TY - JOUR
T1 - Anaplastic histology Wilms’ tumors registered to the Japan Wilms’ Tumor Study Group are less aggressive than that in the National Wilms’ Tumor Study 5
AU - Oue, Takaharu
AU - Koshinaga, Tsugumichi
AU - Takimoto, Tetsuya
AU - Okita, Hajime
AU - Tanaka, Yukichi
AU - Nozaki, Miwako
AU - Haruta, Masayuki
AU - Kaneko, Yasuhiko
AU - Fukuzawa, Masahiro
AU - Renal Tumor Committee of the Japanese Children’s Cancer Group, Tumor Committee of the Japanese Children’s Cancer Group
N1 - Publisher Copyright:
© 2016, Springer-Verlag Berlin Heidelberg.
PY - 2016/9/1
Y1 - 2016/9/1
N2 - Purpose: To evaluate the clinical features and treatment results of anaplastic histology (AH) Wilms’ tumor (WT) patients registered in the Japan Wilms’ Tumor Study (JWiTS) group to elucidate the clinical characteristics of AH in the Japanese population. Patients and methods: Of 344 WT patients who were enrolled in JWiTS between 1995 and 2013, 17 had AH. Treatment using the JWiTS protocols was similar to the fifth National Wilms’ Tumor Study 5 (NWTS-5) protocols. Clinical characteristics and mutation status of TP53 gene were evaluated and compared with those in NWST-5 study. Results: AH incidences in JWiTS were 4.9 %, lower than that in NWTS-5. Seven tumors had focal AH and 10 had diffuse AH. Clinical stages of AH patients were stage I in seven, stage II in three, stage III in five, stage IV in one and unknown in one. Four-year event-free survival and overall survival rates were 90.9 and 86.7 %, respectively. Two patients with diffuse AH and none with focal AH had TP53 mutation. Conclusion: Japanese patients presented with higher incidence, earlier stages and may have better outcomes than American patients, indicating a possible biological heterogeneity of AH WT. Further analysis is necessary to elucidate the different characteristic of AH WT between Japanese and American populations.
AB - Purpose: To evaluate the clinical features and treatment results of anaplastic histology (AH) Wilms’ tumor (WT) patients registered in the Japan Wilms’ Tumor Study (JWiTS) group to elucidate the clinical characteristics of AH in the Japanese population. Patients and methods: Of 344 WT patients who were enrolled in JWiTS between 1995 and 2013, 17 had AH. Treatment using the JWiTS protocols was similar to the fifth National Wilms’ Tumor Study 5 (NWTS-5) protocols. Clinical characteristics and mutation status of TP53 gene were evaluated and compared with those in NWST-5 study. Results: AH incidences in JWiTS were 4.9 %, lower than that in NWTS-5. Seven tumors had focal AH and 10 had diffuse AH. Clinical stages of AH patients were stage I in seven, stage II in three, stage III in five, stage IV in one and unknown in one. Four-year event-free survival and overall survival rates were 90.9 and 86.7 %, respectively. Two patients with diffuse AH and none with focal AH had TP53 mutation. Conclusion: Japanese patients presented with higher incidence, earlier stages and may have better outcomes than American patients, indicating a possible biological heterogeneity of AH WT. Further analysis is necessary to elucidate the different characteristic of AH WT between Japanese and American populations.
KW - Anaplastic
KW - JWiTS
KW - NWTS
KW - TP53 mutation
KW - Wilms’ tumor
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U2 - 10.1007/s00383-016-3929-7
DO - 10.1007/s00383-016-3929-7
M3 - Article
C2 - 27473009
AN - SCOPUS:84980027722
SN - 0179-0358
VL - 32
SP - 851
EP - 855
JO - Pediatric surgery international
JF - Pediatric surgery international
IS - 9
ER -