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Primitive neuroectodermal tumor (PNET) in the differential diagnosis of malignant kidney tumors.
Pathol Res Pract. 2002; 198(8):563-9.PR

Abstract

Primitive neuroectodermal tumors (PNETs) of the kidney, a rare neoplastic disease of high malignancy with a tendency towards early metastasis, affect young adults (26-30 years) irrespective of the gender. Differential diagnosis from other renal tumors is very important for an effective therapy. Herein, we report on a 24-year-old male patient with a renal tumor consisting of small, round cells, and summarize the diagnostic procedures that establish the diagnosis of PNET. Light microscopy revealed not only areas containing small, round cells forming rosettes and pseudorosettes, but also areas containing spindle cells. Expression of CD 99 in combination with neural markers, such as NSE, was detected by immunohistochemistry, and further evidence of neural differentiation was provided by electron microscopy. Image cytometry revealed a peridiploid DNA-stemline. A reciprocal translocation of the chromosomes 11 and 22 [t(11;22)(q24;q12)] with expression of a EWS/FLI-1 fusion transcript was demonstrated by molecular pathology. Using these methods, the diagnosis of PNET was firmly established, and the tumor was treated by surgical resection and subsequent adjuvant chemotherapy. Eighteen months after therapy, the patient is in excellent health condition without any evidence of tumor recurrence.

Authors+Show Affiliations

Department of Pathology, Universitiy of Bonn, Germany. Nicolaus.Friedrichs@ukb.uni-bonne.deNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

12390000

Citation

Friedrichs, Nicolaus, et al. "Primitive Neuroectodermal Tumor (PNET) in the Differential Diagnosis of Malignant Kidney Tumors." Pathology, Research and Practice, vol. 198, no. 8, 2002, pp. 563-9.
Friedrichs N, Vorreuther R, Poremba C, et al. Primitive neuroectodermal tumor (PNET) in the differential diagnosis of malignant kidney tumors. Pathol Res Pract. 2002;198(8):563-9.
Friedrichs, N., Vorreuther, R., Poremba, C., Schafer, K. L., Böcking, A., Buettner, R., & Zhou, H. (2002). Primitive neuroectodermal tumor (PNET) in the differential diagnosis of malignant kidney tumors. Pathology, Research and Practice, 198(8), 563-9.
Friedrichs N, et al. Primitive Neuroectodermal Tumor (PNET) in the Differential Diagnosis of Malignant Kidney Tumors. Pathol Res Pract. 2002;198(8):563-9. PubMed PMID: 12390000.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Primitive neuroectodermal tumor (PNET) in the differential diagnosis of malignant kidney tumors. AU - Friedrichs,Nicolaus, AU - Vorreuther,Roland, AU - Poremba,Christopher, AU - Schafer,Karl-Ludwig, AU - Böcking,Alfred, AU - Buettner,Reinhard, AU - Zhou,Hui, PY - 2002/10/23/pubmed PY - 2003/5/8/medline PY - 2002/10/23/entrez SP - 563 EP - 9 JF - Pathology, research and practice JO - Pathol Res Pract VL - 198 IS - 8 N2 - Primitive neuroectodermal tumors (PNETs) of the kidney, a rare neoplastic disease of high malignancy with a tendency towards early metastasis, affect young adults (26-30 years) irrespective of the gender. Differential diagnosis from other renal tumors is very important for an effective therapy. Herein, we report on a 24-year-old male patient with a renal tumor consisting of small, round cells, and summarize the diagnostic procedures that establish the diagnosis of PNET. Light microscopy revealed not only areas containing small, round cells forming rosettes and pseudorosettes, but also areas containing spindle cells. Expression of CD 99 in combination with neural markers, such as NSE, was detected by immunohistochemistry, and further evidence of neural differentiation was provided by electron microscopy. Image cytometry revealed a peridiploid DNA-stemline. A reciprocal translocation of the chromosomes 11 and 22 [t(11;22)(q24;q12)] with expression of a EWS/FLI-1 fusion transcript was demonstrated by molecular pathology. Using these methods, the diagnosis of PNET was firmly established, and the tumor was treated by surgical resection and subsequent adjuvant chemotherapy. Eighteen months after therapy, the patient is in excellent health condition without any evidence of tumor recurrence. SN - 0344-0338 UR - https://www.unboundmedicine.com/medline/citation/12390000/Primitive_neuroectodermal_tumor__PNET__in_the_differential_diagnosis_of_malignant_kidney_tumors_ L2 - https://medlineplus.gov/kidneycancer.html DB - PRIME DP - Unbound Medicine ER -