Parallel Meeting on Wednesday 17, April 2024, 17:50 - 18:20
17:50 - 17:53
S06-1 (OP)
Andrea ZULLI 1, Alberto MANTOVANI 1, Anna PERRONE 2, Chiara CAPORALINI 3, Luca LANDI 1, Maria TAVERNA 1, Chiara CINI 1, Giulia BORTOT 1, Laura OLIVERA 1, Antonio ELIA 1 and Lorenzo MASIERI 1
1) Meyer Children's Hospital IRCCS, Pediatric Urology, Florence, ITALY - 2) Meyer Children's Hospital IRCCS, Pediatric Radiology, Florence, ITALY - 3) Meyer Children's Hospital IRCCS, Pahology, Florence, ITALY
INTRODUCTION
UMBRELLA protocol aims to reduce the unilateral Wilms Tumour (WT) volume with pre-operative chemotherapy, thus facilitating surgery. Previous AIEOP protocol allowed surgery as first-line approach instead. We evaluated the efficacy of UMBRELLA protocol in reducing tumour volume, and compared surgical outcomes of UMBRELLA vs AIEOP treatments.
METHODS
Data form patients treated at our institute for unilateral WT with AIEOP (2007-2016) and UMBRELLA (2017-2023): demographics, pre/post-chemotherapy tumour volumes, surgical outcomes, histological patterns were analysed. Mann-Whitney U test was used to compare data.
RESULTS
25 patients treated with AIEOP and 18 with UMBRELLA were collected (44 nephrectomies). Mean pre-op volume was 147 ml (79-284) and 205 ml (138-521) for AIEOP and UMBRELLA respectively (p=0,14). No positive resection margins were found, except one for rupture of the tumour capsule in the AIEOP group. AIEOP group showed increased operative time compared to UMBRELLA (193 vs 140 min, p=0,04).
Among patients treated with UMBRELLA, 16/18 received pre-operative chemotherapy. All these tumours shrunk of a mean of 77 ml (13-193), with a median reduction of 74% (60-87, p=0,04). Blastemal and Mixed tumours reduced less compared to Epithelial and Regressive types: 69%-55% vs 87%-78%, respectively (p=0,48).
CONCLUSIONS
Our study reports the efficacy of UMBRELLA pre-operative chemotherapy in obtaining significant volume reduction in all WT histotypes, allowing a quicker and safer surgical procedure in terms of surgical time and complications compared to not pre-operatively treated WT.
17:53 - 17:56
S06-2 (OP)
Carolina BEBI 1, Tom WATSON 2, Alberto MANTOVANI 1, Jesper BROK 3, Alexander CHO 1, Riwa MESHAKA 2, Neil SEBIRE 4, Catriona DUNCAN 3, Tanzina CHOWDHURY 3, Imran MUSHTAQ 1 and Naima SMEULDERS 1
1) Great Ormond Street Hospital for Children, Paedriatric Urology, London, UNITED KINGDOM - 2) Great Ormond Street Hospital for Children, Paedriatric Radiology, London, UNITED KINGDOM - 3) Great Ormond Street Hospital for Children, Paedriatric Oncology, London, UNITED KINGDOM - 4) Great Ormond Street Hospital for Children, Paedriatric Histopathology, London, UNITED KINGDOM
PURPOSE
Nephron Sparing Surgery (NSS) for syndromic or bilateral renal tumours seeks maximal preservation of renal function. However, incomplete surgical resection risks increased post-operative treatment or total nephrectomy. This study assesses intraoperative ultrasound's role in reducing positive surgical margins at NSS.
MATERIAL AND METHODS
This single centre prospective cohort study compares patients who underwent US-guided-NSS with historical controls who underwent NSS without US guidance. On-table US was performed by a Consultant Radiologist using a linear array transducer ("hockey stick") on the renal capsule. NSS sought tumour resection with a rim of normal parenchyma. It was utilised for multiple lesions in the same kidney, if necessary. Data included clinical characteristics, demographics, histological diagnoses, surgical margins. Fisher's exact test explored significance of margin positivity for malignant lesions; nephrogenic rests (NR) were excluded in view of their unencapsulated nature.
RESULTS
Among the 87 non-US-guided NSS (November 2001 to November 2016) in 45 patients (median age 29 months), we encountered 58 Wilms tumours (WT), 2 equivocal WT, and 27 NR. In the 20 US-guided NSS (performed from November 2016 to June 2023) in 13 patients (median age 18 months), we identified 7 WT, 3 RCC, and 10 NR. Positive margins for malignant lesions occurred in 14/60 (23.3%) non-US-guided NSS, but in 0/10 US-guided NSS (p=0.19).
