PMID- 2556868
OWN - NLM
STAT- MEDLINE
DCOM- 19900123
LR  - 20061115
IS  - 0340-241X (Print)
IS  - 0340-241X (Linking)
VI  - 133
DP  - 1989
TI  - [Nephroblastomas (Wilms' tumors) and special variations of nephroblastomas].
PG  - 1-174
AB  - The results of the National Wilms' Tumor Study (NWTS) enabled the subdivision of 
      nephroblastomas into subtypes with "favorable and unfavorable histology". 
      Nephroblastomas with "unfavorable histology" could be discriminated by 
      identifying those tumors not responding to therapeutic regimes proven successful 
      for most cases with "favorable histology". A major disadvantage of the NWTS 
      classification has been the exclusion of cytodifferentiated nephroblastoma 
      variants, which, in contrast to typical nephroblastomas, can be cured by complete 
      nephrectomy with wide excision of perinephric soft tissue. In the current study 
      all types of nephroblastoma and nephroblastoma variants were included to 
      encompass the whole morphological spectrum which these tumors may assume. This 
      unselected material is necessary to define the relation between morphology and 
      prognosis and to compare the treatment results of various clinical trials. Three 
      hundred and four cases of nephroblastoma and related neoplasms on file at the 
      Pediatric Tumor Registry, Kiel, were investigated by conventional light 
      microscopy, electron microscopy, immunohistochemistry and DNA-flow cytometry. Of 
      the "typical" nephroblastomas 50% occurred in the left kidney, 45% in the right 
      kidney, and 5% were bilateral. Five cases were located in extrarenal sites. There 
      were 121 males and 114 females. The peak incidence was noted in the third year of 
      life. Of 135 patients 111 are alive and well, nine are living with disease, and 
      10 patients have died of disease. The blastemal predominant and stromal 
      predominant types in our study were more frequent than in the NWTS. By contrast, 
      the mixed and epithelial predominant types were more frequent in the NWTS. 
      Patients with nephroblastomas of mixed or blastemal predominant type were older 
      than those with epithelial predominant or stromal predominant type. Electron 
      microscopy showed that nephroblastoma is derived from metanephric blastema. 
      Blastemal cells are capable of differentiating into tubular epithelial cells and 
      stromal cells. Undifferentiated blastemal cells contain exclusively vimentin 
      intermediate filaments, better differentiated blastemal cells vimentin and 
      cytokeratin, and stromal cells exclusively vimentin. Preoperative radio- and/or 
      chemotherapy led to a marked reduction of undifferentiated blastema and poorly 
      differentiated tubules, whereas better differentiated tubules, striated muscle, 
      hyaline cartilage, cells with anaplastic and sarcomatous elements were not 
      affected. Thus, identification of highly malignant nephroblastomas with anaplasia 
      and sarcomatous renal tumors was even possible after preoperative treatment. 
      Congenital mesoblastic nephroma (CMN; n = 17) is a low-grade malignant, 
      cytodifferentiated nephroblastoma which very rarely occurs beyond the fourth 
      month of life and has an excellent prognosis, provided it has been completely 
      resected.(ABSTRACT TRUNCATED AT 400 WORDS)
FAU - Schmidt, D
AU  - Schmidt D
AD  - Institut fur Pathologie, Universitat Kiel.
LA  - ger
PT  - English Abstract
PT  - Journal Article
TT  - Nephroblastome (Wilms-Tumoren) und Nephroblastom-Sondervarianten. Pathologie, 
      Klassifikation, Differentialdiagnose.
PL  - Germany
TA  - Veroff Pathol
JT  - Veroffentlichungen aus der Pathologie
JID - 7504167
RN  - 0 (Antigens, Neoplasm)
RN  - 0 (DNA, Neoplasm)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Antigens, Neoplasm/analysis
MH  - Child
MH  - Child, Preschool
MH  - DNA, Neoplasm/analysis
MH  - Female
MH  - Flow Cytometry
MH  - Humans
MH  - Immunoenzyme Techniques
MH  - Immunohistochemistry
MH  - Infant
MH  - Infant, Newborn
MH  - Kidney Neoplasms/analysis/*pathology/ultrastructure
MH  - Male
MH  - Microscopy, Electron
MH  - Prognosis
MH  - Wilms Tumor/analysis/*pathology/ultrastructure
EDAT- 1989/01/01 00:00
MHDA- 1989/01/01 00:01
CRDT- 1989/01/01 00:00
PHST- 1989/01/01 00:00 [pubmed]
PHST- 1989/01/01 00:01 [medline]
PHST- 1989/01/01 00:00 [entrez]
PST - ppublish
SO  - Veroff Pathol. 1989;133:1-174.