Surgery in Bilateral Wilms Tumor-A Single-Center Experience.

The treatment of bilateral Wilms tumors (BWT) involves curing the cancer, preserving long-term renal function, and maintaining a good quality of life. Established methods for achieving these goals include preoperative chemotherapy and nephron-sparing surgery (NSS). This study aimed to evaluate the experience of a single institution in treating patients with BWT. We analyzed cases of BWT treated at the Pediatric Oncology Institute-GRAACC-Federal University of São Paulo over a period of 35 years. Bleeding control was performed with manual compression of the renal parenchyma. Thirty-three patients were included in the study. Thirty cases were synchronous tumors. The mean age at diagnosis was 30.4 months (±22 m) and 66.7% were girls. The median follow-up period was 83 months. Neoadjuvant chemotherapy was the primary approach in most patients (87.9%), with a simultaneous upfront surgical approach performed in 84.8%. Most patients underwent bilateral NSS (70.4%). There were no early complications in this series, but 39.4% had clinical complications. The five-year survival rate was 76%. Therefore, it is clear that the surgical approach to BWT plays a crucial role in achieving good outcomes. However, it is difficult to standardize surgical techniques and technology may have the potential to enhance safety.

Hemophagocytic lymphohistiocytosis: presentation and outcome of twenty-one patients at a single institution

ABSTRACT Introduction: Hemophagocytic lymphohistiocytosis comprises a systemic hyperactivation of macrophages that requires prompt recognition of symptoms and early treatment. Objective and Method: In this context, we described clinical and laboratory characteristics, therapeutic modality and outcome of 21 patients with HLH treated at a pediatric oncology hospital between January 2000 and February 2019. Results: HLH mainly affected females, fever was the most frequent clinical sign and hyperferritinemia was the most prevalent laboratory abnormality. All patients were admitted to the intensive care unit (ICU) at some point. Fifteen (71.4%) patients presented resolution criteria and eight (53.3%) of them presented reactivation. The mortality rate was 57.1% and the mean time between diagnosis and death was 9.98 months. The 5-year overall survival (OS) was 36.7%. We observed a significant difference in prognosis associated with reactivation of HLH. These patients demonstrated an estimated 5-year OS of 25%, while all patients that did not reactivate were alive until the end of the follow-up. Conclusion: In conclusion, HLH is a rare disease with a high mortality rate, especially in patients with disease reactivation and those with familial- or immunodeficiency-associated forms, which makes early recognition and genetic testing crucial for appropriate management and prompt SCT indication.

Preserved executive functioning and low stress symptoms in children treated for acute lymphoblastic leukemia.

Pediatric cancer treatment can negatively impact cognitive and psychosocial development, although it has been suggested that these adverse effects may be minimized when children have higher resilience and better executive functioning. We aimed to evaluate the impact of pediatric Acute Lymphoblastic Leukemia (ALL) treatment on executive function, resilience and stress in survivors and to investigate correlations between executive functioning and resilience and between executive functioning and stress. The neuropsychological assessment was performed in 32 ALL survivors aged 7-17 years and 28 age-, sex- and socioeconomic status matched controls. Executive functioning was assessed by inhibitory control, mental flexibility and working memory tasks. Children's self-report scales were used to assess stress symptoms and resilience. Results revealed no executive function impairment nor stress symptom differences between ALL survivors and control group. In the ALL group, executive function and resilience were positively correlated, whereas executive function and stress were negatively correlated. We concluded that ALL treatment was not associated with impairment in executive functioning nor to increased stress symptoms in our sample. ALL survivors with better performance in mental flexibility and inhibition tasks reported fewer stress symptoms and more resilience, indicating a possible relationship between these variables.

Hemophagocytic lymphohistiocytosis: presentation and outcome of twenty-one patients at a single institution.

INTRODUCTION: Hemophagocytic lymphohistiocytosis comprises a systemic hyperactivation of macrophages that requires prompt recognition of symptoms and early treatment. OBJECTIVE AND METHOD: In this context, we described clinical and laboratory characteristics, therapeutic modality and outcome of 21 patients with HLH treated at a pediatric oncology hospital between January 2000 and February 2019. RESULTS: HLH mainly affected females, fever was the most frequent clinical sign and hyperferritinemia was the most prevalent laboratory abnormality. All patients were admitted to the intensive care unit (ICU) at some point. Fifteen (71.4%) patients presented resolution criteria and eight (53.3%) of them presented reactivation. The mortality rate was 57.1% and the mean time between diagnosis and death was 9.98 months. The 5-year overall survival (OS) was 36.7%. We observed a significant difference in prognosis associated with reactivation of HLH. These patients demonstrated an estimated 5-year OS of 25%, while all patients that did not reactivate were alive until the end of the follow-up. CONCLUSION: In conclusion, HLH is a rare disease with a high mortality rate, especially in patients with disease reactivation and those with familial- or immunodeficiency-associated forms, which makes early recognition and genetic testing crucial for appropriate management and prompt SCT indication.

Diagnostic Errors in Wilms' Tumors: Learning From Our Mistakes.

