ESPU Meeting on Friday 26, April 2019, 11:14 - 12:00
Sarah GARNIER 1, Julie VENDRELL 2, Bernard BOILLOT 3, Gilles KARSENTY 4, Jean Michel GUYS 5, Thomas BLANC 6, Stephen LORTAT JACOB 7, Laurent SOUSTELLE 8, Veronique PHE 9, Alexia EVEN 10, Emmanuel CHARTIER KASSLER 11, Pierre COSTA 12, Francois IBORRA 13, Ourdia BOUALI 14, Xavier GAME 15, Jerome SOLASSOL 16 and Nicolas KALFA 17
1) CHU Montpellier Hopital Lapeyronie, Service de Chirurgie et Urologie Pédiatrique, Montpellier, FRANCE - 2) CHU Montpellier, Laboratoire de Biologie des Tumeurs Solides, Montpellier, FRANCE - 3) CHU de Grenoble, , France, Service d'urologie, La Tronche, FRANCE - 4) CHU Hôpital la Conception, APHM, Service d'Urologie et de Transplantation Rénale, Marseillle, FRANCE - 5) CHU Hôpital Timone,, APHM, Service de Chirurgie Pédiatrique, Marseille, FRANCE - 6) Hôpital Necker-Enfants-Malades, APHP, Service de Chirurgie Pédiatrique, Paris, FRANCE - 7) Hôpital Necker-Enfants-Malades, Paris, FRANCE - 8) Hôpital Universitaire Carémeau, Service d'urologie, Nîmes Cédex 9, FRANCE - 9) Hôpital universitaire La Pitié Salpêtrière, Service urologie, Paris Cedex 13, FRANCE - 10) Hôpital Raymond-Poincaré, Service de médecine physique et réadaptation,, Garches, FRANCE - 11) Hôpital universitaire La Pitié Salpêtrière, Paris Cedex 13, FRANCE - 12) Hôpital Universitaire Carémeau, Nîmes Cédex 9, FRANCE - 13) CHU Montpellier, Service d'Urologie et de Transplantation Rénale, Montpellier, FRANCE - 14) Hôpital des enfants de Toulouse, Service de chirurgie pédiatrique,, Toulouse Cedex 9, FRANCE - 15) CHU Toulouse, Urologie, andrologie et transplantation rénale, Toulouse Cedex 9, FRANCE - 16) CHU Montpellier, Montpellier, FRANCE - 17) CHU Montpellier, Service de Chirurgie et Urologie Pédiatrique, Montpellier, FRANCE
Malignancy after augmentation enterocystoplasty (AE) is often diagnosed at an advanced stage with a high mortality. Comprehensive characterization of mutations in key cancer pathways may be of interest for the development of personalized treatment.
MATERIAL AND METHODS
A multicenter nationwide retrospective study included 14 patients. From our cohort, 11 tumor samples were retrospectively analyzed using a Next-Generation-Sequencing assay specifically dedicated to the detection of clinically actionable somatic alterations of 21 cancer genes.
Over half of analyzable samples (n=5/9) harbored missense mutations in known oncodriver genes. Two samples exhibited mutations in KIT and PDGFRA and one sample in KRAS hotspot. Two samples showed an alteration in the exon 3 of CTNNB1. One tumor that harbored a CTNNB1 mutation, also exhibits a concomitant ERBB4 alteration.
|Histology of the tumor||Cancer genes Alterations detected||Initial stage of disease / outcome|
|UCC||Not analyzable||localized/disease free|
|UCC||wild-type||N+/ disease free|
|SC||Missing sample||localized/ recurrence and death|
|AC||Not analyzable||M+/recurrence and death|
|AC||Missing sample||M+/ death|
|UCC||Missing sample||N+/ death|
|UCC||PDGFRA||N+/ recurrence and death|
|AC||KRAS||M+/recurrence and death|
|SC||wild-type||M+/recurrence and death|
UCC: urothelial-cell carcinoma, SC: squamous-cell carcinoma, AC: adenocarcinoma
M+:distant metastasis, N+:lymph-nodes involvement
This is the first report of mutations of cancer genes in malignancy after AE, for witch target therapies are available or under development.
