patients of sarcomatoid carcinomas of adrenal can present with non - functional adrenal swellings with pressure symptoms by themselves or the result of their metastatic damage to other organs . diagnostic modalities include endocrine as well as radiological evaluation of the patient , but the definitive diagnosis is by histopathological and immunohistochemical examination postoperatively after excision . we present a case of sarcomatoid carcinoma of right adrenal gland for which open adrenalectomy was done . however , she succumbed after 4 months as she had developed similar swelling in left adrenal gland along with metastases to para aortic nodes . a 62-year old female presented with complaints of on and off episodes of pain in the right upper quadrant of abdomen since 4 months . computed tomography ( ct ) scan of abdomen and pelvis suggested 4.32.5 cm well defined moderately enhancing right adrenal mass with central necrosis ( figure 1 ) . histopathology suggested it to be tumor composed of spindle cells and permeated by intense inflammatory infiltrate chiefly neutrophils . the tumor cells showed strong immunopositivity for vimentin and focal immunopositivity for epithelial membrane antigen . ct scan of pelvis and thorax was done to search for the primary focus of malignancy but both suggested no obvious focus except patient having a 60% thrombosis of distal arch of aorta . the surgery was completed uneventfully . on gross examination , specimen was brown and nodular measuring 6.55.53.5 cm and weighed 55 g. its cut surface showed an unencapsulated firm greyish white tumor ( figure 2 ) . on microscopy the tumor showed pleomorphic tumor cells with epithelial and spindle cell morphology with evidence of foci of necrosis and intense inflammatory infiltrate with neutrophils and lymphocytes ( figure 3 ) . focal immunopositivity for pancytokeratin ( figure 5 ) and bcl-2 . the tumor cells were immunonegative for synaptophysin , chromogranin , melan - a and inhibin and described as sarcomatoid carcinoma of right adrenal gland . follow up after 3 months showed recurrence with involvement of left adrenal and para - aortic nodes on ct scan . it is broadly classified into : i ) conventional adrenocortical carcinomas , and ii ) adrenocortical carcinoma variants . variants of adrenocortical carcinomas include : i ) oncocytic adrenocortical neoplasms ; ii ) myxoid adrenocortical neoplasms ; iii ) adrenocortical carcinomas with a sarcomatous or sarcoma - like component ; iv ) adrenocortical blastoma . the tumor has been reported in relatively common organs such as the respiratory tract , gastrointestinal tract , and mammary gland . sarcomatoid carcinomas of the adrenal glands are extremely rare and extremely malignant tumors known with 1 year mortality of 100% . patients usually present with complaints of vague abdominal pain , abdominal lump in the flanks , loss of weight and generalized weakness . if the tumor is functional patients may present with the corresponding endocrine abnormalities like hypercortisolism causing cushing s syndrome , hyperaldosteronism causing severe hypertension , muscle cramps , testosterone secretion in women causing deepening of voice , acne , balding , hyperestrogenemia in males causing gynaecomastia , impotence and in females causing irregular menses , menorrhagia . in the review of literature , six out of 9 patients diagnosed with sarcomatoid carcinoma of adrenal , presented with flank or abdominal pain or discomfort as in present study . tumours tend to be very large at the time of presentation ( mean size 13 cm , weight 1113 g ) ( table 1 ) . these tumors are notoriously known for their metastasis at the time of presentation and the routes of metastasis include hematogenous , lymphatic or transcoelomic spread . the tumor cells on microscopy show dual line of differentiation into mesenchymal ( sarcomatous ) and epithelial ( carcinomatous ) elements . the mesenchymal differentiation may be towards muscle ( rhabdomyosarcoma ) , bone ( osteosarcoma ) , fibrosarcoma etc . or just undifferentiated spindle cell type . there are many theories proposed to explain the dual line of differentiation of these tumors . they are : i ) the composition tumor theory ( paradoxically requiring a non - malignant non - epithelial component as a reactive proliferative response induced by the epithelial component via paracrine secretion ) ; ii ) the collision or biclonal tumor theory ( a collision between two synchronous , histogenetically independent , biclonal tumors ) ; iii ) the conversion tumor theory ( neoplastic transformation within a monoclonal tumor recapitulating the naturally occurring event of conversion of epithelial to mesenchymal cells during embryogenesis ) ; and iv ) the combination or divergent tumor theory ( deriving from a common monoclonal stem cell precursor ) , molecular genetic evidence of monoclonality supports the single pluripotential stem - cell - divergence hypothesis and the epithelial - to - mesenchymal transition as well . adrenal cortical carcinosarcomas tend to be aggressive tumours , with locoregional recurrence and rapid metastases from sarcomatoid component . endocrine assays and ct are the modalities available to diagnose as well as to rule out primary focus from elsewhere . dynamic enhanced scan with multi - detector row ct may show the compositions and the blood supply of a tumor . ct scan shows heterogenous enhancement which may raise the suspicion of sarcoma , however further studies are required to confirm . the best available treatment option is surgical excision of the tumor with clearance of metastasis . in radical resection adrenal sarcomatoid carcinoma seems to be a highly malignant tumour and has very poor prognosis . adrenal sarcomatoid carcinomas even though are operable on early presentation , may present with metastases quite early and be fatal .
adrenal sarcomatoid carcinomas are extremely rare tumors presenting with extensive locoregional spread at the time of diagnosis . 
 patients succumb to metastases within a couple of months . as a result , 
 very few cases are reported in the literature until now . 
 we present a case of a 62-year old female with non - functional sarcomatoid carcinoma of the right adrenal gland . 
 there was no radiological evidence of locoregional metastases . 
 patient underwent right adrenalectomy . 
 follow up after 3 months showed para - aortic lymphadenopathy and similar left adrenal mass on computed tomography . 
 patient refused further treatment and succumbed to the disease . a brief case report with review of literature 
 is presented .