological differentiation , from a well differentiated neoplasm showing anastomosing vascular channels lined by atypical endothelial cells with low mitotic activity to poorly differentiated tumors without prominent vasoformative activity , composed of solid sheets of epithelial - like or spindle cells4 . fanburg - smith et al.8 stated that the most common morphologic type of as is solid spindled vasoformative , but that almost one - third of oral and salivary gland ass are the rare epitheloid variant . immunohistochemical analysis is essential for differentiating as from similar lesions such as hemangioendothelioma , hemangiopericytoma , epithelioid sarcoma , kaposi 's sarcoma611 , perivascular epithelioid tumor ( malignant pecoma ) , spindle cell carcinoma , malignant melanoma , intravascular endothelial hyperplasia , epithelioid as , and lymphangiosarcoma6 . positive staining for factor vii and ulex europaeus agglutinin has been used to diagnose vascular pathology . cd31 , a membrane glycoprotein , is the best vascular marker of endothelial phenotypes , particularly in poorly differentiated as11 , where factor viii and u. europaeus are negative . tumors showing vasoformative channels and intracytoplasmic vacuoles on hematoxylin and eosin sections are likely angiogenic . positive reactions to f - viii - ra , cd34 , and cd31 endothelial markers indicate endothelial characteristics46 . the presence of red blood cells in the vasoformative channels suggests that the tumor is not a lymphatic tumor but a vascular tumor6 . surgery is the main stay of treatment , and local primary tumors can be effectively excised . however , there is a high associated rate of local recurrence and distant metastasis , which negatively impacts patient survival9 . according to khler et al.2 , the lesion must be excised with adequate three - dimensional surgical margins , which can be difficult to achieve in the head and neck region . the general outcome of primary as of the oral cavity is good , with a mean survival period of 7.3 to 7.6 years , compared to primary cutaneous and deep soft tissue tumors611 . because most patients present with systemic metastasis , chemotherapy is often involved in the treatment of as . complete remission of as has been reported with the use of liposomal doxorubicin alone or in association with radiotherapy23 . though there was no evidence of residual disease on follow - up , the risk of recurrence without further adjunct therapy can not be overlooked . due to its rarity , as is associated with no significant or clearly definitive prognostic factors2 . lesion size has been used as the only significant predictor , while histology has had no impact . additionally , the lesion site was considered a significant prognostic factor as tumors in the scalp had a worse prognosis . metastatic spread is usually hematogenous , and a common site of involvement is the lungs . lymphatic spread is reported in less than 20% of ass involving the head and neck region11 . in our case , no lymph node involvement or distant metastases were observed at initial presentation or during the follow - up period ; however , we did not carry out any lymph node survey . primary as of the cheek is rare , and its diagnosis and treatment still pose a challenge in clinical settings .
angiosarcoma is a rare and aggressive malignant tumor that has a poor prognosis . 
 it represents less than 1% of all malignancies occurring in the oral cavity and salivary glands . 
 we present a 35-year - old male with angiosarcoma of the cheek following traumatic injury and a review of the current literature .