Please use the arrow to scroll right for additional sessions
THE NEPHROMETRY SCORING SYSTEMS FOR NEPHRON SPARING SURGERY IN CHILDREN WITH RENAL TUMORS.
Abstract
Background and Aims
The surgical decision to attempt a nephron sparing surgery (NSS) in children with renal tumors can be difficult and dependent on the experience of the surgeon. In adults, nephrometric tools such as the RENAL, PADUA or RTII scoring systems can help. While low complexity tumors are often eligible to NSS (more than 90%), a high complexity tumor often requires radical nephrectomy (RN). We sought to evaluate the application of those nephrometric tools to the pediatric population.
Methods
In a retrospective monocentric study, we applied the RENAL, PADUA & RTII scores to the preoperative CT-scan or MRI in children undergoing surgery for renal tumors from October 2015 to October 2019.
Results
Forty-five tumors (13 NSS, 32 RN) were removed in 31 children (25 after neo-adjuvant chemotherapy following SIOP protocols and 6 without pre-operative chemotherapy): Wilms tumors (n=22, 4 bilateral), nephrogenic rests (one unilateral, one bilateral), mesoblastic nephroma (n=2), cystic nephroma (n=1), synovialosarcoma (n=1), clear cell sarcoma (n=1), rhabdoid tumor (n=1) and papillary carcinoma (n=1). The mean tumor length was 66.2 mm in RN and 26 mm in NSS groups (length ratio 60% smaller in NSS group; p < 0.001). Two thirds, 60% and 63.6% of the patients had NSS in the low complexity category of RENAL, PADUA and RTII respectively. Conversely, 10.5%, 16% and 9.5% in the high complexity groups of the RENAL, PADUA and RTII, respectively, underwent NSS.
Conclusions
The application of the RENAL, PADUA and RTII scoring systems was able to accurately classify most of the tumors undergoing radical or partial nephrectomy, according to their complexity. Nevertheless approximately one third of the patients in the low complexity groups still underwent radical nephrectomy although the scores are in favor of NSS. These scores question about how we could increase NSS indications in pediatric renal tumors.
SURGICAL VIOLATIONS AND THEIR IMPACT ON THE OUTCOME OF UNILATERAL WILMS TUMOR – A MULTICENTER REVIEW OF PRACTICE
Abstract
Background and Aims
Wilms tumor (WT) is a surgical neoplasm as per surgeons' view. This study aimed to assess the types and numbers of surgical violations (SV) performed during WT nephrectomy, and their influence on the prognosis.
Methods
Retrospective review of 112 patients with unilateral WT who presented to three tertiary Egyptian institutions between January 2015 and January 2020. All participating centers adopt children's oncology group protocol. Massive, initially unresectable tumors were managed by delayed surgery (after biopsy and chemotherapy). The SV were analyzed by chi-square test and logistic regression.
Results
Upfront nephrectomy was performed in 62 patients, whilst the remaining 50 underwent delayed resection. Thirty-three SV were practiced among 31 patients (22 during primary surgery and 11 during delayed surgery) with an overall incidence of 29.5%. Absence of lymph nodes sampling represented 78.8% (26/33) of all violations (fifteen in the primary surgery patients and eleven in the delayed surgery patients). Intraoperative tumor rupture and spillage accounted for 15.2% (5/33), and unnecessary resection of organs occurred in 6% (2/33). The two aforementioned SV happened during primary surgeries. There were no SV regarding incorrect abdominal incisions or unwarranted preoperative biopsies in any patient included. Follow-up to April 2020 showed that 14 patients had tumor relapses (6 local, 6 distant and two both). Three-year overall and event free survival for the entire cohort were 89% and 87%, respectively. The SV were not significantly correlated with both, mortality (p-value=0.326) and distant relapse (p-value=0.861). However, SV had a significant impact on local recurrence (p-value=0.003).
Conclusions
Surgical violations are significant predictors for local recurrence, and they were more frequent in the primary surgery patients. Failure of lymph nodes documentation was the main problem encountered in either primary or delayed surgery. Careful assessment of tumor resectability and adequate lymph nodes sampling can surely reduce the SV and improve the future practice.
