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Is primitive neuroectodermal tumor of the kidney a distinct entity?
Cancer. 1997 Jun 01; 79(11):2243-50.C

Abstract

BACKGROUND

Primitive neuroectodermal tumors (PNETs) constitute a family of neoplasms of presumed neuroectodermal origin, most often presenting as bone or soft tissue masses in the trunk or axial skeleton in adolescents and young adults. As a soft tissue neoplasm, PNET arising in the kidney has not been well described, with only three cases previously reported.

METHODS

Four patients with PNET of the kidney were diagnosed and treated at St. Jude Children's Research Hospital. The authors reviewed the clinical, radiologic, and pathologic features and outcomes of these cases and of those previously described.

RESULTS

The authors' patients were age 4-20 years. They presented with unilateral renal masses and metastatic disease in the lymph nodes (three patients), lungs (three patients), bone (two patients), liver (one patient), and bone marrow (one patient). Treatment included surgery, radiotherapy, and multiagent chemotherapy. Three of the patients died of progressive disease within 14 months of diagnosis. Features and outcomes were similar to those of the three previously reported cases.

CONCLUSIONS

PNET of the kidney appears to be a distinct entity. Although rare, it must be included in the differential diagnosis of renal tumors in children and young adults. Patients usually present with advanced disease and show poor response to combined-modality therapy.

Authors+Show Affiliations

Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105-2794, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

9179073

Citation

Rodriguez-Galindo, C, et al. "Is Primitive Neuroectodermal Tumor of the Kidney a Distinct Entity?" Cancer, vol. 79, no. 11, 1997, pp. 2243-50.
Rodriguez-Galindo C, Marina NM, Fletcher BD, et al. Is primitive neuroectodermal tumor of the kidney a distinct entity? Cancer. 1997;79(11):2243-50.
Rodriguez-Galindo, C., Marina, N. M., Fletcher, B. D., Parham, D. M., Bodner, S. M., & Meyer, W. H. (1997). Is primitive neuroectodermal tumor of the kidney a distinct entity? Cancer, 79(11), 2243-50.
Rodriguez-Galindo C, et al. Is Primitive Neuroectodermal Tumor of the Kidney a Distinct Entity. Cancer. 1997 Jun 1;79(11):2243-50. PubMed PMID: 9179073.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Is primitive neuroectodermal tumor of the kidney a distinct entity? AU - Rodriguez-Galindo,C, AU - Marina,N M, AU - Fletcher,B D, AU - Parham,D M, AU - Bodner,S M, AU - Meyer,W H, PY - 1997/6/1/pubmed PY - 2000/6/20/medline PY - 1997/6/1/entrez SP - 2243 EP - 50 JF - Cancer JO - Cancer VL - 79 IS - 11 N2 - BACKGROUND: Primitive neuroectodermal tumors (PNETs) constitute a family of neoplasms of presumed neuroectodermal origin, most often presenting as bone or soft tissue masses in the trunk or axial skeleton in adolescents and young adults. As a soft tissue neoplasm, PNET arising in the kidney has not been well described, with only three cases previously reported. METHODS: Four patients with PNET of the kidney were diagnosed and treated at St. Jude Children's Research Hospital. The authors reviewed the clinical, radiologic, and pathologic features and outcomes of these cases and of those previously described. RESULTS: The authors' patients were age 4-20 years. They presented with unilateral renal masses and metastatic disease in the lymph nodes (three patients), lungs (three patients), bone (two patients), liver (one patient), and bone marrow (one patient). Treatment included surgery, radiotherapy, and multiagent chemotherapy. Three of the patients died of progressive disease within 14 months of diagnosis. Features and outcomes were similar to those of the three previously reported cases. CONCLUSIONS: PNET of the kidney appears to be a distinct entity. Although rare, it must be included in the differential diagnosis of renal tumors in children and young adults. Patients usually present with advanced disease and show poor response to combined-modality therapy. SN - 0008-543X UR - https://www.unboundmedicine.com/medline/citation/9179073/Is_primitive_neuroectodermal_tumor_of_the_kidney_a_distinct_entity L2 - https://medlineplus.gov/kidneycancer.html DB - PRIME DP - Unbound Medicine ER -