carcinosarcoma ( also known as pseudosarcoma , pseudosarcomatous squamous cell carcinoma , pleomorphic carcinoma , and spindle cell carcinoma ) is a highly malignant tumor characterized by dual malignant histologic differentiation of the epithelial component consisting of a focally squamous cell carcinoma and a mesenchymal component having a sarcomatoid stroma .
carcinosarcoma may arise in any squamous epithelium of the body , however its occurrence is extremely rare in the sinonasal cavity [ 3 - 13 ] .
we present a case of a carcinosarcoma located in the maxillary sinus that was is aggressive and displayed a high recurrence rate even when a multimodality therapeutic approach was adopted .
a 62-year - old male was admitted to the emergency room due to uncontrolled epistaxis and complaining of swelling and tenderness in the right cheek .
the patient was receiving warfarin as an anticoagulative measure , having previously suffered a stroke .
an examination of the nasal cavity was not possible at the emergency room due to the severity of bleeding .
two days later , when the nasal packing was removed and endoscopic examination performed , a mass in the middle meatus and bulging of the medial wall of the maxillary sinus were observed ( fig .
computed tomography ( ct ) and magnetic resonance imaging ( mri ) scans revealed a large mass comprised of soft tissue with an ill - defined outline and an irregular peripheral enhancement on the right maxillary sinus .
the mass had pushed against the right inferior wall of the maxillary sinus ; destroyed the infraorbital foramen ; completely destroyed the medial wall of the maxillary sinus ; partially damaged the superior , middle and inferior turbinate ; and obstructed the entire right nasal cavity .
the lateral , anterior , and inferior walls of the right maxillary sinus were focally destroyed and a focal tumor extension was exposed .
the mass extended superiorly to some part of the ethmoid sinus but did not involve the sphenoid sinus and frontal sinus ( fig .
an endoscopic biopsy revealed a carcinosarcoma with an osteosarcomatous element by hematoxylin - eosin staining and immunohistochemical stain ( fig .
2 ) . fluorodeoxyglucose positron emission tomography ( fdg pet)-ct was used to evaluate metastasis to adjacent organs .
fdg pet - ct revealed the presence of large mass of soft tissues with increased fdg uptake accompanied by bony erosion in the right maxillary sinus . also , nodular lung lesions with increased fdg uptake in lateral basal and posterior basal segment of right lower lung were observed .
hence , the clinical tnm staging of this patient was t3n0m0 ( stage iii ) .
a surgical resection with postoperative chemoradiation therapy was planned and the patient underwent a total maxillectomy on the right side .
examination in the operating room revealed a large tumor filling the nasal cavity , maxillary sinus , and ethmoid sinus .
the tumor had eroded through the inferior and medial orbital walls , however , periorbital invasion was not evident .
the patient 's postoperative course was uncomplicated , and he received postoperative radiation therapy with a total dose of 5,580 cgy .
however , during the radiation therapy , a mass lesion in the periorbital area was found in an endoscopic examination .
as a consequence , wide local excision was performed on the mass and the patient received a full course of irradiation , followed by chemotherapy ( cisplatin+5 fluorouracil+taxotere ) .
two months later , the patient developed a massive recurrence infiltrating the orbit , cheek , and the masseter muscle .
the patient decided not to receive further surgical treatment or chemotherapy , and was lost to follow - up .
carcinosarcoma is a biphasic tumor composed of dual components : an epithelial portion with squamous cell carcinoma and sarcomatoid lesion .
this tumor can arise in any squamous epithelium of the body , such as the respiratory tract , upper aerodigestive tract , and reproductive organs . in the head and neck region ,
this tumor is extremely rare in the sinonasal cavity , as indicated by the few case reports available ( table 1 ) [ 3,5 - 13 ] .
because of this rarity , tumor histogenesis , clinical course and resulting prognosis , and management are contentious issues .
concerning histogenesis , whether the cellular composition of carcinosarcoma is that of a true heterologeous tumor or whether it originates from a single malignant component having both epithelial and mesenchymal differentiation   is debatable .
recent electron microscopic and immunohistochemical investigations revealed epithelial differentiation in some of the spindle cells in the sarcomatous element , suggesting that the sarcomatous element is derived from mesenchymal transformation of the carcinoma cells . from the known cases ( table 1 ) , carcinosarcoma of the maxillary sinus seems to preferentially appear in older patients .
clinically , the symptoms are often nonspecific , but unilateral nasal obstruction and bleeding are initial symptoms . because of the high stage presentation at the time of diagnosis , this tumor presents significant therapeutic challenges and a fateful prognosis for the patient .
carcinosarcoma in the maxillary sinus was treated by surgery in eight of the 11 cases ( table 1 ) . except for one case ,
but , clinical effectiveness of chemotherapy is impossible to determine with statistical significance due to the small number of cases to date .
although an accurate estimation of survival outcome is not conclusive , carcinosarcoma at the maxillary sinus confers a poor survival rate and a high local recurrence rate . in the known cases , tumors in
the maxillary sinus recurred after management in seven of 11 cases ( table 1 ) .
local recurrences and metastases typically were lethal . an overall mortality rate of 42% at 30 months was reported in carcinosarcoma at other sites .
survival periods were from 2 - 40 months and local recurrences eventually occurred in most cases ( table 1 ) . when a carcinosarcoma tumor is located within the maxillary sinus , multimodal therapy ( i.e. , surgery , radiotherapy , and/or chemotherapy ) should be considered .
also , before aggressive multimodal therapy is initiated , patients should be counseled concerning the tendency for aggressive patient behavior , poor prognosis , and likelihood of recurrence .
 carcinosarcoma is a highly malignant tumor characterized by dual malignant histologic differentiation of epithelial and mesenchymal components . 
 the tumor is extremely rare in the sinonasal tract . 
 we report a case of a 62-year - old man with carcinosarcoma involving the maxillary sinus . 