calcifying fibrous tumor ( cft ) is a very rare , usually benign soft tissue tumor characterized by a proliferation of bland spindled mesenchymal cells with abundant collagen , background lymphoplasmacytic inflammation and variable amounts of stromal calcification . herein , we report the case of a patient with cft arising within soft tissue densely adherent to the left adrenal gland . a 22-year - old man was evaluated and treated from age 10 for chronic epigastric pain . egd and other diagnostic studies failed to elucidate a cause . computed tomography ( ct ) he underwent ct imaging which revealed a solid 6.2    5.9    4.8  cm well - circumscribed retroperitoneal mass thought to be emanating from the lateral limb of the left adrenal gland ( fig . the mass was abutting the posterior pancreatic tail and antero - superior aspect of the left kidney . adrenal biochemical studies , including acth , cortisol , dehydroepiandrosterone sulfate , free testosterone , aldosterone , renin , plasma fractionated metanephrines , were within normal ranges . insulin , proinsulin , c - peptide and glucose levels were normal . 
 figure 1:(a ) ct scan of the abdomen showing a 6.2    5.9    4.8  cm retroperitoneal mass appearing to emanate from the lateral limb of the left adrenal gland . ( b ) photograph of the gross specimen revealing a firm , white - tan and well - circumscribed tumor . ( a ) ct scan of the abdomen showing a 6.2    5.9    4.8  cm retroperitoneal mass appearing to emanate from the lateral limb of the left adrenal gland . ( b ) photograph of the gross specimen revealing a firm , white - tan and well - circumscribed tumor . a portion of normal adrenal tissue is visible . given the concern for an adrenocortical or other malignancy ( pancreatic or soft tissue ) , an exploratory laparotomy via subcostal incision was performed . findings revealed a firm , circumscribed mass emanating from the posterior aspect of the left adrenal gland . the mass did not involve any adjacent structures and there was no evidence of lymphadenopathy . a left adrenalectomy with en bloc resection of periadrenal soft tissue was performed without violation of the capsule . histologically , the mass  comprised  a hypocellular proliferation of bland spindle cells , densely hyalinized collagen , chronic lymphoplasmacytic inflammation and dystrophic calcifications ( fig . while there was no infiltration into the adrenal gland , there was early entrapment of small vessels . most of the spindle cells that appeared morphologically fibroblastic were normochromatic , and had a low mitotic rate ( 01 per 10 high - powered fields ) . figure 2:(a , b ) cft demonstrating hypocellular proliferation of bland spindle cells , densely hyalinized collagen , chronic lymphoplasmacytic inflammation and dystrophic calcifications ( h&e ; x400 and x20 ) . ( c , d ) inflammatory component of cft , comprised  small lymphocytes and plasma cells , the latter are occasionally binucleate or have russell bodies ( h&e ; x200 and x400 ) . ( a , b ) cft demonstrating hypocellular proliferation of bland spindle cells , densely hyalinized collagen , chronic lymphoplasmacytic inflammation and dystrophic calcifications ( h&e ; x400 and x20 ) . ( c , d ) inflammatory component of cft , comprised  small lymphocytes and plasma cells , the latter are occasionally binucleate or have russell bodies ( h&e ; x200 and x400 ) . immunophenotypically , the spindle cells were negative for smooth muscle actin , desmin , alk , s100 , cd34 and cd117 . the plasma cells were polytypic without restricted kappa or lambda light chain expression , and the majority expressed immunoglobulin g ( igg ) with a subset positive for igg4 . in three  x400 plasma cell - rich fields (  hotspots ) , the average number of igg - positive cells was 183 and the average number of igg4-positive cells was 11 ( ratio  =  16 ) ( fig . figure 3:immunohistochemical stains for ( a ) kappa light chain expression ( x200 ) , ( b ) lambda light chain expression ( x200 ) , ( c ) igg ( x200 ) and ( d ) igg4 ( x200 ) . immunohistochemical stains for ( a ) kappa light chain expression ( x200 ) , ( b ) lambda light chain expression ( x200 ) , ( c ) igg ( x200 ) and ( d ) igg4 ( x200 ) . cft , initially described by rosenthal and abdul - karim   in 1988 (  childhood fibrous pseudotumor with psammoma bodies ) , was further characterized by fetsch et al .   in 1993 (  calcifying fibrous pseudotumor ) , and then reported as a series of 15 cases by nascimento et al .   in 2002 . cfts can arise in a variety of locations , but are more commonly described arising in or from the pleura , thoracic cavity , extremities , mediastinum and abdominal cavity [ 3 , 4 ] . to date , there have only been four previously reported cases of adrenal cft [ 58 ] . these lesions were previously called calcifying fibrous pseudotumor . however , due to a local recurrence rate of ~10% , these lesions were renamed cfts in the current world health organization classification . this case is similar to the one reported by lau and weiss , although in that particular case , the mass nearly replaced the entire adrenal gland , whereas in this current case , the adrenal gland was spared . closer scrutiny of these previous cases suggests that the tumors were perhaps adjacent to rather than arising from the adrenal gland as in this case [ 58 ] . the gross description , histology , immunophenotype and polytypic nature of the plasma cells are identical . radiologic characterization of these masses is not well described other than being solid tumors that are usually well circumscribed . magnetic resonance imaging findings described in a case report revealed signal iso - intense to skeletal muscle on t1 weighted images and low signal intensity on t2 weighted images . other types of benign fibrous tumors reveal intense peripheral enhancement after gadolinium injection ; however , the cft showed only mild peripheral enhancement . histologically , cfts are composed of a hypocellular proliferation of non - atypical spindle cells , dense collagen , lymphoplasmacytic inflammation and stromal calcifications . in the majority of cases , the spindle cells are negative for actins , s100 , desmin and alk . cd34 expression is more variable and can be focal or diffuse . similar to cft , inflammatory myofibroblastic tumor ( imt ) contains a proliferation of spindle cells accompanied by inflammation - containing plasma cells . spindle cells of imt are usually positive for muscle specific actin , sma and desmin , but staining can be focal . of note , 2540% of extrapulmonary imts recur , but metastasis is rare ( < 2% of cases ) . the histological similarities between cft and  imt have raised the question as to whether cfts are a sclerosing end stage of imts . both lesions consist of spindle cells , collagen deposition and lymphocytic infiltrates . despite similarities , , cft is an uncommon soft tissue lesion of uncertain etiology low on the list of differential diagnoses often occurring in younger patients in a variety of locations and usually associated with a clinically benign course .
establishing the etiology of a retroperitoneal tumor may be difficult due to close proximity of multiple organs . 
 evaluation of retroperitoneal tumors often leads to surgery , many times to obtain a definitive diagnosis and rule out malignancy . calcifying fibrous tumors ( cft ) are very rare soft tissue tumors occurring most often in young patients . 
 they are most often found arising in the thoracic cavity , mediastinum , abdominal cavity and extremities and usually have a benign clinical course . 
 macrocscopically , the tumors are well circumscribed and firm with a white - tan appearance 
 . histologically , cft comprised  a hypocellular proliferation of bland spindle cells , densely hyalinized collagen , chronic lymphoplasmacytic inflammation and dystrophic calcifications . 
 other considerations in the pathologic differential diagnosis include solitary fibrous tumor and inflammatory myofibroblastic tumor .