Fadi ZUBI 1, Mandy RICKARD 1, Mohammed BEAITI 1, Nathan KAHN 1, Anne-Sophie BLAIS 1, Jessica HANNICK 1, Roberto IGLESIAS LOPES 2 and Armando J. LORENZO 1
1) The Hospital for Sick Children, Urology, Toronto, CANADA - 2) Hospital das Clínicas, University of São Paulo, Pediatric Urology Unit, Division of Urology, São Paulo, BRAZIL
Testicular Leydig cell tumor (LCT) is a rare disease in children, with paucity of information related to its natural history and most appropriate management. Herein we review our single institution experience and management of a cohort of children with testicular LCT.
MATERIAL AND METHODS
We reviewed all children presenting with testicular lesions between 2003-2017 (n=68), and excluded patients with alternative pathologies (n=57). We subsequently collected data on the following variables: age at surgery, laterality, type of surgery, operative time, presenting symptoms, serum markers, imaging findings, frozen section and margins, final pathology and follow-up time.
During the study period, a total of 11 children were treated for LCT of the testis. Age at surgery was 8.4 ± 1.6 years and the majority (82%) presented with unilateral disease. Most patients presented with a testicular mass and only 3 (27%) presented with testicular pain. None of the patients were found to have gynecomastia or elevated tumor markers. The primary method of management was ultrasound-guided testicular sparing surgery (82%); operative time was 90.2± 58.8 minutes. Mean tumor size was 27 ± 30 mm and of nine patients who had testis sparing surgery (TSS), two patients had final pathology prompting radical orchidectomy without residual disease encountered. At a follow-up time of 41± 31 months none of the patients had disease relapse.
To our knowledge, this is the largest reported series of pediatric LCT. Our data suggests that LCT in children is associated with a good prognosis and that TSS is a reasonable surgical approach with no obvious detrimental effects to perioperative morbidity or long-term outcomes. This is particularly true when tumor markers are negative. Moreover, pathological diagnosis after TSS should not prompt completion orchidectomy. These results may helpful during family counseling with respect to prognosis and expected outcomes.
Conca MA 1, Romeu G 1, Fernández J.M. 2, March JO 1, Polo A 1, Agustín SERRANO-DURBÁ 1 and Domínguez C 1
1) La Fe Universitarian Hospital, Pediatric Urology, Valencia, SPAIN - 2) La Fe Universitarian Hospital, Pediatric Oncology Unit, Valencia, SPAIN
Testicular relapse ofacute lymphoblastic leukemia (ALL)although it is very scarce is one of the extramedullary "sanctuaries". We try to clarify its true incidence with new protocols and evaluate the management of ALL patients with testicular relapse.
MATERIAL AND METHODS
Prospective study (2008-2018) of 334 boys afected of ALL. Review of the Spanish Society of Pediatric Hematology and Oncology-Program for the Study of Therapeutics Malignant Hematology(SEHOP-PETHEMA) protocol.
Only three cases (0,89% incidence) of testicular relapse were diagnosed.
Case 1: 3-years-old, right testicular relapse after 3 years. Bilateral biopsy confirmed right infiltration, treated by high dose radiotherapy(RT).
Case 2: 4-years-old, right testicular relapse after 2 years. Right orchiectomy + contralateral biopsy was performed, unilateral testicular relapse was confirmed. He is undergoing treatment with consolidation chemotherapy(CH), pending RT.
Case 3: 9-years-old, early bone marrow relapse and suspicious testicular asymmetry. Bilateral biopsy was performed, testicular relapse was not confirmed.
According SEHOP-PETHEMA protocol, testicular involvement at ALL diagnosis does not require anatomopathological confirmation(AP). Testicular biopsy will be performed when ultrasound involvement persists after consolidation CH. If confirmed, treatment will be bilateral RT. In testicular relapse, it will always be necessary to confirm AP and rule out contralateral subclinical infiltration, treatment includes orchiectomy + contralateral biopsy + low dose RT or bilateral biopsy + full dose RT, with CH.
Therapeutic combination described in our cases supposes an important multidisciplinary task. Collaboration and knowledge by different specialties is mandatory to offer the patient optimal treatment.
Kristina DZHUMA 1, Huber DUCOU LE POINTE 2, Aurore COULOMB 3 and Sabine IRTAN 4
1) APHP Hôpital Armand Trousseau, Visceral Pediatric Surgery,, Paris, FRANCE - 2) APHP Hôpital Armand Trousseau, Pediatric Radiology, Paris, FRANCE - 3) Sorbonne Universités, UPMC Univ Paris 06, Centre de Recherche St Antoine Inserm UMRS.938 - APHP Hôpital Armand Trousseau, Pediatric anatomopathology, Paris, FRANCE - 4) Sorbonne Universités, UPMC Univ Paris 06, Centre de Recherche St Antoine Inserm UMRS.938 - APHP Hôpital Armand Trousseau, Visceral Pediatric Surgery, Paris, FRANCE
Despite typical imaging features described for Wilms tumors (WT), nephroblastomatosis and nephrogenic rests (NR), the radiologic assessment is not always congruent with the final histology. So, the aim of this study was to compare the imaging features of renal nodules with their final histology and identify radiological factors that would better differentiate WT from NR.
