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Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies.
Urology. 2006 Aug; 68(2):257-62.U

Abstract

OBJECTIVES

Peripheral primitive neuroectodermal tumor (PNET) is a highly aggressive neoplasm belonging to the Ewing family of tumors. It is characterized by the expression of MIC2 and neural markers (eg, neuron-specific enolase, synaptophysin, S-100, vimentin, Leu-7), and the presence of the EWS-FLI1 translocation.

METHODS

We performed a MEDLINE search for PNET in urologic malignancies. Additionally, we report on 2 cases of renal and 1 case of bladder PNET. The data obtained by reviewing patients with renal PNET were analyzed using Kaplan-Meier analysis.

RESULTS

Renal PNET is diagnosed in young adults (median age 24 years). In contrast, the incidence of bladder PNET seems to be dependent on a defective immune mechanism. Patients often present with pain (84%), palpable tumor (60%), and hematuria (38%). The radiologic findings are uncharacteristic; therefore, the diagnosis should be based on the histologic and immunohistochemistry findings. Renal and bladder PNET are both often diagnosed at an advanced stage and, therefore, the prognosis is poor, despite aggressive multimodal treatment (surgery, polychemotherapy, radiotherapy). We identified palpable tumor masses (log-rank test, P = 0.0027) and synaptophysin expression (log-rank test, P = 0.0422) as prognostic unfavorable markers for renal PNET.

CONCLUSIONS

Renal PNET should be considered in young patients who present with the classic triad of renal cancer, hematuria, and pain and palpable tumor. Once PNET is diagnosed, multimodal treatment (radical surgery, multidrug chemotherapy, radiotherapy) must be initiated. Despite this, the prognosis is poor if distant metastases are present. Furthermore, palpable tumor masses and synaptophysin expression are associated with a shorter cancer-specific survival.

Authors+Show Affiliations

Klinik und Poliklinik für Urologie, Universitätsklinikum Bonn, Rheinische Friedrich-Wilhelms-Universität Bonn, Bonn, Germany.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

16904430

Citation

Ellinger, Jörg, et al. "Primitive Neuroectodermal Tumor: Rare, Highly Aggressive Differential Diagnosis in Urologic Malignancies." Urology, vol. 68, no. 2, 2006, pp. 257-62.
Ellinger J, Bastian PJ, Hauser S, et al. Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies. Urology. 2006;68(2):257-62.
Ellinger, J., Bastian, P. J., Hauser, S., Biermann, K., & Müller, S. C. (2006). Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies. Urology, 68(2), 257-62.
Ellinger J, et al. Primitive Neuroectodermal Tumor: Rare, Highly Aggressive Differential Diagnosis in Urologic Malignancies. Urology. 2006;68(2):257-62. PubMed PMID: 16904430.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies. AU - Ellinger,Jörg, AU - Bastian,Patrick J, AU - Hauser,Stefan, AU - Biermann,Katharina, AU - Müller,Stefan C, PY - 2005/10/30/received PY - 2006/01/06/revised PY - 2006/02/21/accepted PY - 2006/8/15/pubmed PY - 2006/8/30/medline PY - 2006/8/15/entrez SP - 257 EP - 62 JF - Urology JO - Urology VL - 68 IS - 2 N2 - OBJECTIVES: Peripheral primitive neuroectodermal tumor (PNET) is a highly aggressive neoplasm belonging to the Ewing family of tumors. It is characterized by the expression of MIC2 and neural markers (eg, neuron-specific enolase, synaptophysin, S-100, vimentin, Leu-7), and the presence of the EWS-FLI1 translocation. METHODS: We performed a MEDLINE search for PNET in urologic malignancies. Additionally, we report on 2 cases of renal and 1 case of bladder PNET. The data obtained by reviewing patients with renal PNET were analyzed using Kaplan-Meier analysis. RESULTS: Renal PNET is diagnosed in young adults (median age 24 years). In contrast, the incidence of bladder PNET seems to be dependent on a defective immune mechanism. Patients often present with pain (84%), palpable tumor (60%), and hematuria (38%). The radiologic findings are uncharacteristic; therefore, the diagnosis should be based on the histologic and immunohistochemistry findings. Renal and bladder PNET are both often diagnosed at an advanced stage and, therefore, the prognosis is poor, despite aggressive multimodal treatment (surgery, polychemotherapy, radiotherapy). We identified palpable tumor masses (log-rank test, P = 0.0027) and synaptophysin expression (log-rank test, P = 0.0422) as prognostic unfavorable markers for renal PNET. CONCLUSIONS: Renal PNET should be considered in young patients who present with the classic triad of renal cancer, hematuria, and pain and palpable tumor. Once PNET is diagnosed, multimodal treatment (radical surgery, multidrug chemotherapy, radiotherapy) must be initiated. Despite this, the prognosis is poor if distant metastases are present. Furthermore, palpable tumor masses and synaptophysin expression are associated with a shorter cancer-specific survival. SN - 1527-9995 UR - https://www.unboundmedicine.com/medline/citation/16904430/Primitive_neuroectodermal_tumor:_rare_highly_aggressive_differential_diagnosis_in_urologic_malignancies_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0090-4295(06)00272-X DB - PRIME DP - Unbound Medicine ER -