CONCLUSIONS
Intraoperative ultrasound delineation of renal tumours at NSS eliminated positive surgical margins for malignant lesions in our cohort. This failed to reach statistical significance and an extended cohort is warranted to clarify significance.
17:56 - 17:59
S06-3 (OP)
Kristina DZHUMA 1, Alexander CHO 1, Muthialu NAGARAJAN 2, Tanzina CHOWDHURY 3, Catriona DUNCAN 3, Alice TAYLOR 4, Mary MATHIAS 4, Tom WATSON 5, Susan SHELMERDINE 5, Neil SEBIRE 6, Abraham CHERIAN 1, Imran MUSHTAQ 1 and Naima SMEULDERS 1
1) Great Ormond Street Hospital NHS Foundation Trust, Department of Paediatric Urology, London, UNITED KINGDOM - 2) Great Ormond Street Hospital NHS Foundation Trust, Department of Cardiothoracic Surgery, London, UNITED KINGDOM - 3) Great Ormond Street Hospital NHS Foundation Trust, Department of Oncology, London, UNITED KINGDOM - 4) Great Ormond Street Hospital NHS Foundation Trust, Haemophilia Comprehensive Care Centre, London, UNITED KINGDOM - 5) Great Ormond Street Hospital NHS Foundation Trust, Department of Clinical Radiology, London, UNITED KINGDOM - 6) Great Ormond Street Hospital NHS Foundation Trust, Department of Histopathology, London, UNITED KINGDOM
PURPOSE
Incomplete tumour-thrombus resection(TTR) is associated with reduced survival. Cavectomy carries significant morbidity/mortality. Alternatively, after TTR of adherent tumour-thrombus, patching may be required to maintain inferior-vena-cava(IVC) patency. We report the outcomes of IVC-patch-reconstruction(IVC-PR) following TTR in children with intravascular-extension of renal-tumours.
MATERIAL AND METHODS
With institutional approval, prospective cohort of patients who underwent TTR-IVC-PR (January2007-January2023). Doppler US assessed IVC patency. Secondary outcomes included: 30-day surgical mortality/morbidity and 5-year event-free survival(EFS).
RESULTS
Wilms tumour 19(90%) and renal Ewings-sarcoma 2(10%)comprised diagnoses in 21 patients, age 2-16years. At diagnosis, 8(38%) were metastatic, 1(5%) bilateral. TT proximal extent was right-atrium-8(38%), hepatic-veins-9(43%) and infrahepatic-IVC-4(19%); distal extent was iliac veins-4(19%); contralateral renal vein-10(48%). 5(24%) received anticoagulation preoperatively, 4 with bleeding complications. TTR-IVC-PR occurred alongside primary tumour-nephrectomy in 19(90%);as a secondary procedure after disease progression/relapse despite radiotherapy in 2(10%). Cardio-pulmonary-bypass was used in 17(81%) for median 104(range:63-195)minutes. The patch was Bovine Collgen-19(90%), Gor-Tex®-1(5%), Dacron®-1(5%). A macroscopically complete TTR was achieved for all, en-bloc in 17(81%). Stage III was assigned to 19(90%), with viable TT in 15(71%). While there was no 30-day surgical mortality, complications in 9(43%) required a return-to-theatre for 4(19%): bleeding-2, drain insertion-2.
At median 54(range 10-180)months follow-up, the IVC was patent in 15(71%). Collateral vessels, without varicosity, lower-limb-oedema or venous-claudication were demonstrated in 8(38%). Postoperative anticoagulation was continued for median 5(range 2-14)months in 20(95%), without bleeding complications. Relapse occurred in 2(10%) patients, both after secondary TTR, of whom 1 died.
CONCLUSIONS
TTR at radical nephrectomy appears important for EFS. No surgical mortality occurred; morbidity, though common, required a return-to-theatre for <20%. The IVC was patent in most, without clinically-significant adverse-effects for children without patency.