Aim: This study aimed to analyze clinical characteristics and image findings in patients initially diagnosed with renal masses and treated on the Société Internationale d'Oncologie Pédiatrique (SIOP) 2001 protocol for Wilms tumor (WT) that eventually were diagnosed with different pathologies. Methods: We reviewed the preoperative symptoms, laboratory tests, and images of patients who were initially treated for WT and proved to have other diagnoses. Data from these patients were compared to those of the last 10 patients with WT and the last 10 patients with neuroblastoma (NBL) treated at a single institution. Results: From June 2001 to December 2020, we treated 299 patients with NBL and 194 with WT. Five patients treated with preoperative chemotherapy for WT were postoperatively diagnosed with NBL (one patient had bilateral renal masses and one with multifocal xanthogranulomatous pyelonephritis). Three underwent nephrectomy, two biopsies only, and one adrenalectomy due to intraoperative characteristics. Regarding clinical presentation, abdominal mass or swelling was very suggestive of WT (p = 0.011); pain, although very prevalent in the study group (67%), was not statistically significant, as well as intratumoral calcifications on computed tomography (CT) (67%). Urinary catecholamines were elevated in all patients mistreated for WT with the exception of the patient with pyelonephritis in which it was not collected. Conclusion: Some pathologies can be misdiagnosed as WT, especially when they present unspecified symptoms and dubious images. Diagnostic accuracy was 98.1%, which highlights the quality of the multidisciplinary team. Abdominal mass or swelling is highly suggestive of WT, especially in the absence of intratumoral calcifications on CT. If possible, urinary catecholamines should be collected at presentation as they help in the differential diagnosis of NBL.

Management of Pediatric Tumors With Vascular Extension.

Background: Pediatric tumors can present with vascular extension to the inferior vena cava and right atrium, which impacts the surgical strategy and can be challenging during surgical treatment. Wilms tumor (WT) is the most common retroperitoneal tumor that can present with vascular extension, but also adrenal tumors, clear cell tumors from the kidney, and hepatoblastomas can present with this situation. Surgical aims include obtaining complete tumor resection without risk for patients, to avoid severe bleeding, cardiac arrest, and embolization, and to avoid cardiac bypass if possible. Objective: To describe and discuss the surgical strategies to deal with pediatric tumors with vascular extension and propose a protocol. Method: Retrospectivly review the experience of treating patients with vascular extension in a single institution, describing different scenarios and a decision making fluxogram based on the preoperative evaluation regarding the surgical techniques and the need for cardiac bypass that are adequate for each situation. Image studies are important to guide the surgical strategy. Depending on the quality of image available, computerized tomography (CT) or magnetic resonance imaging (MRI) can be enough to give the information needed for surgical decisions. Ultrasonography (US) with Doppler is helpful to confirm diagnosis and describes factors to guide the adequate surgical strategy, like the upper level extension and presence or absence of blood flow around the thrombus. Neoadjuvant chemotherapy is indicated in most cases, in order to reduce the upper level of extension (and avoid the need for cardiac bypass) and to lower the risk of embolization. The approach is based on the upper level of the thrombus and can include cavotomy or cavectomy, sometimes with cardiac bypass and cardiac arrest with hypothermia, when the thrombus reaches the diaphragmatic level or above. Pathology analysis of the thrombus can guide staging and the need for radiotherapy postoperatively. Results: A decision making fluxogram protocol is presented focusing on the surgical treatment of such condition. Conclusion: Surgery strategy is highly impacted by the presence of vascular extension in pediatric tumors. Surgeons should be aware of potential complications and how to prevent them. Such cases should be treated in reference centers.

Obesity in Survivors of Childhood Cancer: a Review.

Obesity is a late effect of antineoplastic treatment in childhood cancer survivors and this correlates with chronic complications. This review examines the data currently available to health professionals, for increasing awareness and identifying strategies to address the treatment and prevention of late effects. The mechanism involved in the pathophysiology of obesity remains unclear. However, damage to the hypothalamus and endocrine disorders (e.g. insulin and leptin resistance) and a positive energetic balance may play a role in increasing obesity rates. A patient's diet during, and after treatment may also influence the weight of survivors. Implementation of an effective educational program by professionals during all stages of treatment enables children to obtain basic knowledge regarding food and nutrition, thereby encouraging them to take responsibility for developing healthy eating behaviors.

The influence of antineoplastic treatment on the weight of survivors of childhood cancer / A influência do tratamento antineoplásico sobre o peso de sobreviventes do câncer na infância

Abstract Purpose: Obesity is a late effect in survivors of childhood cancer and correlates with chronic complications. Survivors of leukemia, brain tumors, and hematopoietic stem cell transplantation are more likely to develop obesity resulting from treatment modalities such as radiotherapy and glucocorticoids. This paper analyzes and integrates the current data available to health professionals in order to clarify strategies that can be used to treat and prevent obesity in childhood cancer survivors. Sources: This is a literature review from on scientifically reliable electronic databases. We selected articles published in the last five years and earlier articles of great scientific importance. Data synthesis: The mechanisms involved in the pathophysiology of obesity in cancer survivors are not completely understood, but it is believed that damage to the hypothalamus and endocrine disorders such as insulin resistance, leptin resistance, and hormone deficiency may be involved. The body composition of this group includes a predominance of adipose tissue, especially in those undergoing hematopoietic stem cell transplant and total body irradiation. The use of body mass index in these patients may lead to an underestimation of individuals' risk for metabolic complications. Conclusion: Early identification of groups using accurate anthropometric assessments, interventional treatment, and/or preventative measures and counseling is essential to minimize the adverse effects of treatment. Physical activity and healthy eating to promote adequacy of weight in the whole population should be encouraged.

