Management of WT in developing countries provides well-known challenges such as late presentations particularly in malnourished children, failure or abandonment of therapy, insufficient capacity of specialized hospitals, and deficiency in treatment facilities [11, 12]. Some experts analyzed that primary surgery might be unsuitable to be practiced in resource-constrained settings due to the aforementioned problems; therefore, delayed resection should be the principal modality . The current study has special considerations as being carried out in a resource-limited society where patients presented with huge masses due to delayed diagnosis; meanwhile, surgeons adopt upfront nephrectomy and COG guidelines.
Median age at presentation in this study was found to be nearly the same compared to other reports from North Africa, Asia, and Europe [7, 14, 15], and slightly lower than that reported by a North American study . Gender distribution among our cases was in favor of males and the same result was documented in an Asian study , in contrast with Western data . Palpable abdominal mass was the commonest presentation in this study and the same findings were observed in other studies conducted at developing countries [7, 14], while other complaints in addition to palpable mass were reported in African patients due to a more advanced disease . In contrast, there were earlier referrals in affluent societies, and even there were differences between them regarding the percentage of cases discovered incidentally. Pritchard-Jones et al. observed that a lesser proportion of patients in the UK were diagnosed incidentally when compared to Germany .
The percentage of metastatic disease was 10.8% in this study, and this was similar to an Indian study . Interestingly, this incidence was lesser when compared to several studies at other developing countries ranging between 14 and 30.5% including one of them also reported in India [14, 20,21,22]. Such difference could be due to the number of patients, duration of series, and referral bias and might be that locally advanced tumors were more than the metastatic disease as in our cohort.
The majority of patients in this study were managed by primary surgery. Although the aforementioned difficulties of selecting such protocol in our environment, it was positive to observe that intraoperative complications were only due to tumor ruptures, and there were no other operative morbidity or mortality. However, relapse related to rupture was a cause of postoperative death in one patient. It is also very interesting to notice that some centers in developing countries adopt the International Society of Paediatric Oncology (SIOP) protocol [7, 14, 22] in order to overcome the delayed presentations, whereas others depend on COG recommendations [20, 23, 24].
The participating centers in this study prefer COG guidelines to avoid administration of preoperative chemotherapy to non-WT or benign disease . Additionally, there is an evident psychological factor of upfront nephrectomy on families in our community and their comfort towards surgery as a primary step. Relying on the SIOP protocol needs specialized radiologists and pathologists to avoid imaging misdiagnosis or understaging due to change of tumor histology, whereas COG protocol exposes patients to more abdominal radiation due to more tumor rupture . Eventually, the end results are very similar between both approaches , and every center whatever its location can choose according to the experiences.
The survival rates were reported above 90% in high-income countries . In this study, such data was better than the declared in a previous national series [OS 78.9%] . Meanwhile, our results were within reasonable range among those reported from other developing countries in North Africa and Asia [OS 74–89%, EFS 73–86%] [7, 14, 19, 20]. Impressive outcomes were achieved by a study in Latin America with OS and EFS of 91% and 85%, respectively . While, dismal survival rates were observed in sub-Saharan Africa of 25–46% [17, 21].
Improvements in the management could be achieved by adherence to the evidence-based guidelines and avoidance of violations. Due to the crucial role of surgeons in complete resection, staging, and avoiding tumor rupture, recommended surgical guidelines must be adopted in order to optimize outcomes . Despite the high percentage of violations practied in this study, the majority of them lack or fault in lymph node management, and this was similar when compared to a recent COG study reporting that 65% of all violations were absence of lymph node sampling . Furthermore, the same problem was declared in an SIOP study, which documented that incorrect sampling occurred in 88.2% of patients . The minimum of seven nodes sampled is crucial for detecting metastases by COG , and it is of six nodes by SIOP, which formerly reported that only three nodes were sufficient [27, 28]. Such violation remains the commonest mistake made by surgeons everywhere, and its consequences lead to less aggressive adjuvant therapy and high risk of recurrence. However, it was not correlated with relapse in our series and also had no impact on EFS by a COG report .
Tumor rupture was documented as a predictor for recurrence as per NWTSG , and we observed the same with a significant difference in this study. Intraoperative tumor rupture in this cohort was similar to that reported by a COG report (11.5%) , whereas it was higher than the declared by an SIOP study (1.45%) . This notable difference is due to adoption of delayed surgery by SIOP and the role of preoperative chemotherapy in making tumors more solid and downsized. Biopsy due to an equivocal initial diagnosis with other neoplasms, such as nueroblastoma or lymphoma, was not considered as a violation in the current protocol . Thus, this study had minor violations of unwarranted preoperative biopsy. We also did not report any violation regarding surgical incisions or extensive organs resection. Finally, The authors of this study propose that careful assessment of resectability in multidisciplinary team meetings and adequate lymph node sampling can surely reduce the occurrence of violations in the future.
Several limitations were observed in this study as its retrospective nature, small sample size, and few numbers of participating centers with short-term results. The last drawback might be due to lacking of registration systems in our nation and the authors invited other centers to share their experience; however, data loss was the main obstacle. We believe that a further study with more number of patients is warranted for more confirmation.