MATERIAL AND METHODS
Patients with multifocal bilateral or unilateral renal tumors with histologically confirmed NR or nephroblastomatosis were included. A senior radiologist blinded to histology reviewed CT images at diagnosis and after neoadjuvant chemotherapy. The difference in findings between the initial cross-sectional imaging at diagnosis and preoperative phase and the histopathology results was noted and correlations were made.
Among 205 nodules identified in 48 patients (mean 4.3 nodules/patient) at diagnosis, 175 were resected after a median of five courses of chemotherapy for the first side and 13 courses for the second side. The radiology/pathology correlation showed 118 (74,6%) nodules adequately classified, 20 (11,3%) false positive (9 patients) and 20 (11,3%) false negative (9 patients) for WT. WT was significantly bigger than other tumor types at diagnosis and preoperative assessment (p<0.0001). The homogeneity rate was not different between WT and NR and 95% of nodules thought NR that were finally WT were homogeneous while 83% of nodules initially diagnosed as WT that were finally NR were heterogeneous.
Radiological misdiagnosis occurred in 25% of kidney nodules in bilateral renal tumors. The size of nodules at diagnosis and their decrease under neoadjuvant chemotherapy may be an interesting factor to help recognizing WT from NR before surgery. On the contrary, other criteria like homogeneity did not seem key features.
Armando LORENZO 1, Mandy RICKARD 1, Anne-Sophie BLAIS 1, Nicolas FERNANDEZ 1, Asfaneh AMIRABADI 2, Wayne LEE 2, Ahmed SHALABI 2 and Jeffery TRAUBICI 3
1) The Hospital for Sick Children, Division of Urology, Toronto, CANADA - 2) The Hospital for Sick Children, Toronto, CANADA - 3) The Hospital for Sick Children, Radiology, Toronto, CANADA
Advances in the management of Wilms’ tumor (WT) have led to changes in management and increased survival. To date, quantification of tumor/parenchyma volume remains cumbersome without standardization. Herein, we present our initial experience and proof-of-concept with automatic image recognition/segmentation technology for calculation of tumor/parenchyma volume in WT patients.
MATERIAL AND METHODS
We reviewed WT patients between 2000-18, capturing CTscan images at baseline, post-neoadjuvant chemotherapy (NaC;if applicable) and post-operatively. Images were uploaded into MATLAB-3-D volumetric image processing software. The algorithm was developed at our institution and trained by 2 clinicians who supervised the demarcation for tumor and parenchyma, recognition/delineation of tumor margins while differentiating it from uninvolved parenchyma and autocalculation of volume for both on serial imaging.
During the study period 98 patients were identified. Of these 32 (38moieties) were selected for the pilot/proof-of-concept. Most patients (65%) were female, age 50+37 months and 80% had unilateral disease. NaC was employed in 64%. Surgical management included 27-radical and 11-partial nephrectomies. Tumors measured 9x7x6cm and weighed 433+/-352g. Mean gain in parenchyma was 15-38% from baseline to postoperative studies and tumor volume decreased by 60-68% from baseline to post NaC (Table 1.)
|Baseline Volume (cm3)||Post-NaC Volume (cm3)||Post-operative Volume (cm3)||Change (%)|
Machine driven, semi-independent volumetric analysis is feasible, and allows analysis of tumor and parenchyma volume in response to chemotherapy and surgery. Our data shows changes after therapy that may be otherwise difficult to quantify. Utilizing such technology may enhance surgical planning, monitoring response to treatment, and predict long-term changes in renal function.
