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St. Jude Solid Tumor Board St. Jude Solid Tumor Board
27 February 2009 27 February 2009
PET Imaging
PET Imaging of a Wilms Tumor
of a Wilms Tumor
Shulkin,
Barry Shulkin, MD
Jesse Jenkins, III, MD
Andrew Davidoff, MD
Pai-Panandiker,
Atmaram Pai-Panandiker, MD
Shulkin,
Barry L. Shulkin, MD, MBA
• 4-year-old girl
• Physical exam – large right sided
• May 2006 – 1 week constipation and abdominal mass crossing midline
abdominal enlargement
• PH – unremarkable
• Physical exam – abdominal mass
• FH – nothing particularly informative
• Referred to LBCMC – CT confirmed
• SH – lives with parents and 21 y/o brother
abdominal mass
• Sent to SJCRH
1
Jeffrey S. Dome MD JD Main Considerations
• Clear cell variant of Wilms
• Anaplastic Wilms
• Less likely
– ACC
– Primary hepatic tumor (HCC)
– Epithelioid sarcoma
http://www.childrensnational.org/research/faculty/bios/ccir/Dome_j.aspx
St. Jude Solid Tumor Board
27 February 2009
Needle biopsy
• 5/26/06 Needle Biopsy of Right Kidney
and Omentum
• Interventional radiology
5/26/06
Jesse J. Jenkins, III, MD
2
WT1
Immunohistochemistry
St. Jude Solid Tumor Board
27 February 2009
FDG PET CT Scan
PET Imaging
of a Wilms Tumor • 5/30/06
Shulkin,
Barry L. Shulkin, MD, MBA
3
Therapy
• ICE
– Ifosfamide
– Carboplatin
– Etoposide
Alternating with
• VDC
– Vincristine
– Doxorubicin
– Cyclophosphamide
4
St. Jude Solid Tumor Board
27 February 2009
National Wilms Tumor Study
Pre- or Postoperative Therapy
Pre- Group (NWTSG)
for Wilms tumor?
• immediate nephrectomy
• post-operative chemotherapy and
radiation therapy administered
based on the surgical pathologic
Andrew Davidoff, MD stage
International Society of Pediatric Primary Nephrectomy
Oncology (SIOP) • Benefits
– accurate histology
• pre-nephrectomy chemotherapy • no influence of treatment effect
• benign v. malignant
• delayed surgery – congenital lesions, mesoblastic nephroma, angiolipoma
– anaplastic Wilms, rhabdoid, clear cell sarcoma, renal cell
– accurate stage
• post-nephrectomy adjuvant therapy • particularly lymph node involvement
as dictated by histology, stage, • Disadvantages
response – higher incidence of tumor rupture
• similar incidence in single-institution review
– Hall et al, J Pediatr Surg, 2006 (Hospital for Sick Children, Toronto)
Pre-nephrectomy Chemotherapy COG indications for
• Advantages pre-nephrectomy chemotherapy
– Fewer complications (SIOP-93-01 vs NWTS-5)
• Overall: 6.4% vs 9.8% (p=0.12) • Intravenous tumor thrombus extending above the level of the
• lower incidence of tumor rupture hepatic veins
– 2.2% vs 15.3% (p<0.001) – risk of tumor embolus
– Assess tumor response → change therapy – need for cardiopulmonary bypass
– “downstaging” of patients
• Unable to tolerate surgery
• Stage III: 14.2% vs 30.4% (p<0.001)
• lower the use of XRT
– general condition (e.g. nutrition)
– extensive pulmonary metastases→respiratory insufficiency
• Disadvantages
– 5% diagnostic error rate
• Bilateral Wilms tumor
• benign tumors or non-Wilms malignant tumors – tumor in solitary, horseshoe kidney
– “Downstaging” • “Evidence” of pre-operative rupture
• SIOP-6, stage II +/- XRT • “Unresectable”
– more local recurrence when not given XRT
– now give adriamycin for stage II – Size alone is NOT an indication
5
St. Jude Solid Tumor Board
27 February 2009
Pre- or Postoperative
Right Nephrectomy with Sampling of
therapy for Lymph Nodes, Peritoneal Nodules &
Paraduodenal Mass
Wilms tumor? 7/14/06
Remains an unresolved controversy
Jesse J. Jenkins, III, MD
Right Kidney
Right Kidney
?
Questions and Answers
Peritoneal Nodules
Some questions were asked without
Paraduodenal Mass
a microphone nearby
and may be difficult to hear.
