title: Primitive neuroectodermal tumor of the kidney--another enigma: a pathologic, immunohistochemical, and molecular diagnostic study.
abstract: Primitive neuroectodermal tumor (PNET), the second most common type of sarcoma in the first two decades of life, rarely presents as an organ-based neoplasm. Rather, it is seen typically in the soft tissues of the chest wall and paraspinal region. We report a case of primary PNET of the kidney in a 17-year-old girl who presented with abdominal pain, hematuria, and an abdominal mass. Nodules and sheets of monotonous-appearing primitive round cells and the formation of rosettes focally were the principal microscopic features. The tumor cells were uniformly immunoreactive for vimentin, cytokeratin, neuron-specific enolase, and 013 (CD99). In addition, the characteristic translocation of PNET and Ewing sarcoma, t(11;22)(q24;q12), was detected by polymerase chain reaction (PCR). Eight previous examples of renal PNET have been reported in the literature in the past 2 years, but only three of these cases have had complete immunohistochemical evaluation with the demonstration of 013 positivity. To our knowledge the present case is the only one to date demonstrating the recurrent translocation t(11;22)(q24;q12) by PCR. Assuming that the previous cases in the literature are bona fide examples of PNET, the kidney may be another site of predilection for this usual soft-tissue neoplasm. We are once again confronted with the dilemma about the nature of the progenitor cell.
authors.affiliation: Department of Pathology, Washington University School of Medicine, St. Louis, Missouri 63110, U.S.A.
authors.forename: E F
authors.initials: EF
authors.lastname: Marley
authors.forename: H
authors.initials: H
authors.lastname: Liapis
authors.forename: P A
authors.initials: PA
authors.lastname: Humphrey
authors.forename: R B
authors.initials: RB
authors.lastname: Nadler
authors.forename: C L
authors.initials: CL
authors.lastname: Siegel
authors.forename: X
authors.initials: X
authors.lastname: Zhu
authors.forename: J M
authors.initials: JM
authors.lastname: Brandt
authors.forename: L P
authors.initials: LP
authors.lastname: Dehner
mesh_terms: D000071443:12E7 Antigen; D000293:Adolescent; D015703:Antigens, CD / Q000032:analysis; D015815:Cell Adhesion Molecules / Q000032:analysis; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007633:Keratins / Q000032:analysis; D007680:Kidney Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D018242:Neuroectodermal Tumors, Primitive / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D016133:Polymerase Chain Reaction; D012512:Sarcoma, Ewing / Q000235:genetics / Q000628:therapy; D014057:Tomography, X-Ray Computed; D014178:Translocation, Genetic / Q000235:genetics; D014746:Vimentin / Q000032:analysis
chemical_list: D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; D014746:Vimentin; D007633:Keratins; D010751:Phosphopyruvate Hydratase