THE STRUCTURE OF SURGICAL PROCEDURES FOR RENAL TUMOURS IN A MEMBER CENTRE OF THE EUROPEAN REFERENCE NETWORK
Abstract
Background and Aims
Despite an excellent general prognosis, some renal tumours (RT) remain at risk and require multidisciplinary team and high technology for cure. The “European Reference Network, Paediatric Cancer (ERNPaedCan)” aimed to create the network of centres having a potential to consult and treat them. Aim: to analyse the characteristics and results of surgery in children with RT in one of the ERNPaedCan centres.
Methods
Ninety six operations in 65 RT patients (2017-2019): 47 nephroblastoma, 3 nephroblastomatosis, 7 cystic nephromas, 3 mesoblastic nephromas, 3 renal cell carcinomas, 2 other. Eighteen were regional patients (28%), 47 transferred from other regions/countries (72%). Procedures: 10 USG-guided tru-cuts, 39 nephron sparing surgeries (NSS: unilateral/22, bilateral/17), 26 tumour-nephrectomies including 4 with high inferior vena cava (IVC) thrombus, 15 metastasectomies, 6 resections of the intraabdominal recurrences. Patients followed SIOP 2001 protocol.
Results
Of 10 tru-cuts, 4 revealed non-nephroblastoma malignancy (40% doubt-confirmation-rate). Of 23 operations in bilaterals, 6 were tumour-nephrectomies (26%), 17 (74%) NSS. Of 26 tumour-nephrectomies, 16 (61%) were unusual due to IVC thrombus/4 (by-pass needed/3), non-response to chemotherapy/4, pre-treatment rupture with persistent haematoma/2, other/6. Metastasectomies were performed on lungs/12, liver/3 and spleen/1. Resected intraabdominal recurrences were peritoneal/3 or tumour-bed/3. Four/65 patients (6%) relapsed (follow-up: 3 – 38 months). Comparing regional patients to those from outside the region/foreign, the procedure rates were respectively: NSS 17% vs. 55% (p=0.0058), IVC thrombectomy 5% vs. 6% (NS), metastasectomy 5% vs. 29% (p=0.0494), relapse surgery 0 vs. 13% (p=0.007). Major complications: 3 (3%) prolonged leak from the collective system after NSS. Outcome (4-37 months) 4/65 died (6%)
Conclusions
The disease extension in regional- and transferred patients differs. ERNPaedCan centres offer a good chance for cure with acceptable complications rate also for difficult cases. In authors’ opinion accurate indicators of surgical quality in RT of childhood are the rates of successful NSS, relapse resections and IVC thrombectomies and low complications rate.
PEDIATRIC NON-WILMS RENAL TUMORS: A CLINICOPATHOLOGICAL REVIEW OF SUBTYPES, DIAGNOSTIC DILEMMA AND SURVIVAL FROM A TERTIARY CARE CENTER
Abstract
Background and Aims
Pediatric Non-Wilms renal tumors (NWRT) are a heterogenous group of tumors and their pathological behavior and outcome are poorly understood because of their relative rarity. They always possess a diagnostic and therapeutic challenge. we planned a study to review the clinicopathologic behavior and outcome of pediatric NWRT.
Methods
Retrospective review of data all infant and children with Non-Wilms renal tumors from 2009 to 2019 were collected and analyzed.
Results
Of the 19 cases of pediatric non-Wilms renal tumors, the most common tumors were cystic nephroma (5), followed by mesoblastic nephroma (3), clear cell sarcoma (3), , Xp11.2 translocation renal cell carcinoma (TRCC) (3), primary primitive neuro-ectodermal tumor (PNET) of kidney (2), Rhabdoid tumor of Kidney (1), Xanthogranuloma (1) and a case of cystic partially differentiated neuroblastoma (CPDN) in a bilateral WT. Usual presentation is abdominal mass and hematuria. Twelve patients received upfront surgery and the rest seven had received neoadjuvant chemotherapy beacuse of diagnostic error and other reasons. Disease relapse was seen in two patient; one patient of PNET, who refused further treatment and lost to follow up and the one with clear cell sarcoma kidney who presented with lung metastasis and expired. The child with rhabdoid tumor expired 2 months after surgery while on chemotherapy because of febrile neutropenia and sepsis. All other children are in follow up with no recurrence.
