natural killer ( nk)/t - cell lymphoma is a distinct clinicopathologic entity highly associated with epstein - barr virus ( ebv ) . the disease has protean clinical features characterized by a destruction of the upper respiratory tract , particularly the nasal cavity , palate and paranasal sinuses . pathologically , angiocentric and angiodestructive patterns , marked necrosis and infiltration of atypical lymphoid cells are found in most of the lesions . nk / t - cell lymphoma also has a characteristic immunophenotype : cd2-positive , cd56-positive , but usually surface cd3-negative . nk / t - cell lymphoma is closely linked to a variety of medical complications , such as hemophagocytic syndrome , sepsis and bleeding . rarely , patients can develop a second primary cancer during the follow - up period and this can be a cause of death . we experienced a case of nk / t - cell lymphoma complicated by a squamous cell carcinoma of the hard palate during combination chemotherapy and radiation therapy and report the case with a review of the literature . a 50-year - old male patient was admitted to the hospital with complaints of sore throat , fever and bloody sputum for three months . the patient had been suffering from a recurrent sore throat for one year without seeking medical attention . on admission , he had a fever up to 39c mainly at night , but he denied weight loss or night sweating . diffuse erythematous lesions and wall thickness in the oropharyngeal area were seen on otolaryngeal examination . laboratory findings were as follows : wbc 7,500/mm , hb 12.5 g / dl , hct 37% , platelet 250,000/mm , normal urine analysis , alt / ast 25/40 u / l , serum alkaline phosphatase 95 iu / l and ldh 288 u / l . chest x - ray finding was normal . a computed tomographic ( ct ) scan of the neck showed a soft tissue mass in both oropharynx , soft palate and posterior laryngeal wall , and multiple lymph node enlargements of both cervical areas ( figure 1 ) . for a pathologic confirmation , multiple biopsies were performed on the oropharyneal mass and other surrounding areas . microscopic examination showed an infiltration of atypical small irregularly shaped lymphoid cells admixed with plasma cells , neutrophils and eosinophils . on immunophenotyping study , cd56 and cytoplasmic cd3 were positive and cd20 was negative ( figure 2 ) . according to the microscopic examination and immunophenotype , a diagnosis of nk / t - cell lymphoma was made . for a staging work - up , bilateral bone marrow aspiration and biopsy , gastroscopic examination , bone scan and abdomino - pelvic ct were performed . the patient was treated initially with combination chemotherapy with copblam - v ( cyclophosphamide , vincristine , adriamycin , bleomycin , prednisolone and procarbazine ) . after 2 cycles of chemotherapy , a follow - up ct scan showed a partial improvement of the mass lesions in the oropharynx . however , after further two cycles of chemotherapy , he developed high fever , severe sore throat and neutropenia . since an aggravation of the mass lesion was noted on a follow - up ct scan , salvage radiation therapy was given to the patient . unfortunately , the radiation therapy discontinued due to a sustained neutropenia during the treatment period . the patient was referred to the hanyang university kuri hospital for further evaluation and treatment . on admission the ulcerative lesion did not heal and rather , increased in size during the hospital stay . since the new lesion was separated from the initial lymphoma site , we performed a biopsy on the ulcerating lesion of the hard palate . unexpectedly , the biopsy revealed a moderately differentiated squamous cell carcinoma with a p53 immunoreactivity ( figure 3 ) . however , the patient could not tolerate the radiation therapy due to a poor general condition . although a salvage combination chemotherapy was administered to the patient , a severe hepatic dysfunction and sustained pancytopenia developed without an improvement of the lymphoma or carcinoma lesion and he eventually died of sepsis . the nasal lymphoma expressing a t- or nk - cell phenotype is a disorder with protean clinical features characterized by a destruction of the upper respiratory tract , particularly the nasal cavity , palate and paranasal sinuses . pathologically , most of the lesions show angiocentric and angiodestructive patterns , marked necrosis and infiltration of atypical lymphoid cells and are highly associated with ebv . the nasal nk / t - cell lymphoma has a characteristic immunophenotype : cd2-positive , cd56-positive , but usually surface cd3-negative . epidemiologically , nk / t - cell lymphoma is rare in the united states and europe , but relatively common in asia . the most important aspect of the nasal nk / t cell lymphoma , as in our case , is that the disease is frequently associated with a variety of medical