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Primary Ewing's Sarcoma/Primitive Neuroectodermal Tumor Of The Kidney: A Clinicopathologic And Immunohistochemical Analysis Of 11 Cases

R. E. Jiménez, A. Folpe, R. Lapham, J. Ro, P. O'shea, S. Weiss, M. Amin
Published 2002 · Medicine

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Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an extraordinarily rare primary tumor in the kidney and can be mistaken for a variety of other round cell tumors, including blastema-predominant Wilms' tumor (WT). Approximately 90% of ES/PNET have a specific t(11;22), which results in a chimeric EWS-FLI-1 protein. Immunohistochemistry for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET. WT-1, the WT-associated tumor suppressor gene, is overexpressed in WT but not in ES/PNET. No study has examined FLI-1 or WT-1 expression in renal ES/PNET. The clinicopathologic features of 11 renal ES/PNET were studied along with immunohistochemistry for cytokeratin, desmin, CD99, FLI-1, and WT-1. WT were also immunostained for CD99 (5 cases), FLI-1 (10 cases), and WT-1 (9 cases). The patients (6 men, 5 women) ranged from 18 to 49 years of age (mean, 34 yr). The mean tumor size was 11.8 ± 3.8 cm (mean ± standard deviation). Presenting symptoms included abdominal/flank pain and/or hematuria. Grossly, all tumors showed necrosis and hemorrhage, and 4 had cystic change. Microscopically, all tumors showed vaguely lobular growth, primitive round cells, and variable rosette formation. Epithelial, myogenous, or cartilaginous differentiation was not seen. Immunohistochemical results on the renal ES/PNET were cytokeratin (2/8 focal), desmin (0/9), CD99 (8/8), FLI-1 (5/8), and WT-1 (0/8). In comparison, the WT only rarely expressed CD99 (1/5) and did not express FLI-1 (0/10), but were usually WT-1-positive (7/9). Follow-up on 8 cases (mean, 28 mo; range, 6–64 mo) showed 4 lung and pleural metastases, 1 bone metastasis, liver metastasis, 2 local recurrences, and 5 deaths from disease (median time to death, 16.8 mo). No case had distant metastatic disease at presentation. Adjuvant therapy included chemotherapy (8 cases), radiation (3 cases), and bone marrow transplantation (1 case). Our study affirms a unique proclivity of renal ES/PNET for young adults and that it is a highly aggressive neoplasm, with rapid death in many cases, usually after the development of treatment-resistant lung metastases. These tumors must be distinguished from blastema-predominant WT and other primitive renal tumors that require different therapy. FLI-1 and WT-1 immunohistochemistry may be valuable in this differential diagnosis, given the known immunophenotypic overlap between ES/PNET and blastema-predominant WT with regard to CD99, cytokeratin, and desmin. The accurate distinction between these two entities has clear prognostic and therapeutic implications.
This paper references
Primitive neuroepithelial tumors of kidney.
B. Beckwith (1996)
Is primitive neuroectodermal tumor of the kidney a distinct entity? Cancer 1998;82:1414–6
M Benesch (1998)
10.1007/BF02889858
Distinction of nephroblastomas from other childhood tumors using antibodies to intermediate filaments
M. Altmannsberger (1984)
10.1002/MPO.2950230508
Malignant peripheral primitive neuroectodermal tumor (PNET) of the kidney.
Y. Mor (1994)
10.1097/00022744-199712000-00006
Antineuroblastoma antibody NB-84 also identifies a significant subset of other small blue round cell tumors
A. Folpe (1997)
10.1097/00000478-200003000-00010
Cytokeratin immunoreactivity in Ewing's sarcoma: prevalence in 50 cases confirmed by molecular diagnostic studies.
M. Gu (2000)
10.1097/00000478-200012000-00010
Immunohistochemical Detection of FLI-1 Protein Expression: A Study of 132 Round Cell Tumors With Emphasis on CD99-Positive Mimics of Ewing's Sarcoma/Primitive Neuroectodermal Tumor
A. Folpe (2000)
Cloning and characterization of the Ewing’s sarcoma and peripheral neuroepithelioma t(11;22) translocation breakpoints. Genes Chromosomes Cancer 1992;5:271–7
J Zucman (2002)
10.2106/00004623-200111000-00036
Enzinger and Weiss’s Soft Tissue Tumors. 4th ed.
E. Mindell (2001)
10.1016/0165-4608(88)90285-3
Chromosomes in Ewing's sarcoma. I. An evaluation of 85 cases of remarkable consistency of t(11;22)(q24;q12).
C. Turc-Carel (1988)
Precise developmental regulation of Ets family transcription factors during specification and commitment to the T cell lineage.
