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Management of Wilms Tumors

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Santam Chakraborty
Santam Chakraborty

Multimodality management of Wilms Tumor

Health & MedicineTechnology
1 of 127
Management of Wilms' Tumor Moderator: Dr Sushmita Ghoshal
History ,[object Object],[object Object],[object Object]
Epidemiology ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Epidemiology ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Pathogenesis
Renal Development Metanephric blastema
Nephrogenic Rests ,[object Object],[object Object],[object Object],[object Object]
Wilms Tumorigenesis
Genetics of Wilms Tumor ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Pathology
Histology ,[object Object],[object Object],[object Object],[object Object]
Tumor Components
Special Points ,[object Object],[object Object],[object Object],[object Object],[object Object]
Histology: Anaplastic ,[object Object],[object Object],[object Object],[object Object]
Diffuse Anaplasia ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Anaplastic Wilms Tumor ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Classification ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Clinical Features
Symptoms ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Physical Examination ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Congenital Anomalies ,[object Object],[object Object]
Congenital Anomalies ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
WAGR Syndrome Wilms Tumor Aniridia Genitourinary abnormalities Mental Retardation
Denys Drash Syndrome Diffuse Mesangial Sclerosis Male Pseudohermaphorditism Wilms Tumor
Beckwith-Wiedemann Syndrome Microcephaly Umbilical Hernia Ear lobe crease Macroglossia Hemihypertrophy
Workup
Investigations ◄ To establish the diagnosis ◄ Delineate tumor extent ◄ Confirm contralateral renal functional status ◄ Discover any metastasis ◄ Assess fitness for surgery / anaesthesia AIMS
Routine Investigations ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Imaging ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Special investigations ,[object Object],[object Object],[object Object],[object Object]
Pediatric Renal Mass ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Pediatric Renal masses
Staging
Staging: Historical Aspects ►
Stage I ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Stage II ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Stage III ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Stage IV ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Stage V ,[object Object],[object Object],[object Object],[object Object],N.B.: Both systems specify that the tumors should be individually staged.
Overview of the recent system
Treatment History
Radiation Therapy ,[object Object],[object Object],[object Object],[object Object]
Results ,[object Object],[object Object],[object Object],[object Object],[object Object]
Roles of Radiotherapy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Chemotherapy ,[object Object],[object Object],[object Object],[object Object],[object Object]
Treatment
Study Groups ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Multimodality Management: Why? ,[object Object],[object Object],[object Object],[object Object],[object Object]
Management Protocol ,[object Object],[object Object],[object Object],[object Object],[object Object]
Treatment Outline ,[object Object],[object Object],[object Object]
Neoadjuvant Chemotherapy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Surgery
Surgery ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Issues in surgery ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Nephrectomy alone ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Surgical Complications ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Post nephrectomy management
Stage I ,[object Object],[object Object],[object Object],[object Object],A = Actinomycin D (1.35 mg/m 2 ; max 2.3 mg) V = Vincristine (1.5 mg/m 2 ; max 2.0 mg) V*= Vincristine (2.0 mg/m 2 ; max 2.0 mg)
Stage II ,[object Object],[object Object],[object Object],[object Object],[object Object],A = Actinomycin D (1.35 mg/m 2 ; max 2.3 mg) V = Vincristine (1.5 mg/m 2 ; max 2.0 mg) V*= Vincristine (2.0 mg/m 2 ; max 2.0 mg)
Stage III ,[object Object],[object Object],[object Object],[object Object],A = Actinomycin D (1.35 mg/m 2 ; max 2.3 mg) V = Vincristine (1.5 mg/m 2 ; max 2.0 mg) V*= Vincristine (2.0 mg/m 2 ; max 2.0 mg) D = Doxorubicin (45 mg/m 2 ) D* = Doxorubicin (30 mg/m 2 )
Flank Radiation ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Surface Marking: Anterior Upper pole: Midway between the plane of the lower end of the body of the sternum and the transpyloric plane 5 cm Lower pole: Situated midway between the transpyloric and intertubercular planes 7 cm Hilum is on the transpyloric plane, 5 cm. from the middle line 5 cm