Conclusions
Pediatric NWRT comprise around 25% of all renal mass in our study and the approach (upfront surgery/upfront chemotherapy) depends on the age at presentation and imaging finding. Simple hematoxyline & eosin stain, immunohistochemistry and the aid of cytogenetics are needed to confirm the diagnosis. The overall outcome in general is very good except for the case with advance stage renal PNET, CCSK and Malignant Rhabdoid Tumor of kidney which has a poor outcome.
NEPHRON SPARING SURGERY IN WILMS TUMORS WITH OBSTRUCTING BOTRYOID GROWTH PATTERN IN CHILDREN WITH BILATERAL DISEASE
Abstract
Background and Aims
Botryoid growth pattern (BGP), characterized by a polypoid mass growing into the renal pelvis may be observed in around 10% of Wilms tumors (WT). WT with large BGP may produce obstruction of the collecting system. BGP in bilateral WT is extremely rare, and represents a challenge for their surgical management. We report our experience of nephron sparing surgery (NSS) in bilateral WT with BGP.
Methods
In this retrospective monocentric study, we reviewed all cases of BGP in bilateral WT between January 1990 and January 2020.
Results
Among 35 patients with bilateral disease, three (8.5%) aged 6, 9 and 16 months were diagnosed with unilateral BGP before (n=1) or during (n=2) surgery. Two children presented a WT1 predisposition syndrome. The collecting system of the kidney with BGP was obstructed in 2 patients. After neo-adjuvant chemotherapy, the three BGP were operated on by NSS. A two-step surgery was performed with one tumorectomy, two inferior and central hemi-nephrectomies in the side with BGP and a partial (n=2) or total (n=1) nephrectomy on the contralateral kidney. BGP was easily removed with no adherences to the pelvic wall. The histology of the tumor with BGP was intermediate risk (stromal type n=2, mixte type n=1), stage I (n=2) and stage II (n=1).The histology of the BGP was Intra Lobar Nephrogenic Rest (ILNR) in all the three cases. No postoperative complications occurred despite calyx opening. One patient had an early hepatic relapse treated with chemotherapy and surgery. At a follow up of 4 months, 22 and 23 years, all patients are alive, with a moderate renal insufficiency and hypertension.
Conclusions
NSS appeared safe and possible in bilateral WT with BGP, which allowed parenchyma preservation. In our series, BGP corresponds to ILNR in all cases.
SURGICAL STRATAGIES IN THE MANAGEMENT OF WILMS TUMOR IN CHILDREN WITH BILATERAL NEPHROBLASTOMATOSIS: A TEN-YEAR-EXPERIENCE
Abstract
Background and Aims
Nephroblastomatosis (NBL) is an uncommon pathologic process characterized by the presence of persistent embryonic nephrogenic rests. Progression to Wilms tumor (WT) occurs in an estimated 35% of patients. Despite the advances in WT management, the treatment of WT with bilateral NBL is still challenging.
Methods
We conducted a retrospective analysis of patients with WT and bilateral NBL held in the unit of pediatric oncology and the department of pediatric surgery of the Children's hospital of Tunis, during a 10 years’ period between January 2009 and December 2018.
Results
5 girls and 4 boys were treated. The mean age of our patients was 2.5 years (9months-5years). Initial ultrasonographic and computed tomographic assessments demonstrated synchronous Bilateral WT in 6 cases and unilateral WT in 2 case. One patient had tumor progression during monitoring. All of our patients had primary chemotherapy according to SIOP. Surgery consisted in total nephrectomy in 5 cases with nephron sparing surgery in three cases for bilateral tumor. For the four other cases a nephron sparing surgery was indicated (6/8). Operated kidneys revealed pathologic evidence of NBL in 4 cases, perilobar nephrogenic rests in 3 cases, and two with insufficient information. Histological examination revealed an intermediate risk in 7 cases and a high risk in 2 patients who had local recurrence after total remission. A total nephrectomy was performed in one case and a partial nephrectomy in the other case with a chemotherapy regimen and radiation. All patients are currently alive and free of disease, except one patient, with a median follow-up of 4.5 (range 1.5-9) years.
Conclusions
The major clinical challenge in WTs with bilateral NBL is preservation of functioning renal tissue while achieving cure with minimum therapy-related morbidity. With Both appropriate pre- and post-operative chemotherapy and skilled surgical techniques, an excellent outcome can be achieved in most cases.