M. K. Anderson (1999)
10.1097/00000478-200012000-00011
Isochromosome 7q in Adult Wilms' Tumors: Diagnostic and Pathogenetic Implications
B. Rubin (2000)
Tumeur neuroectodermique primitive (PNET) rénale. A propos d'un cas
V. Vigouroux (1998)
Hématome spontané du rein révélant une tumeur neuroectodermique primitive chez l'adulte.
P. Wilshire (2000)
10.1002/MPO.1296
Primitive neuroectodermal kidney tumor.
A. Segura (2002)
10.1093/ANNONC/8.7.691
Long-term survival in an adult metastatic renal peripheral primitive neuroectodermal tumor (PPNET) with multimodality treatment including high-dose chemotherapy.
C. Fontaine (1997)
10.3122/15572625-13-6-455
Primitive Neuroectodermal Tumor Arising From the Kidney
J. Wells (2000)
10.3346/JKMS.1995.10.6.457
Peripheral neuroepithelioma of the kidney.
K. W. Kim (1995)
10.1080/13577149977839
Detection of EWS/FLI-1 by Immunostaining. An Adjunctive Tool in Diagnosis of Ewing's Sarcoma and Primitive Neuroectodermal Tumour on Cytological Samples and Paraffin-Embedded Archival Material
G. Nilsson (1999)
Primary primitive neuroR
T Mentzel (2002)
10.1097/00000478-200102000-00001
Primary Malignant Neuroepithelial Tumors of the Kidney: A Clinicopathologic Analysis of 146 Adult and Pediatric Cases from the National Wilms' Tumor Study Group Pathology Center
D. Parham (2001)
10.1002/(SICI)1097-0142(19980401)82:7<1414::AID-CNCR34>3.0.CO;2-7
Is primitive neuroectodermal tumor of the kidney a distinct entity?
M. Benesch (1998)
10.1387/IJDB.9694627
The avian fli gene is specifically expressed during embryogenesis in a subset of neural crest cells giving rise to mesenchyme.
A. M. Mager (1998)
10.1111/J.1464-410X.1994.TB07534.X
Intrarenal primitive neuroectodermal tumour.
Y. F. Chan (1994)
10.1002/GCC.2870050402
Cloning and characterization of the Ewing's sarcoma and peripheral neuroepithelioma t(11;22) translocation breakpoints
J. Zucman (1992)
Intrarenal primitive neuroectodermal tu
P Collini (1994)
10.1097/00000478-199111000-00003
Renal Neoplasms Mimicking Rhabdoid Tumor of Kidney: A Report from the National Wilms' Tumor Study Pathology Center
D. A. Weeks (1991)
10.1097/00000478-199703000-00013
Primitive neuroectodermal tumor of the kidney--another enigma: a pathologic, immunohistochemical, and molecular diagnostic study.
E. F. Marley (1997)
10.1016/S0022-5347(05)67301-6
Primitive neuroectodermal tumor (extraskeletal Ewing's sarcoma) of the kidney with vena caval tumor thrombus.
R. J. Karnes (2000)
10.1002/(SICI)1096-911X(199805)30:5<303::AID-MPO9>3.0.CO;2-J
Primitive neuroectodermal tumor (PNET)/extraosseous Ewing sarcoma of the kidney.
C. Antoneli (1998)
Renal primitive neuroec - todermal tumor : an immunohistochemical and cytogenetic analy
T Takeuchi (1996)
Primary primitive neuroectodermal tumor (PNET) of the kidney: a case report.
N. Ranadive (1999)
Primitive neuroectodermal kidney tumour.
M. S. Khan (1995)
10.1016/0165-4608(84)90002-5
Chromosome study of Ewing's sarcoma (ES) cell lines. Consistency of a reciprocal translocation t(11;22)(q24;q12).
C. Turc‐Carel (1984)
10.1002/(SICI)1097-0339(199603)14:2<101::AID-DC1>3.0.CO;2-M
Role of immunocytochemistry, electron microscopy, and DNA analysis in fine‐needle aspiration biopsy diagnosis of Wilms' tumor
D. A. Ellison (1996)
10.1097/00000478-199806000-00004
Poorly differentiated synovial sarcoma: immunohistochemical distinction from primitive neuroectodermal tumors and high-grade malignant peripheral nerve sheath tumors.
A. Folpe (1998)
10.1093/AJCP/114.3.345
WT1 staining reliably differentiates desmoplastic small round cell tumor from Ewing sarcoma/primitive neuroectodermal tumor. An immunohistochemical and molecular diagnostic study.