Tessi CATALINA 1, Lopez Imizcoz FELICITAS 2, Ruiz JAVIER 3, Weller SANTIAGO 3, Corbetta JUAN PABLO 2, Villoldo GUSTAVO 2, Sager CRISTIAN 2, Burek CAROL 2, Lopez JUAN CARLOS 2 and Lopez JUAN CARLOS 2
1) GARRAHAN HOSPITAL BUENOS AIRES ARGENTINA, UROLOGY, Ciudad Autonoma De Buenos Aires, ARGENTINA - 2) GARRAHAN HOSPITAL BUENOS AIRES ARGENTINA, UROLOGY, Ciudad Autonoma De Buenos Aires, ARGENTINA - 3) GARRAHAN HOSPITAL BUENOS AIRES ARGENTINA, Ciudad Autonoma De Buenos Aires, ARGENTINA
Reninoma is a yuxtaglomerular cell tumor and a rare but treatable cause of hypertension. This type of benign renal neoplasm has elevated plasma renin activity with secondary hyperaldosteronism and it is usually well localizated using computed tomography or magnetic resonance imaging. The first choice of treatment consists in the surgical resection of the tumor with a nephron-sparing approach.
MATERIAL AND METHODS
Report a laparoscopic resection of a reninoma tumor in a pediatric patient
A well circumscribed 20 mm diameter tumor was discovered with ultrasound and then confirmed with computed tomography images in the lower pole of the right kidney of a 14-year-old girl with a history of severe high blood pressure.
The patient was positioned laterally with sand bag underneath the lumbar region, and the table was put in a reverse Trendelenburg position. A transperitoneal laparoscopic approach was performed using 4 ports. After mobilization of the colon, the Gerota’s fascia was opened and the tumor located on the anterior surface of the lower renal pole. Both polar and principal renal arteries were dissected and secured with vessel loops. The tumor was resected using an ultrasonic energy device with an 8-minute polar artery clamping. The transected renal surface was closed using 2 continuous barbed sutures and the tumor was removed using a handmade endobag. There were no perioperative complications and a perianastomotic drainage was left for 4 days.
The patient was discharged 2 days after surgery with decreasing blood pressure measurements and normal plasma renin activity levels without any medication. The surgical pathology confirmed the typical reninoma immunohistochemically features.
Reninoma is a rare but benign renal tumor and because of its nature and localization it represents an ideal tumor for minimally invasive nephron-sparing surgery.
Thomas BLANC 1, Luca PIO 1, Pierre MEIGNAN 1, Jules KOHAUT 1, Daniel ORBACH 2, Véronique MINARD 3, Yves HELOURY 1 and Sabine SARNACKI 1
1) Hôpital Necker - Enfants Malades, Department of Pediatric Surgery and Urology, Paris, FRANCE - 2) Institut Curie, Centre oncologie SIREDO (soins, innovation, recherche autour des tumeurs de l'enfant, l'adolescent), Paris, FRANCE - 3) Institut Gustave-Roussy, Département de cancérologie de l'enfant et l'adolescent,, Villejuif, FRANCE
The role of MIS for renal tumour treatment in children has been limited to pioneer groups. Our aim is to report a retroperitoneal robotic approach for nephron-sparing resection of renal tumours.
MATERIAL AND METHODS
Children with Wilms’ tumour were treated according to the SIOP-2001 protocol.
A four-trocar retroperitoneal lateral approach was used. The renal artery was dissected and clamped with bulldog. Direct tumour manipulation was carefully avoided. The tumour was excised with scissors, leaving a margin of normal renal parenchyma around it. The sliding-clip renorrhaphy technique was used to close the cone-shaped defect with running suture incorporating a small piece of Surgicel Fibrillar. The clamp was removed, and haemostasis was confirmed.
Intraoperative frozen sections to assess the surgical margins were not performed.
The tumour was retrieved in an EndoCatch without morcellation.
4 children, aged 3.2-4.7-9.8-14.1 years, underwent RRALPN. Mean weight was 32 kg (15-50)
The 3 younger patients developed a renal tumour on the solitary kidney after contralateral Wilms tumour. The eldest one had undergone multiple laparotomies including liver transplantation for hepatoblastoma. During the work up for a new transplantation, a tumour was found on the left kidney.
Console time was 2 hours. Warm ischemia time reduced from 40 to 26 minutes.
The tumour was completely removed without rupture. No bleeding occurred.
Three patients had a Wilms stage 1 (2.5-2.5 and 3.5 cm), one a 3.5 cm tubulopapillary carcinoma.
The postoperative course was free of complications, all the patients were discharged on day-2 to day-4.
No recurrences or long-term complications have been detected (follow-up:1–12 months).
RRALPN for renal tumour is a feasible and safe procedure in a selected group of children. This approach follows the current aim to decrease burden of treatment in children cancer and has the advantage to avoid peritoneal spread in case of tumour rupture.