natural killer / t - cell ( nk / t - cell ) lymphoma is a rare fast - growing lymphoma arising from the lining of the nose or upper airway .
a 26-year - old young female presented with nasal obstruction , bloody discharge and swelling of the nasal skin and right lower eyelid . biopsy from the soft tissue seen in the nasal cavity revealed nk / t - cell lymphoma ( nasal type ) .
patient was referred to a whole body 18 fluoride ( 18f)-fluoro - deoxy glucose ( fdg ) positron emission tomography - computed tomography ( pet - ct ) .
18f - fdg pet - ct performed showed intense fdg uptake in heterogeneously enhancing soft tissue in the right nasal cavity with extension into the skin of the cheek and right lower eyelid [ figure 1 : a - maximum - intensity projection , b - axial ct , c - fused pet - ct ] .
intense fdg uptake was also noted in enhancing soft tissue bilateral adrenal masses [ d - axial ct ; e - fused pet - ct ] indicating disease involvement . a diagnosis of bilateral adrenal involvement by nk / t - cell lymphoma was made .
18 fluoride - fluoro - deoxy glucose positron emission tomography - computed tomography ( 18f - fdg pet - ct ) performed showed intense fdg uptake in heterogeneously enhancing soft tissue in the right nasal cavity with extension into the skin of the cheek and right lower eye lid ( a - maximum - intensity projection , b - axial ct , c - fused pet - ct ) .
intense fdg uptake was also noted in enhancing soft tissue bilateral adrenal masses ( d - axial ct ; e - fused pet - ct ) indicating disease involvement
morphologically , mature nk cells are large granular lymphoid cells , they are negative for surface cd3 but characteristically express cytoplasmic cd3 epsilon (  ) , cd56 , and cytotoxic molecules .
there are two main categories of nk cell - derived neoplasms , namely , aggressive nk cell leukemia and extranodal nk / t - cell lymphoma , nasal type .
nk / t - cell lymphoma of the nasal type accounts for 710% of all non - hodgkin lymphomas in asia and latin america , but only 1% of that amongst caucasians .
the leukemic counterpart of nk / t - cell lymphoma , aggressive nk - cell leukemia is an uncommon disease .
clinically , it is useful to classify nk / t - cell malignancies into two categories , namely , nasal nk - cell lymphomas , and nonnasal or extranasal nk / t - cell lymphomas , depending on the site of the lesions .
nasal nk / t - cell lymphomas occur in the nose and the upper aerodigestive tract .
bone marrow involvement occurs in < 10% of patients , and distant metastasis is unusual .
extranasal or nonnasal nk / t - cell lymphomas occur outside the typical nasal region , and the common primary sites involved include the skin , gastrointestinal tract , salivary glands , spleen , and testis .
unusual sites of involvement such as the muscle , the adrenal gland , and the female genital tract ( ovaries and uterus ) have been reported .
occult nasal involvement should be ruled out with panendoscopy and multiple random biopsies in patients presenting with extranasal nk / t - cell lymphoma .
disseminated nodal nhl can involve the adrenals in up to 25% of the cases . however , primary adrenal lymphoma is extremely rare with majority of the cases being b cell type .
natural killer - cell lymphomas are fdg - avid and pet - ct offers more accurate definition of the extent of involvement by distinguishing lymphoma involvement from inflammatory masses .
18-fdg pet - ct is increasingly used both at diagnosis and for monitoring of response to therapy .
pet changes the staging in up to 21% of the patients and alters the planned management strategy in 44% of the patients .
pet parameters such as total lesion glycolysis , in addition to the international prognostic index , is useful in predicting prognosis of these patients . for the stage ,
i / ii nasal nk / t - cell lymphomas , radiotherapy is an important modality of treatment .
recent evidence indicates radiation therapy alone is insufficient , and chemotherapy is also needed in these patients .
nonnasal nk cell lymphomas tend to have advanced or disseminated disease at presentation , and the role of radiotherapy is often limited . for the stage iii / iv nasal nk / t - cell lymphomas , extranasal nk / t - cell lymphomas and aggressive nk - cell lymphoma / leukemia , chemotherapy is the primary treatment .
l - asparaginase - containing regimens have shown promise as the tumor cells lack l - asparagine synthetase and are susceptible to l - asparaginase , which depletes l - asparagines in nk lymphoma cells .
 natural killer / t - cell ( nk / t - cell ) lymphoma is a rare condition , which presents as necrotic , granulomatous lesions involving the nose and the upper respiratory tract . 
 the condition usually has an aggressive clinical course . 
 the predominant subtype of nk / t - cell lymphoma noted in asian population is the nasal type . 
 we describe a case of biopsy - proven nk / t - cell lymphoma with bilateral adrenal involvement . adrenal involvement by lymphoma is usually of b - cell type and occurs in disseminated disease and often unilateral . bilateral adrenal involvement by t - cell lymphoma is extremely rare . 
INTRODUCTION
CASE REPORT
DISCUSSION