D. Hill (2000)
CD99 (p30/32MIC2) neuroectodermal/Ewing’s sarcoma antigen as an immunohistochemical marker. Review of more than 600 tumors and the literature experience
A Stevenson (1994)
Is primitive neuroectodermal tumor of the kidney a distinct entity? Cancer 1997;79:2243–50
C Rodriguez-Galindo (1997)
Precise
MK Anderson
Primitive neuroec - todermal tumor ( PNET ) / extraosseous Ewing sarcoma of the kid
EH Baldini (1998)
10.1097/00000478-199205000-00007
Desmin Positivity in Primitive Neuroectodermal Tumors of Childhood
D. Parham (1992)
10.1097/00000478-200102000-00022
Cytokeratin immunoreactivity in 41 cases of ES/PNET confirmed by molecular diagnostic studies.
P. Collini (2001)
10.1016/S0046-8177(97)90147-7
EWS/FLI-1 fusion transcripts in three peripheral primitive neuroectodermal tumors of the kidney.
M. Quezado (1997)
[Primitive neuroectodermal tumor of the kidney manifested as a spontaneous hematoma].
P. Wilshire (2000)
10.5980/JPNJUROL1989.90.639
[A case of primitive neuroectodermal tumor of the kidney].
J. Miyazaki (1999)
10.1093/AJCP/106.3.339
Primary primitive neuroectodermal tumor of the kidney. Case report and review of the literature.
J. Furman (1996)
10.1111/j.1440-1827.1996.tb03613.x
Renal primitive neuroectodermal tumor: An immunohistochemical and cytogenetic analysis
T. Takeuchi (1996)
10.1016/S0090-4295(99)00467-7
Images in clinical urology. Magnetic resonance imaging of primitive neuroectodermal tumor of the kidney.
C. Wu (2000)
10.1200/JCO.2000.18.1.204
Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family.
E. de Álava (2000)
Primitive renal neuroectodermal tumor (PNET)
V Vigouroux (1998)
10.1126/SCIENCE.1654597
Transcriptional repression mediated by the WT1 Wilms tumor gene product.
S. L. Madden (1991)
10.1007/S002920050035
[Primary primitive neuroectodermal tumor of the kidney in an adult. Clinico-pathologic and immunohistochemical case report].
T. Mentzel (1994)
10.1016/S0022-5347(01)67342-7
Primitive neuroectodermal kidney tumor: 2 case reports and review of the literature.
N. Gupta (1995)
10.1097/00000658-199907000-00012
Adults with Ewing's sarcoma/primitive neuroectodermal tumor: adverse effect of older age and primary extraosseous disease on outcome.
E. Baldini (1999)
Enzinger and Weiss's Soft Tissue Tumors
S. W. Weiss (2001)
[Primitive renal neuroectodermal tumor (PNET). Apropos of a case].
V. Vigouroux (1998)
Translocation chromosomique (11; 22) dans des lignées cellulaires de sarcomes d'Ewing
C. Turc‐Carel (1983)
Antibodies to desmin identify the blastemal component of nephroblastoma.
A. Folpe (1997)
10.1007/s002470050200
Peripheral primitive neuroectodermal tumor: report of a case arising in the kidney
T. Herman (1997)
[Primary, primitive (peripheral) neuroectodermal tumor (PNET) of the kidney].
V. Grouls (1994)
10.1097/00019606-199712000-00002
Renal Primitive Neuroectodermal Tumor: A Morphologic, Cytogenetic, and Molecular Analysis with the Establishment of Two Cultured Cell Lines
T. Takeuchi (1997)
10.1128/MCB.13.12.7393
The Ewing's sarcoma EWS/FLI-1 fusion gene encodes a more potent transcriptional activator and is a more powerful transforming gene than FLI-1.
W. May (1993)
10.1097/00000478-200006000-00008
Immunohistochemical expression of WT1 by desmoplastic small round cell tumor: a comparative study with other small round cell tumors.
R. Barnoud (2000)
10.1097/00000478-200009000-00007
Clear Cell Myomelanocytic Tumor of the Falciform Ligament/Ligamentum Teres: A Novel Member of the Perivascular Epithelioid Clear Cell Family of Tumors With a Predilection for Children and Young Adults
A. Folpe (2000)
10.1007/s002619910021
Malignant peripheral neuroectodermal tumor (MPNET) of the kidney
F. Maccioni (2011)



This paper is referenced by
10.1097/PAP.0000000000000017
Wilms Tumor: An Update
Turki O. Alhussain (2014)
10.1016/J.CDIP.2007.02.001
Small round-cell neoplasms of soft tissues: An integrated diagnostic approach
S. Rossi (2007)
10.1016/j.urology.2007.09.051
Primitive neuroectodermal tumor of the kidney: a single institute series of 16 patients.
