title: Malignant peripheral primitive neuroectodermal tumor of the kidney.
abstract: Ewing family of tumors is a group of highly aggressive neoplasias that occur most commonly in the first two decades of life. These tumors are most frequently localized in bones, less frequently in soft tissues. They usually appear as undifferentiated small round-cell tumors. With current treatment regiments, 5-year disease-free survival rates exceed 60% in patients with a localized disease. Patients with metastatic disease at the time of their first presentation have a poor prognosis. We describe a rare case of visceral primitive neuroectodermal tumor with the involvement of the kidney in a 9-year-old girl. The tumor was studied with immunohistochemistry, cytogenetics, and molecular biology methods. Strong expression of protein MIC(2) by immunochemistry (antibody HBA 71) with subsequent demonstration of a translocation consistent with t(11;22)(q24;q12) using cytogenetic and reverse transcriptase polymerase chain reaction (RT-PCR) confirmed the histopathological diagnosis of peripheral primitive neuroectodermal tumor. We detected minimal residual disease in bone marrow using RT-PCR.
authors.affiliation: Department of Pediatric Oncology, Second Faculty of Medicine, Charles University, Prague, Czech Republic.
authors.forename: Ales
authors.initials: A
authors.lastname: Vicha
authors.forename: Eva
authors.initials: E
authors.lastname: Stejskalova
authors.forename: David
authors.initials: D
authors.lastname: Sumerauer
authors.forename: Roman
authors.initials: R
authors.lastname: Kodet
authors.forename: Josef
authors.initials: J
authors.lastname: Malis
authors.forename: Hana
authors.initials: H
authors.lastname: Kucerova
authors.forename: Jiri
authors.initials: J
authors.lastname: Bedrnicek
authors.forename: Josef
authors.initials: J
authors.lastname: Koutecky
authors.forename: Tomas
authors.initials: T
authors.lastname: Eckschlager
mesh_terms: D001854:Bone Marrow Cells / Q000473:pathology; D002648:Child; D002871:Chromosome Banding; D005260:Female; D006801:Humans; D007621:Karyotyping; D007680:Kidney Neoplasms / Q000235:genetics* / Q000473:pathology; D018242:Neuroectodermal Tumors, Primitive / Q000235:genetics* / Q000473:pathology; D012333:RNA, Messenger / Q000235:genetics; D011993:Recombinant Fusion Proteins / Q000235:genetics; D020133:Reverse Transcriptase Polymerase Chain Reaction
chemical_list: D012333:RNA, Messenger; D011993:Recombinant Fusion Proteins