Wilms tumor in childhood: Single centre retrospective study from the National Institute of Oncology of Rabat and literature review

Background: Wilms tumor is a very common renal malignancy in children. Prognosis has been improved dramatically during the last few decades because of multimodal treatment and successful sequential studies. Through a retrospective study conducted in the National Institute of Oncology of Rabat, concerning children with Wilms tumor treated following the International Society of Pediatric Oncology protocol (93-01) between 2005 and 2010, we report the experience of our institute in treatment of this malignancy. We analyze also the clinicopathologic and therapeutic aspects impacting the outcome results and compared to literature data. Results: Fifty-two patients with Wilms tumor treated in the department of radiotherapy after receiving chemotherapy and surgery at the department of hemato-oncology in children hospital of Rabat were enrolled. The main characteristic was the high prevalence of locally advanced and metastatic stages (32.6% of stage IV). With a median follow up of 54.8 months [20–79], we observed a complete response in 32 cases (61.5%), local recurrence in only one case (1.9%), metastatic relapse in 3 cases (5.8%), both local and metastatic recurrence in 3 cases (5.8%) and disease progression in 8 cases (15.4%). The mean duration of overall survival was 91.2 months. The estimated 2-year and 5-year overall survival were 78.7% and 70.1% and for metastatic patients 68.8% and 62.5% respectively. At univariate analysis several parameters were tested for survival, but only age, anaplasia, lymph node involvement, type of metastasis and response to treatment were found to significantly impact the overall survival. Outcome was better for localized tumors (stage I, II and III) compared with disseminated tumors (stage IV and V) combined. Also a better survival rate was found in the low and intermediate risk group compared to high risk, but not statistically significant. Conclusion: The relatively low outcome found in this series compared to literature can be mainly explained by the higher prevalence of metastatic disease compared to other series, but also by diagnosis and therapeutic delay, more likely because of bad socioeconomic conditions and lack of coordination between different operators. However, our results are nevertheless comparable to maghrebian series. Our department has established many procedures for improving the outcome and further studies are necessary to evaluate their efficiency

Neuroblastoma in an adult: a case report of a rare entity with a summary review

Neuroblastoma is a very uncommon neoplasm in adulthood. It occurs almost exclusively in children younger than 10 years (More than 90% of cases). We report the case of a healthy 40-year-old male who complained of a right flank pain in 2010. Clinical examination revealed an enormous abdominal mass involving the adrenal gland as showed at abdominal CT scan. The patient underwent a surgical en-bloc excision of the right adrenal mass, after a laborious dissection of the tumor. Histological and immunohistochemical findings were suggestive of a neuroblastoma. Adjuvant treatment was not indicated since the complete gross excision of the tumor was performed, and subsequently the patient was considered stage 1 disease and because of the unavailability of MYCN status, necessary for achieving risk group stratification and a risk-adapted strategy. The patient remained in good local control, until January 2015 when he accused a locoregional and metastatic relapse consisting of two new para-renal masses and magma of celiac adenomegalies extending into the mediastinum. As the mass was considered unresectable, therapeutic decision of administrating neoadjuvant chemotherapy, followed by local treatment (surgery and / or radiotherapy) based on tumor response was taken. Given the bad tumor response to systemic treatment, a palliative radiotherapy was instaured. Currently the patient is two months after the end of radiation with mild clinical improvement. The purpose of presenting this case is not only to report an uncommon malignancy in adulthood, but also to raise awareness among clinicians adding this clinical entity as a differential diagnosis when a retroperitoneal mass is identified. We also conducted a literature review to enhance clinicians’ acknowledgment about the management of this rare entity in adults