240                                                                                                              Wilms’ Tumor (Nephroblastoma) 

 

51.    Wilms’ Tumor (Nephroblastoma)

 

51.1     General considerations

The National Wilms’ Tumor Study Staging System and the Nephroblastoma Staging  System of the Societe International d’Oncologie Pediatrique (SIOP) have gained greater acceptance than the TNM classification on Wilms’ tumors.

 

National Wilms’ Tumor  Study Staging System    

Stage                             Description

I                           Tumor is limited to the kidney and can be completely resected.

                            The renal capsule has an intact outer surface. The tumor is not

                            Ruptured or has not undergone biopsy before removal. No

                            Involvement of the renal sinus vessels

II                         Tumor extends beyond  the kidney but can be completely

                             resected. There is regional extension of tumor or extensive    

                             invasion of the renal sinus. Blood vessels outside the kidney   

                             contain  tumor.The tumor may have undergone biopsy,or there

                             may have been local spillage of tumor confined to the flank ,

                             but there is not involvement of the peritoneal surface

III                         Residual nonhematogenous tumor, confined to the abdomen.

                             Any of the following may occur.

1)      tumor involvement in lymph nodes within the abdomen or  pelvis (renal hilar, paraaortic, or beyond , excluding extraabdominal or thoracic

2)      tumor penetration through the peritoneal surface or implants on the peritoneal surface.

3)      gross or microscopic tumor remains postoperatively

4)      tumor is not completely resectable because of local infiltration into vital structures, or

5)      tumor spill not confined to the flank occurs either before or durging surgery . 

IV                           hematogenous metastases (lungs, live, bone, brain, or  lymph    

                               node involvement outside abdominal/pelvic region) are

                               present

V                            Bilateral renal involvement is present at diagnosis

 

 

SIOP Nephroblastoma Staging System   

Stage                             Description

I                                       Tumor limited to the kidney , complete excision

II                                      Tumor extending outside the kidney, complete excision

a)      invasion beyond the capaule, perirenal / perihilar

b)      invasion of the regional lymph nodes ( hilar nodes and /or periartic nodes at the origin of the renal artery ) (stage II N1)

c)      invasion of extrarenal vessels

d)      invasion of ureter

III                                     Invasion beyond the capsule, incomplete excision

a)      preoperative or perioperative biopsy (fine-needle biopsy as  method of aspiration cytology should not be considered as a biopsy)

b)      preoperative / perioperative rupture

c)      peritoneal metastases

d)      invasion of paraaortic lymph nodes (paraaortic nodes below the renal artery

e)      Incomplete excision

IV                                    Distant metastases

V                                      Bilateral renal tumors

           

A multidisciplinary- stage- and risk- adapted- approach which combines surgery and multidrug chemotherapy, and , when necessary, radiotherapy, is the basis of current therapy.

 

            Therapy is best provided according to a current protocol in a pediatric oncology 

             center that has the experience of a multidisciplinary team. For that reason only a 

             few review articles and reports from large multicentric studies as well as one

             example for a contemporary protocol are outlined here.

 

            Lierature: e.g.

                        BLAKELY and RITCHEY, Semin. Pediatr. Surg. 10 (2001): 127 – 131

                        (controversies in the management)

                        COPPES et al, Paediatr. Drugs 1 (1999): 251-262

FARHAT et al, Urol. Clin. North Am. 27 (2000): 455-462 (surgical consideration and controversies )

                        GRAF et al, Urol. Clin. North Am. 27 (2000): 443-454 (role of

                        preoperative chemotherapy in SIOP studies)

                        GREEN, Eur. J. Cancer 33 (1997): 409 – 418

                        GREEN et al, J. Clin. Oncol. 16 (1998): 237-245 and  3744-3751

                        (National Wilms’ Tumor study Group Study NWTS-4)

                        MISER and TOURNADE, Hematol. Oncol. Clin. North Am. 9 ( 1995):

                        1287-1301 (relapsed tumors)

                        NEVILLE and RITCHEY, Urol. Clin. North Am. 27 (2000): 435 – 442

                        (overview of National Wilms’ Tumor Study Group results)

                        PRITCHARD et al, J. Clin. Oncol. 13 (1995): 124- 133 (United Kingdom

                        Children’s Cancer Study Group Wilms’ Tumor study UKW1)

                        TOURNADE et al, J. Clin. Oncol. 11 (1993): 1014-1023 (SIOP Wilms’

                        Tumor study 6)

 

242                                                                                                        Wilms' Tumor (Nephroblastoma)

 

51.2        Ninth International Society of Pediatric Oncology wilms' Tumor Trial and Study

 

51.2.1.   SIOP  9 protocol

 

 

 

  

51.2.2.                       SIOP 9 treatment schedules

 

51.2.2.1Pre- operative chemotherapy

 

            

51.2.2.2Post -operative treatment

 

            

 

51.3.      Relapsed and poor risk wilms' tumor

ICE    

Ifosfamide                 1800 mg/m2                i.v.                                 d 1-5

                                                                     With mesna uroprotection  

Carboplatin                400 mg/m2                 i.v.                                  d 1+2

Etoposide                   100 mg/m2                 i.v.                                 d  1-5

To be repeated every 3 weeks

 

Literature:

            ABU-GHOSH et al, Ann. Oncol. 13 (2002): 460 – 469