oncologic emergency JWiTS EE4A 16 Hb 7.6 mg/dl 20 Hb 4.4 mg/dl CT Wilms oncologic emergency Wilms 1 del(11)(p11.2p13) WAGR Wilms X 10 cm 10 cm

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1 pp doi: /jjsps.52.5_ oncologic emergency Wilms WAGR 10 cm Wilms JWiTS EE4A 16 Hb 7.6 mg/dl 20 Hb 4.4 mg/dl CT WAGR Wilms oncologic emergency I Wilms 1) Wilms oncologic emergency 2)3) WAGR Wilms II atrial septal defect ASD AMPLATZER TM Septal Occluders AMPLATZER 100 mg/ ,780 g 11 46XY del(11)(p11.2p13) WAGR 10 cm WAGR CT MRI 10 cm Wilms 109 cm 26 kg GCS C 89/ 123/85 mmhg 18 / 10 cm X 10 cm 1 CT

2 X 10 cm 2 CT 10 cm cm MRI T1 T2 Wilms APTT APTT C WBC 11,800/μl, CRP mg/dl Staphylococcus aureus AMPLATZER AMPLATZER Wilms stage I JWiTS EE4A 4 WBC 6,400/μl, CRP 0.34 mg/dl 16 Hb 7.6 mg/dl 2 20 Hb 4.4 mg/dl CT 20 WBC 7,700/μl Neut 69 RBC 180 /μl Hb 4.4 g/dl Ht 13.3 Plt 36.6 /μl PT 12.7 sec PT INR 1.07 APTT 58.2 Fib 392 mg/dl FDP 8.8 μg/ml TP 7.50 g/dl Alb 4.10 g/dl T-Bil 0.58 mg/dl AST 45 IU/ l ALT 27 IU/ l LDH 920 IU/ l ALP 331 IU/ l CPK 20 IU/ l Amy 93 IU/ l Na 129 mmol/ l K 4.8 mmol/ l Cl 90 mmol/ l BUN 5.0 mg/dl Cr 0.28 mg/dl CRP 6.19 mg/dl CT 20 CT 11.5 cm

3 CT CT 11.5 cm 5HE 400 The National Wilms Tumor Study Group NWTSG stage I JWiTS EE4A mg/ III 4 3 1,080 g 4 5 Mixed type Wilms tumor Wilms Wilms WAGR 4)5) WAGR 4 Wilms WAGR Wilms )7) Wilms 4 WAGR WAGR 8) WAGR 5 Wilms Wilms 7)8) 6 Wilms WAGR Fischbach 7) Wilms

4 NWTSG Children s Oncology Group COG the International Society of Pediatric Oncology SIOP 4 90 JWiTS stage V NWTSG spillage 9) COG stage III upstage SIOP 10) 5 Wilms ) Brisse 3) SIOP Wilms Wilms interventional radiology IVR 2) ASD JWiTS EE4A WAGR Wilms Wilms Bhatnagar S: Management of Wilms tumor: NWTS vs SIOP. J Indian Assoc Pediatr Surg, 14: 6-14, Smith NP, Jesudason EC, McDowell HP, et al: Emergent embolisation to control severe haematuria in Wilms tumour. Pediatr Surg Int, 21: , Brisse HJ, Schleiermacher G, Sarnacki S, et al: Preoperative Wilms tumor rupture. Cancer, 113: , Riccardi VM, Sunansky E, Smith AC, et al: Chromosomal imbalance in the aniridia-wilms tumor association: 11p interstitial deletion. Pediatrics, 61: , Francke U, Holmes LB, Atkins L, et al: Aniridia- Wilms tumor associations: Evidence for specific deletion of 11p13. Cytogenet Cell Genet, 24: , Muto R, Yamamori S, Ohashi H, et al: Prediction by FISH analysis of the occurrence of Wilms tumor in aniridia patients. Am J Med Genet, 108: , Fischbach BV, Trout KL, Lewis J, et al: WAGR Syndrome: A clinical review of 54 cases. Pediatrics, 116: , Breslow NE, Norris R, Norkool PA, et al: Characteristics and outcomes of children with the Wilms tumor-aniridia syndrome. A report from the National Wilms Tumor Study Group. J Clin Oncol, 21: , Bergeron C, Graf N, Van Tinteren H, et al: From results of SIOP-Nephroblastoma Study to SIOP Nephroblastoma 2001 Study. Med Pediatr Oncol, 41: 289, Weirich A, Leuschner I, Harms D, et al: Clinical impact of histologic subtypes in localized non-anaplastic nephroblastoma treated according to the trial and study SIOP-9/GPOH. Ann Oncol, 12: , Mitchell C, Jones PM, Kelsey A, et al: The treatment

5 of Wilms tumour: Results of the United Kingdom Children s Cancer Study Group (UKCCSG) second Wilms tumour study. Br J Cancer, 83: , An Oncologic Emergency Case of Wilms Tumor Accompanying an Intratumoral Hemorrhage During Preoperative Chemotherapy Tomohiro Kurahachi 1, Minoru Yagi 1, Kimio Asagiri 1, Suguru Fukahori 1, Sinji Ishii 1, Naoki Hashizume 1, Saki Sakamoto 1, Shiori Turuhisa 1, and Miho Mitsuo 2 1 Department of Pediatric Surgery, Kurume University School of Medicine 2 Department of Pediatrics and Child Health, Kurume University School of Medicine A 6-year-old boy diagnosed with WAGR syndrome presented with bloody urine and a left abdominal tumor. On ultrasonographic examination, a mass (maximum 10 cm) was observed in the left kidney. The mass was diagnosed as Wilms tumor and tumorectomy was planned. However, Key words: WAGR syndrome, preoperative chemotherapy, Wilms tumor, pediatric, oncologic emergency Correspondence to: Tomohiro Kurahachi, Department of Pediatric Surgery, Kurume University School of Medicine, 67 Asahimachi Kurume City, Fukuoka, JAPAN the boy presented with fever, and infectious endocarditis was strongly suspected owing to his previous history of heart operation with an Amplatzer device. Instead of the postponement of the tumorectomy, preoperative chemotherapy (ActD + VCR for four weeks) was initiated. On post-chemotherapeutic day 20, the boy suffered from severe anemia, and multiple intratumoral hemorrhages were observed on contrast-enhanced CT images. Therefore, an emergency tumorectomy was performed. We herein describe our experience with a difficult case of Wilms tumor treated as an oncologic emergency.