Wilms Tumor and Retinoblastoma-Jordanian Experience

RB status in Jordan prior KHCC

• No standard of care• Enucleation and XRT: RX of choice• No team approach

2001: 38% mortality ??? 100 enucleation for unilateral 92% enucleation for bilateral

• Over decades RB patients in Jordan were treated by different physicians in different institutions. The outcome was sub-optimal .

How could this be solved?Team approach usingTwinning and telemedicine.To develop

The first eye salvage program in Jordan

The concept of this program at KHCC was based on :1. Establishment of a multidisciplinary team in one

center, with comprehensive diagnostic and therapeutic facilities

2. Provision of state-of-the-art care through partnershipwith one of the best international RB centers in the world (St. Jude)

Started building a Team & getting Equipments

King Hussein Cancer Center (KHCC) An integrated multi-disciplinary comprehensive

center in the Middle East for cancer management in April 2003 a dedicated unit for Ocular Oncology

Our team consisted of: Pediatric oncologist Radiation oncologist Ophthalmologist Coordinator

Both Teams

Twinning with St. Jude:

Ophthalmologists (6)

RadiotherapistPediatric oncologist (2) Pathologists

Physicist OcularistOptimetrist

Coordinator NursingOccupational therapy

Ocular Oncologist Retina Surgeon

Pediatric Ophthalmologist Oculoplastic surgeon

Assessment options1. EUA 2. Ret Cam. 3. B-scan + UBM. 4. OCT. X (yes for older kids)5. CT scan (contraindicated for most cases).6. MRI. 7. BM+ LP. 8. Pathology Lab. 9. Genetic Testing. x

Treatment options1. Systemic Chemotherapy. 2. Regional chemotherapy (IAC, ivC, SubTenon) 3. Focal therapy (Laser+ Cryotherapy). 4. Radiation therapy (EBRT, Plaque therapy). 5. Enucleation (integrated and non integrated implants). 6. Prosthesis fitting. 7. Intra thecal Chemotherapy. 8. BMT (for metastatic diseases) 9. Low vision aids.

International Awareness The Eye Cancer Foundation

http://eyecancercure.com/find-a-doctor

Conclusion

• Changing the fate of RB patients in Jordan and the middle east (pre and post KHCC era).

• Results comparable to the developed countries. • This never be achieved without admin support

over years and the assistance and supervision from St. Jude.

• Telemedicine saves time and effort through partnership that can ensure the use of uniform treatment protocols for rare diseases like RB.

Wilms Tumor @ KHCC

• Upfront nephrectomy followed by chemotherapy (NWTS5) till 2006

• Preoperative chemotherapy followed by nephrectomy (SIOP2001) from 2006 till 2010

• Selective protocol based on MDC discussion. Surgery is decided based on imaging studies. From 2011 till 2018

• January 2018 till now: UMBRELLA protocol (not official yet).

• No significant change over time!

Patients diagnosed 2006-2017

AgeMedianRange

3.630.4-18

GenderFemalesMales

6574

StageIIIIIIIVV

1930344115

88% ± 3%81% ± 3.4%

I = 84 %±8.4 II = 85 %± 6.8% III=84 %±6.4% IV =76%± 6.9% V = 72 %±12%

I = 90%±7% II = 92%±5.7% III=93%±4.7 IV = 82%±6.3% V = 79%±11%

Why UMBRELLA?• Delayed surgery is convenient to patients and

surgeons.• Preoperative chemotherapy may decrease risk of

rupture• Less use of radiotherapy• With new data: Stage II and some stage III patients do

not require doxorubicin • Identify blastema predominant tumors (cannot be

defined in COG) –> intensified treatment is very important.

• Most patients with stage I will receive only 8 weeks of chemotherapy (4 preop and 4 postop)

Obstacles

• Patients with huge tumors referred to KHCC: malnourished, anemic, long duration of symptoms

• Patients having surgery outside: No LN sampling, inadequate surgical reports

• Nonjordanians not covered (dependent on the Foundation): yes no difference in outcome

• Bilateral tumors (N=15) requiring multiple surgeries/FU

Lessons learned• MDC discussions are very important• Surgeons experience is very important• Do not underestimate toxicity (a patient with stage I

died because of VOD)• Preoperative chemotherapy is highly recommended• Lung radiotherapy is not needed for most patients

(evaluate CT response to decide)• Aggressive chemotherapy using relapsed NWTS-5

protocol may salvage half of relpased patients• ABMT can be effective in selective cases (2 out of 3

transplants at KHCC are alive and in CR).