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

David M. Parham, Gary J. Roloson, Michael Feely, Daniel M. Green, Julia A. Bridge, J. Bruce Beckwith

Research output: Contribution to journalArticle

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Abstract

Primary malignant neuroepithelial tumors of the kidney (NETKs) comprise a group of primitive, highly malignant neoplasms that histologically and clinically are not well characterized. A large cohort of 146 of these tumors, occurring in adults and children, has been collected at a single depository site, the National Wilms' Tumor Study Group (NWTSG) Pathology Center. The authors undertook a systematic retrospective review of the histologic, ultrastructural, and clinical features of these tumors, based on materials collected by the NWTSG and the consultation files of one of the authors (J.B.B.). Histologic features were generally those of primitive neural tumors with varying amounts of rosettes and neuropil; however, a large proportion of cases displayed unusual features such as spindle cells, ganglion cells, clear cell sarcoma-like foci, rhabdoid cells, epithelioid cells, and organoid foci. CD99 staining had been performed on 69 cases and showed membranous staining in 65. The NETKs were present in patients with a wide age spectrum, ranging from 1 month to 72 years (median, 18 years). EWS/FLI1 fusion analysis using reverse transcriptasepolymerase chain reaction and immunohistochemical stains for cytokeratin, chromogranin, and epithelial membrane antigen were performed successfully on a subset of 45 cases with available paraffin blocks. Only 13 of the 45 were fusion-positive, and there was no correlation between fusion status and histology, presence of rosettes, ultrastructural features, or cytokeratin positivity. CD99-negative cases were usually fusion-negative (six of seven cases), and all three chromogranin-positive cases were fusion-negative. Tumor staging, performed on 72 clearly defined and quantifiable cases by using NWTSG criteria, indicated that these are aggressive tumors, because only six were Stage t, compared with 16 Stage 2, 31 Stage 3, and 19 Stage 4 lesions. The authors conclude that NETKs are a somewhat diverse group of generally aggressive, high-grade lesions that may present in a wide age range and are difficult to characterize without immunohistochemistry and cytogenetics/molecular biology.

Original languageEnglish (US)
Pages (from-to)133-146
Number of pages14
JournalAmerican Journal of Surgical Pathology
Volume25
Issue number2
DOIs
StatePublished - Mar 3 2001

Fingerprint

Neuroepithelial Neoplasms
Wilms Tumor
Pediatrics
Pathology
Kidney
Chromogranins
Neoplasms
Keratins
Clear Cell Sarcoma
Organoids
Staining and Labeling
Epithelioid Cells
Mucin-1
Neuropil
Neoplasm Staging
Cytogenetics
Ganglia
Paraffin
Molecular Biology
Histology

Keywords

  • CD99
  • EWS/FLI1 gene fusion
  • Histology
  • Kidney
  • Primitive neuroectodermal tumor
  • Reverse transcriptase-polymerase chain reaction
  • Small cell carcinoma

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

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. / Parham, David M.; Roloson, Gary J.; Feely, Michael; Green, Daniel M.; Bridge, Julia A.; Beckwith, J. Bruce.

In: American Journal of Surgical Pathology, Vol. 25, No. 2, 03.03.2001, p. 133-146.

Research output: Contribution to journalArticle

Parham, David M. ; Roloson, Gary J. ; Feely, Michael ; Green, Daniel M. ; Bridge, Julia A. ; Beckwith, J. Bruce. / 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. In: American Journal of Surgical Pathology. 2001 ; Vol. 25, No. 2. pp. 133-146.
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