Surface marking: Posterior Medial border 2.5 cm from the midline Lateral border 9.5 cm from the midline Tip of spinous process of T11 Tip of spinous process of L3 Parallelogram of Morris
Flank Radiation: Manual ,[object Object],[object Object],[object Object],[object Object],[object Object]
Flank radiation: Simulator ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Whole Abdomen Radiation ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
WAR Manual Marking ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
WAR: Simulator markings ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Stage IV ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Lung Irradiation ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Lung Bath: Manual ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Lung bath: Simulator marking ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Other recommended doses ,[object Object],[object Object],[object Object],[object Object]
Unfavourable Histology ,[object Object],[object Object],[object Object]
Age adjusted doses ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Rhabdoid Tumors ,[object Object],[object Object],[object Object]
Neoadjuvant Chemotherapy ,[object Object],[object Object],[object Object]
Preoperative Chemotherapy: Merits ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Preoperative Chemotherapy: Demerits ,[object Object],[object Object],[object Object],[object Object],[object Object]
Radiation Timing ,[object Object],[object Object],[object Object]
Modern day results
Trials in Wilms Tumor
Management Practice NWTSG SIOP S x CCT S x CCT
Why the difference ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
SIOP vs NWTS regimens
Time-line of SIOP/NWTS trials SIOP 1 (1971-74) SIOP 2 (1974-76) SIOP 5 (1977-80) SIOP 6 (1980-87) SIOP 9 (1987-93) NWTS 1 (1969-74) NWTS 2 (1974-79) NWTS 3 (1979-85) NWTS 4 (1986-94) NWTS 5 (1995-03) SIOP 93-01 (1993-99)
NWTS 1 (1969-74) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
NWTS 1 Results ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
SIOP 1 (1971-74) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
SIOP 1 results ,[object Object],[object Object],[object Object]
NWTS 2 (1974-79) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
NWTS 2
NWTS 2 results ,[object Object],[object Object],[object Object],[object Object],[object Object]
SIOP 2 (1974-76) ,[object Object],[object Object],[object Object],[object Object],[object Object]
SIOP 5 (1977-80) ,[object Object],[object Object],[object Object],[object Object],[object Object]
SIOP 5 results ,[object Object],[object Object],[object Object],[object Object]
NWTS 3 (1979-85) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
NWTS 3
NWTS 3 Results ,[object Object],[object Object],[object Object],[object Object]
SIOP 6 (1980-87) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
SIOP 6 results ,[object Object],[object Object],[object Object],[object Object],[object Object]
SIOP 9 (1987-93) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
SIOP 9 results ,[object Object],[object Object],[object Object],[object Object],[object Object]
NWTS 4 ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
NWTS 4
NWTS 4
NWTS 5 results ,[object Object],[object Object],[object Object],[object Object],[object Object]
SIOP 93-01/GPOH ,[object Object],[object Object],[object Object],[object Object],[object Object]
NWTS 5 ,[object Object],[object Object],[object Object],[object Object]
UKW-3 trial ,[object Object],[object Object],[object Object],[object Object],Preoperative Chemotherapy* Immediate Surgery Delayed Surgery after 6 weeks Protocol specific Adjuvant Therapy Vincristine: 1.5 mg/m 2 weekly ActinomycinD: 1.5mg/m 2 * Preoperative Adriamycin in doses of 30mg/m 2 x 3 weekly was given in event of pre-op unfavorable histology V V V V V V A A
Adjuvant Therapy
Results: Pretreatment Biopsy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Results: Down-staging ,[object Object],[object Object],[object Object],[object Object],[object Object]
Results: Surgical Aspects ,[object Object],[object Object],[object Object],[object Object]
Impact of the trial ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Wilms Tumor in special situations
Relapsed / Refractory WT ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Relapsed / Refractory WT ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
RT for Recurrent Tumors ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Bilateral Wilms Tumor ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Initial open biopsy / Trucut biopsy and staging Downstaging with Chemotherapy Bench Surgery* Partial nephrectomy
Wilms Tumor in Adults ,[object Object],[object Object],[object Object],[object Object]
Wilms Tumor in Horseshoe Kidneys ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Follow up and Toxicity
Treatment toxicity ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Follow up Protocol
Conclusion ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Thank You

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