4 yrs old girl underwent left nephrectomy due to 1 month pretreated Wilms tumor (nephroblastoma).  
MACRO: 1 largest multicystic myxoid gelatinous nodule in lower pole 4,5x4,5 cm with flesh dark red nidus. 1.5cm yelow nodules: 1 closed to main, another in upper pole (photos).  
HISTO:  
MAIN TUMOR: 3-component nephroblastoma with therapic effect (necrosis and xanthogranulomatous inflammation). Outside capsule of the tumor- confluent proliferating or dormant/fibrotic blastema rest. In the center of blastemal/epithelial component- focal accumulation of large lipidised "adrenal adenoma like" cells.  
SATELITES (2): Identical blastema rests with necrosis.  
IH (adrenal rest tumor only): Vimentin(+/++) 70%, PANCK(+/++) 70%, MelanA(+++)100%, CD10(-), EMA(-), RCC(-), CD117(-), HMB45(-), p504s(-), CD68(-), Calretinin(+) 5%, ChromograninA(-), WT1(-), CD57(-), NSE(+/+++) 30%, Inhibin A(++/+++) 100%, Synaptophysin (+/+++) 100%, p53(+) 30%, Ki67<2% (++).  
 
PROPOSAL: Nephroblastoma with posttherapy changes and perifocal and 2 separate nidus of blastema (nephroblastomatosis?). Adrenal rest tumor within nephroblastoma.  
Thankyouforalldone.  
 
 

kunze 2011-02-07 12:51	I support your concept of a nephroblastoma (regressive type after preoperative chemotherapy) with involved adenomatous adrenocortical tissue and separately localized nephrogenic rests (nephroblastomatosis).
ugnius 2011-02-07 14:01	Thank you a lot.
tzankov 2011-02-11 06:55	Dear Ugnius,   I consulted our paidopathologist ELizabeth Bruder, she agrees with the diagnosis of Nephroblastoma, but considers the large cell, inhibin+ component as a specially differentiated tumor part and not as adrenal rest.   Best wishes   Alex
ugnius 2011-02-16 14:16	Thanx, Alex. Unfortunatelly I have not met any proof of such epithelial differentiation in literature. My opposite idea was translocation asociated RCC, but full immunophenotype of this unusual component is typical for adrenal tissue, organised in disordered manner with some cytological atypia. It were the main argues for adrenal rest tumor, not so rare in infancy.
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