6
St. Jude Solid Tumor Board
27 February 2009
Historical data
Radiation Therapy • Now 7 y/o (dx at 4) with hemi-hypertrophy and FH Stage
IV (lung) Wilms’ tumor of the right kidney
8/30/06
• Pre-operative chemotherapy initiated 5/2006
Sup/ant di ti l t diagnosis
• S / t mediastinal LAD at di i
• Pre-operative (7/14/06) diffuse tumor spillage noted
(prior biopsy on 5/26/06)
• Peri-nephric and aorto-caval LN negative
Pai-Panandiker,
Atmaram Pai-Panandiker, MD – LN chains negative by PET, primary heterogeneous uptake
Association with 3 congenital
NWTSG RT dose data
syndromes
• Denys-Drash point mutation WT-1 NWTS-1
– Intersexual disorder (ambiguous genitalia, • Group I
pseudohermaphrodism, streak gonads) – Arm 1 = AMD + RT (18-40Gy by stage/age)
– Nephropathy – Arm 2 = AMD alone
– >90% incidence of Wilms’ Tumor • < 2 y/o RT made no diff when added to AMD
• WAGR • ≥ 2 y/o RT + AMD adds benefit
– Wilms, Aniridia, GU malformation, Mental Retardation • Groups II/III
– 33% develop Wilms’ Tumor – VCN vs AMD vs VA
• For VCN or AMD alone, no difference @ 4yrs ~70% OS
• Beckwith-Wiedemann linked to WT-2 • VA – OS significantly ~ 80-90% @ 4yrs
– Overgrowth complex –hemihypertrophy, organomegaly, • Group IV
macroglossia, omphalocoele, predilection for embryonal tumors – Pre-operative VCN, then VA post-op vs
– <5% incidence of Wilms’ Tumor, but synch/metach bilateral – Post-op RT + VA
disease rates are higher • Pre-op VCN not helpful
ALL NWTS studies showed decrement to OS if post-op RT delayed > post-op day 9
NWTS – 3 (added staging and
NWTS - 2
histology to stratification)
• Gp I: post-op VA (no RT)
• Stage I: post-op, no RT
– 6 months VA vs 15 months VA – No diff between 10 wks vs 6 months VA
– No diff, 6 months standard (OS~90%) • Stage II: post-op, 4 arms (no RT vs 20 Gy)
• Gp II-IV: post-op RT (same age dependent scale – Chemo = VAA x 15 months vs Intensive VAA x 15 months
• No benefit to adding Doxo or RT
from NWTS 1) & chemo
• Stage III: post-op, RT (RND 10 vs 20 Gy)
– 15 months VA vs 15 months VAA – Intensive VA x 15 months vs VAA x 15 months
– FH II/III = 3 yr OS ~80-90% • RT to 10 Gy for St III FH suffices, Doxo adds OS benefit
• Doxo adds significant survival benefit~10% • Stage IV(all UH/any St): post-op, RT to 20 Gy
– Improved 82% OS to 92 % OS with doxo – RND VAA vs VAA + CYT x 15 months
• CYT no benefit to St IV FH, helps focally anaplastic and CCSK, not
• Gp IV VAA = 3 yr OS ~ 60% vs 44% w/o doxo RTK
• VAA + WLI/WAI had 4 yr OS ~ 81%
Lung mets initially got 14 Gy, but 10% developed pneumonitis: dropped dose to 12 Gy
7
Stage FH Anaplastic CCK/Rhabdoid
NWTS - 4 I-II •No RT •Stage I: no RT •All receive flank or
•Stage II: flank RT abdomen RT
• Minimization of therapy and toxicity III •Flank RT: If involving renal hilar •The same as FH •The same as FH
– Economic assessment nodes, gross or microscopic disease stage III stage III
confined to the flank, or PA nodes
• For St III/IV FH, St I-IV CCSK •WART: peritoneal seeding, gross
– VAA vs pulsed/intense VAA residual abdominal disease, intra-
p p p y
peritoneal spill or rupture. 10.8Gy in
Chemotx was RND 26 vs 65 weeks
• Ch t k 6 fx, boost tumor >3cm to an
• FH got 10.8 Gy abdominal RT additional 10.8Gy.
• 12 Gy WLI for St IV
– No diff, between risk groups, P/I vs non-P/I or length IV •Treat abdomen as per the intra- •The same as FH • The same as FH
of chemotherapy abdominal stage (ie for local stage Stage IV stage IV except stage I
• Risk of relapse increased for: I/II, no RT) and II abdominal
– Higher stage, aggressive histologies, + margins, + nodes, older •Whole lung: resect or 12Gy, boost disease is also
children, incomplete resection, spillage or tumor rupture persistent mets to 19.5Gy irradiated.