complications or immunologic disorders . it has been suggested that the angiocentric lymphomas occasionally retain an immunologic dysfunction , which may be reflected in some clinical manifestations observed in patients with t - lymphocyte malignancies . ishii et al and sordillo et al found a profound immunologic deficit in a few patients with angiocentric lymphomas , probably resulting from the impairment of the t - lymphocyte function . kim et al and ng et al reported that a hemophagocytic syndrome can occur at any time during the clinical course of the disease and is usually rapidly fatal over several weeks . also , there is a tendency toward an extranodal involvement of sites such as lungs , liver or gastrointestinal tract . more widespread involvement of unusual sites , such as testes or skin , has been occasionally observed . some patients develop second primary cancers during the follow - up period . the most striking clinical outcome in such patients is a rapid progression of the second primary cancer after diagnosis . despite an aggressive treatment for the second primary cancer , most of the patients die from a rapid deterioration of the cancer within 1 year , which was the case in our patient . interestingly , our patient developed a squamous cell carcinoma of the hard palate during combination chemotherapy for the nk / t cell lymphoma , with a very short latent period of the development of the second primary cancer . although it is plausible that the squamous cell carcinoma of the hard palate coexisted initially with the nk / t cell lymphoma , the fact that multiple blind biopsies were performed on the oral cavity , including soft palate , hard palate , oropharynx and pharyngeal wall , at the initial diagnosis argues against this possibility . furthermore , the hard palate was initially shown to be normal on physical examination , pathological examination , and ct scan . only limited information is now available regarding the relationship between angiocentric lymphoma and the development of second primary cancers . in an earlier study of 10 patients with midline lethal granuloma by fauci et al . three patients developed second primary cancers and the authors speculated such patients with aberrant immunologic reactions would have an increased propensity for the development of new cancers . until recently , there has been no clear consensus on the optimal management for the nk / t cell lymphoma , probably because of the rarity of the tumor and little understanding of its natural history . despite the inconsistent responses elicited , radiation treatment is still accepted as the treatment of choice for the localized form of angiocentric lymphoma , and achievement of a complete response after radiation treatment is still the most important determinant of favorable survival . firstly , the patient initially showed multiple systemic failures , which can often be observed in nk / t - cell lymphoma . although we did not perform a liver biopsy in this patient , unexplained severe hepatic dysfunction with hyperbilirubinemia and prolonged pancytopenia , even after the withdrawal of chemotherapy , suggested that the patient would have a hemophagocytic syndrome and liver involvement by the lymphoma . secondly , the patient developed a second primary cancer during the combination chemotherapy and radiation therapy . since the full dose of chemotherapy or radiation therapy was not administered to the patient and the latent period of the development of the second primary malignancy was very short , the exact mechanism should be elucidated . finally , although radiation therapy is regarded as the standard treatment for the patients with nk / t cell lymphoma , a large proportion of these patients are frequently associated with a systemic involvement by lymphoma and , therefore , need a multimodality treatment , including more effective chemotherapeutic agents . also , the development of new drugs specifically targeted against the nk / t lymphoma cells is needed . in summary , we experienced a case of nk / t - cell lymphoma complicated by a squamous cell carcinoma of the hard palate during combination chemotherapy and radiation therapy .
nk / t - cell lymphoma , which often shows an angiocentric growth pattern , is a distinct clinicopathologic entity highly associated with epstein - barr virus . 
 the disease is characterized by a destruction of the upper respiratory tract , particularly the nasal cavity , palate and paranasal sinuses . 
 interestingly , nk / t - cell lymphoma is closely linked to a variety of complications , such as hemophagocytic syndrome , second primary cancer , sepsis and bleeding . here 
 we report a case of a 50-year - old man diagnosed initially as nk / t - cell lymphoma of the oropharynx and who developed a second primary carcinoma of the hard palate during combination chemotherapy and radiation therapy .