Resumo Objetivo: A obesidade é um efeito tardio em sobreviventes do câncer na infância e está correlacionada com complicações crônicas. Os sobreviventes da leucemia, tumores cerebrais e transplante de células-tronco hematopoiéticas têm maior probabilidade de desenvolver obesidade como resultado das modalidades de tratamento, como radioterapia e glicocorticoides. Este artigo analisa e integra os dados atuais disponíveis a profissionais da saúde para esclarecer as estratégias que podem ser usadas para tratar e prevenir a obesidade em sobreviventes do câncer na infância. Fontes: Esta é uma análise da literatura de bases de dados eletrônicas cientificamente confiáveis. Selecionamos artigos publicados nos últimos cinco anos e artigos mais antigos de grande importância científica. Resumo dos dados: Os mecanismos envolvidos na fisiopatologia da obesidade em sobreviventes do câncer não são completamente entendidos, porém acredita-se que o dano no hipotálamo e disfunções endócrinas, como resistência à insulina, resistência à leptina e deficiência hormonal, possam estar envolvidos. A composição corporal desse grupo inclui uma predominância de tecido adiposo, principalmente em pacientes submetidos a transplante de células-tronco hematopoiéticas e irradiação de todo o corpo. O uso do índice de massa corporal nesses pacientes poderá levar a uma subestimação do risco de complicações metabólicas nessas pessoas. Conclusão: A identificação precoce de grupos por meio de avaliações antropométricas precisas, o tratamento intervencional e/ou medidas preventivas e aconselhamento são fundamentais para minimizar os efeitos colaterais do tratamento. A atividade física e alimentação saudável devem ser incentivadas para promover a perda de peso na população em geral.

The influence of antineoplastic treatment on the weight of survivors of childhood cancer.

PURPOSE: Obesity is a late effect in survivors of childhood cancer and correlates with chronic complications. Survivors of leukemia, brain tumors, and hematopoietic stem cell transplantation are more likely to develop obesity resulting from treatment modalities such as radiotherapy and glucocorticoids. This paper analyzes and integrates the current data available to health professionals in order to clarify strategies that can be used to treat and prevent obesity in childhood cancer survivors. SOURCES: This is a literature review from on scientifically reliable electronic databases. We selected articles published in the last five years and earlier articles of great scientific importance. DATA SYNTHESIS: The mechanisms involved in the pathophysiology of obesity in cancer survivors are not completely understood, but it is believed that damage to the hypothalamus and endocrine disorders such as insulin resistance, leptin resistance, and hormone deficiency may be involved. The body composition of this group includes a predominance of adipose tissue, especially in those undergoing hematopoietic stem cell transplant and total body irradiation. The use of body mass index in these patients may lead to an underestimation of individuals' risk for metabolic complications. CONCLUSION: Early identification of groups using accurate anthropometric assessments, interventional treatment, and/or preventative measures and counseling is essential to minimize the adverse effects of treatment. Physical activity and healthy eating to promote adequacy of weight in the whole population should be encouraged.

Fifteen Years' Experience of the Brazilian Osteosarcoma Treatment Group (BOTG): A Contribution from an Emerging Country.

PURPOSE: Little information is available regarding the tumor features, prognostic factors, and treatment results in children and adolescents and young adults (AYAs) with osteosarcoma diagnosed in developing countries. We reviewed the results of three observational cohorts of osteosarcoma patients treated in an emerging country. METHODS: A total of 604 patients below the age of 30 years with high-grade osteosarcoma were prospectively enrolled in the Brazilian Osteosarcoma Treatment Group (BOTG) studies III, IV, and V. Gender, age, time from onset of symptoms to diagnosis, primary tumor site, presence or absence of metastases at diagnosis, tumor size, type of surgery (limb-sparing or amputation), treatment protocol, and histological response were correlated with survival. RESULTS: The estimated 5-year overall survival and event-free survival (EFS) rates for the 553 eligible patients were 49% and 39% respectively; of the 390 non-metastatic patients included in the total, overall- and event-free survival were 59% and 48% respectively. Metastases at diagnosis, primary tumor site, type of surgery, and histological response were significant predictors of overall survival and EFS in univariate and multivariate analysis, whereas tumor size and treatment protocol lost prognostic significance in multivariate analysis. CONCLUSION: We report on the outcome of three consecutive studies for the treatment of osteosarcoma carried out in Brazil over 15 years. Although the survival rates presented are below those reported in current literature, it represents the result of a favorable experience gathered from the national collaborative work.