17:59 - 18:02
S06-4 (OP)
Kieran J MOORE 1, Ainsley BETHUNE 2, Daniel KEEFE 1, Jack BRZEZINSKI 3, Mandy RICKARD 4, Armando J LORENZO 5 and Rodrigo Lp ROMAO 6
1) Dalhousie, Division of Pediatric Urology: IWK Health Centre, Halifax, CANADA - 2) Dalhousie University, Faculty of Medicine, Halifax, CANADA - 3) Sick Kids, Paediatric Oncologist, Division of Haematology/Oncology, Toronto, CANADA - 4) Sick Kids, Division of Pediatric Urology, Hospital Sick Kids, Toronto, CANADA - 5) Sick Kids, Division of Urology, The Hospital for Sick Children, Toronto, CANADA - 6) Sick Kids, Division of General & Thoracic Surgery and Division of Urology, Toronto, CANADA
PURPOSE
Wilms’ tumor (WT) carries a high survival rate. Treatment-related gonadotoxicity is perceived as low. Herein, we challenge this assumption by reporting on exposure to gonadotoxic treatments in a population-based national cohort of patients with WT.
MATERIAL AND METHODS
The following variables were collected for patients with WT from the CYP-C (cancer in young people in Canada) database from 2001 to 2018: sex, age at diagnosis, chemotherapy agents and doses, cancer relapse, and death. Risk of infertility was defined as exposure to at least one of the following three treatments: a cyclophosphamide equivalent dose (CED) greater than 4000 mg/m2 for males and 6000 mg/m2 for females, a carboplatin dose greater than 2000mg/m2, and whole abdominal irradiation in females (10.8Gy).
RESULTS
A total of 816 patients were included (53% female; mean age at diagnosis 3.7+/-2.6 years). Of these patients, 390 were exposed to radiation, 217 to an alkylating agent, and 65 to carboplatin chemotherapy. 169 patients received at least one gonadotoxic treatment at risk for infertility (169/816-21%), 27 patients received two, while 2 received all three. The most common gonadotoxic exposure was to alkylating agents (151/217-70%- received toxic CED) followed by abdominal irradiation in females (59/215-27%) and carboplatin chemotherapy (19/65-29%). Of the patients exposed to gonadotoxic treatments, 84 relapsed (19%) and 53 died (12%). However, 88% patients exposed to at least one gonadotoxic treatment survived.
CONCLUSIONS
In a national population-based cohort of patients with WT, 21% were exposed to at least one gonadotoxic treatment placing them at risk for infertility, and of those, 88% were survivors. The risk of infertility in children treated for WT may not be as low as previously thought given these exposures. Treatment with alkylating agents posed the highest risk of gonadotoxicity in our cohort. These findings should be discussed during counseling and raise the potential need for fertility preservation interventions.
18:02 - 18:04
S06-5 (CP)
Sadaf ABA UMER KODWAVWALA, Sajid SULTAN, Bashir AHMED, Philip G. RANSLEY and Adeeb Ul Hassan RIZVI
SINDH INSTITUTE OF UROLOGY AND TRANSPLANTATION, PHLIP G.RANSLEY DEPARTMENT OF PAEDIATRIC UROLOGY, Karachi, PAKISTAN
PURPOSE
Tuberous sclerosis is an autosomal dominant and multisystem genetic disorder.RCC is rarely associated with TSC in children.We report a case of TSC with RCC in a seven year old girl with bilateral renal involvement and its management with targeted therapy with mammalian target of Rapamycin inhibitor (mTOR inhibitor) “Everolimus”.
MATERIAL AND METHODS
Seven year old girl presented with hematuria and abdominal distention.History of seizures at 6 months of life.Examination findings:fascial angiofibroma,dental enamel pits,hypomelanotic macules,periungual fibroma and shagreen patch.Palpable mass on the left side of abdomen.CT scan showed bilateral heterogeneously enhancing soft tissue density renal masses (left larger than right)with multiple cysts in the periphery of right kidney and one small angiomyolipoma(AML) in segment III of liver.Multiple cortical and subcortical tubers and subependymal hamartomas are seen on MRI brain.
RESULTS
Diagnosis of TSC with Bilateral renal masses is established.Nephrectomy of a left non functiong kidney with huge mass was performed,which showed “papillary renal cell carcinoma”.Keeping in view the right solitary kidney with mass (of similar character as of left) where nephron-sparing surgery was not possible, long-term complications and parental concerns for hemodialysis and transplant, after discussion in MDT meeting and with family decided to start on targeted medical management with mTOR inhibitor, everolimus. Over 4 months, there was a 50% reduction in the right renal mass size. She has been on Tab. everolimus for the last 4.5 years, without any significant side effects. Right renal mass is stable in size, and Fascial angiofibroma has decreased.
CONCLUSIONS
In pediatric patients, the association between RCC and TSC is very rare and preserving the maximum renal tissues is preferred. The use of targeted medical therapy with mTOR inhibitor yielded significant clinical benefits evidenced by a reduction in right tumour size and other TSC manifestations.