Y. Thyavihally (2008)
10.1038/s41598-020-72680-6
Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor
Yidi Liu (2020)
10.1080/01913120801897141
Contribution of Cytogenetics to the Management of Poorly Differentiated Sarcomas
J. Bridge (2008)
10.1007/s11255-007-9202-1
Ewing’s sarcoma/primitive neuroectodermal tumor (ES/PNET) of the penis
I. Kılıçaslan (2007)
10.1016/S0242-6498(04)94016-3
Tumeurs du rein de l’enfant
E. Perlman (2004)
10.1053/J.SEMDP.2003.08.006
Ectopic neural and neuroendocrine neoplasms.
M. Wick (2003)
10.1159/000382118
A Case of Renal Primitive Neuroectodermal Tumor Confirmed by Fluorescence in situ Hybridization
Toshiki Etani (2015)
10.1002/pbc.20831
Ewing sarcoma/primitive neuroectodermal tumor of the kidney in a child
M. Maeda (2008)
10.1007/s00408-012-9405-9
Primary Pulmonary Primitive Neuroectodermal Tumor (PNET): A Clinicopathological and Immunohistochemical Study of Six Cases
A. Weissferdt (2012)
10.1097/PAS.0000000000001098
Clinicopathologic Features of a Series of Primary Renal CIC-rearranged Sarcomas With Comprehensive Molecular Analysis
S. Mangray (2018)
10.5301/uro.5000221
Primitive neuroectodermal tumour of kidney with thrombosis of the inferior vena cava and good responsive to surgical and medical treatment: description of a case and revision of literature
Giovanni Luca Giliberto (2018)
10.1155/2009/504654
Primary Adult Renal Ewing's Sarcoma: A Rare Entity
Ravindra Mukkunda (2009)
10.1016/J.TAAP.2007.08.008
Comparison of toxicogenomic profiles of two murine strains treated with HIV-1-based vectors for gene therapy.
Y. Zhao (2007)
Mechanisms and Consequences of Chromosomal Instability in Malignant tumours
Y. Stewénius (2008)
10.1016/S0027-9684(15)31142-1
Primitive neuroectodermal tumor of the kidney with inferior vena cava tumor thrombus.
L. Zhang (2009)
10.1053/J.SEMDP.2005.11.004
An immunohistochemical approach to the differential diagnosis of renal tumors.
B. Skinnider (2005)
10.1017/9781316535097
Soft Tissue Sarcomas: A Pattern-Based Approach to Diagnosis
A. P. D. Tos (2019)
10.1016/j.avsg.2014.12.007
A Primary Primitive Neuroectodermal Tumor Arising from Left Subclavian Vein and Extending along Left Brachiocephalic Vein and Superior Vena Cava into Right Atrium.
J. Wang (2015)
10.1016/J.AFJU.2014.08.004
Primary renal osteosarcoma: A case report
C. Ahomadegbe (2014)
10.3816/CGC.2009.n.032
Primary extraosseous ewing sarcoma of the kidney with level III inferior vena cava thrombus.
A. Fergany (2009)
10.1038/modpathol.2011.202
Mesotheliomas with small cell features: report of eight cases
N. Ordóñez (2012)
10.1155/2012/190581
Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: Two Unusual Presentations of a Rare Tumor
E. Castro (2012)
10.1016/j.humpath.2010.12.019
Primary renal carcinoid tumors: clinicopathologic features of 9 cases with emphasis on novel immunohistochemical findings.
Jennifer A. Jeung (2011)
10.1586/14737159.8.1.97
Ewing sarcoma family of tumors: a model for the new era of integrated laboratory diagnostics
J. Khoury (2008)
10.1007/s00247-008-0971-1
Primitive neuroectodermal tumour (PNET) of the kidney: a rare renal tumour in adolescents with seemingly characteristic radiological features
W. C. Chu (2008)
10.1007/s11255-006-6675-2
Primary primitive neuroectodermal tumor of the kidney: A case report
S. Erkılıç (2006)
10.1177/000313481908500912
Ewing's Sarcoma Family Tumor (EFT) Presents as Spontaneous Splenic Rupture
Mohamed Nagi (2019)
10.1016/J.CLL.2005.01.005
Tubulocystic carcinoma, mucinous tubular and spindle cell carcinoma, and other recently described rare renal tumors.
G. Maclennan (2005)
EWING SARCOMA OF ADRENAL GLAND CAUSING CUSHING’S SYNDROME; AN EXCEPTIONALLY RARE TUMOR
Zia ud Din Afridi (2018)
10.5146/tjpath.2015.01340
Primary Paediatric Renal Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature.
Binny Khandakar (2018)
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