•Irradiate liver in certain
circumstances
Wilms’ Tumor – Low and Standard Wilm’s Tumor – High Risk Therapy
Risk Therapy (AREN 0532) (AREN 0533)
• No RT for FH Stage
I/II
– Relapsed disease
treated at primary or
met site as indicated
• Focal anaplasia now
treated (incl. Stage I)
• Radiotherapy starts by post-op day 9
• Stage III FH: flank vs
WAI • By nature, planning commonly 2-dimensional
– Local vs diffuse • Patient age and under-development toxicities
disease – Bone growth, renal, bowel, lung and secondary cancers
Wilm’s Tumor – High Risk Therapy Wilms Tumor – High Risk Therapy
(AREN 0533) (AREN 0533)
8
Toxicities
• Orthopedic
• Hepatic
• Renal
• Pregnancy
• Cardiac
• Secondary
Malignancy
9
St. Jude Solid Tumor Board
27 February 2009
PET Scanning of Wilms Tumors
PET Scanning • Shulkin BL, Chang E, Strouse PJ et al. PET FDG studies
of Wilms tumors. J Pediatr Hematol Oncol.
1997;19(4):334-338.
– Metabolic activity
• Misch D, Steffen IG, Schonberger S et al. Use of
it i i tomography f staging, preoperative
positron emission t h for t i ti
response assessment and posttherapeutic evaluation in
children with Wilms tumour. Eur J Nucl Med Mol
Imaging. 2008;35(9):1642-1650
– 9 patients with newly diagnosed Wilms’ tumor and
– perfect concordance between the PET-CT and conventional
Barry L. Shulkin, MD, MBA
Shulkin, imaging studies
– None of the patients had metastatic disease
PET Scanning of Wilms Tumors PET Scanning of Wilms Tumors
• AKM Moinul Hossain MD PhD • 27 patients
• 3 institutions • 58 scans
– St. Jude Children’s Research Hospital – 1 at diagnosis
Cincinatti Children’s Hospital
– Ci i tti Child ’ H it l following
– 1 f ll i surgery
– Vancouver Children’s Hospital – 56 relapse, progressive disease, monitoring of
therapy
PET Scanning of Wilms Tumors PET Scanning of Wilms Tumors
• 34 scans abnormal • Two of 8 patients with lung metastases
– 8 patients (24 scans) had pulmonary metastases
larger than 10 mm in diameter
had variable uptakes.
– 10 patients (12 scans) had hepatic metastases • Lung lesions 10 mm or smaller were not
– 11 patients (11 scans) had regional nodal scans.
consistently visualized on PET scans
involvement
– 3 patients (3 scans) had bone metastases • One patient with liver metastasis showed
– 1 patient (1 scan) had chest wall involvement no uptake on PET scan after treatment
– 2 patients (2 scans) had pancreatic metastasis (size decreased from 45 mm to 15 mm)
– 5 patients (5 scans) had abdominal and pelvic soft
tissue involvement
10
8-year-old girl with recurrent Wilms’ tumor showing 9-year-old girl with bilateral lung metastases
focal uptake in the liver
11 year old
11-year-old girl with recurrent
Wilms’ tumor showing focal
uptake and sclerotic process in the
left proximal tibia; consistent with
bony metastatic disease
7-year-old girl with newly diagnosed right sided Wilms’ tumor
showing extensive metastatic disease in the chest, abdomen and
pelvis
PET Scanning of Wilms Tumors
• 27 patients
• 58 scans
– 1 at diagnosis
following
– 1 f ll i surgery
– 56 relapse, progressive disease, monitoring of
therapy
10-year-old girl with left sided recurrent Wilms’ tumor showing two liver
metastases (yellow arrows) on contrast enhanced CT and three foci on MRI not
detectable on PET-CT fusion image (without intravenous contrast). The lesion in
the pancreatic tail area (red arrows) is evident in all images.
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Patient Followup Conclusion
• Per Fariba Navid MD • FDG PET CT useful in Wilms
tumors
– Staging +/-
– Recurrent disease
– Monitoring during and following
therapy
– Lung metastases < 1 cm better
visualized with CT
Thanks
?
Jesse Jenkins, MD
Andrew Davidoff, MD
Atman Pai, MD
Moinul Hossain, MD Questions and Answers
Najat Daw, MD Some questions were asked without
a microphone nearby
and may be difficult to hear.
End
Shulkin,
Barry Shulkin, MD
Jesse Jenkins, III, MD
Andrew Davidoff, MD
Pai-Panandiker,
Atmaram Pai-Panandiker, MD
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