Pitfalls in the differential diagnosis of renal tumor in an adolescent.
The differential diagnosis of renal tumors, particularly in adolescents, may be challenging. We describe an 11-year-old female with a primary intra-renal mass. Initial differential diagnoses included primitive neuroectodermal tumor (PNET), desmoplastic small round cell tumor (DSRCT), and Wilms Tumor (WT). Extensive pathologic and molecular analysis on initial and relapsed tumor samples confirmed WT. The EWS-WT1 and EWS-FLI1 rearrangements, distinctive of DSRCT and PNET were negative. The differential diagnosis on monophasic blastemal WT may be complex. Primary renal DSRCT and PNET have been rarely described. Nevertheless, molecular confirmation for these rare conditions may be necessary in selected cases.
Division of Pediatric Oncology, Department of Pediatrics, University of São Paulo, São Paulo, Brazil. etvalera@hcrp.fmrp.usp.br
Elvis Terci
ET
Valera
María Sol
MS
Brassesco
Maria Angélica Abdalla
MA
de Freitas Cortez
Rosane Gomes
RG
Queiroz
Angel Mauricio
AM
Castro-Gamero
Marcus Vinicius de Castro
MV
Barros
Yvone Avalloni
YA
Vicente
Silvio Tucci
ST
Junior
Carlos Alberto
CA
Scrideli
Luiz Gonzaga
LG
Tone
D000293:Adolescent; D002648:Child; D020732:Cytogenetic Analysis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000473:pathology*; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000473:pathology; D012509:Sarcoma / Q000473:pathology; D009396:Wilms Tumor / Q000473:pathology*