Cancer Prevalence in Children with Inborn Errors of Immunity: Report from a Single Institution.

Background: Inborn Errors of Immunity (IEI) comprise several genetic anomalies that affect different components of the innate and adaptive responses, predisposing to infectious diseases, autoimmunity and malignancy. Different studies, mostly in adults, have reported a higher prevalence of cancer in IEI patients. However, in part due to the rarity of most of these IEI subtypes (classified in ten categories by the Primary Immunodeficiency Committee of the International Union of Immunological Societies), it is difficult to assess the risk in a large number of patients, especially during childhood.

Rare tumors in pediatrics. First report in Argentina.

Introduction: Collaborative clinical trials have enlarged the knowledge base about pediatric tumors; however, this is not the case for rare tumors (RT).

Objective: To describe the prevalence, clinical characteristics, and course of RT in pediatric patients diagnosed at Hospital Garrahan.

Material And Methods: Descriptive, retrospective study of patients aged 0-18 years diagnosed with a RT and admitted between January 2007 and December 2017.

Outcomes of event-free survival in patients with Wilms tumor undergoing preoperative chemotherapy. Analysis of lymph-node yield in a single-center cohort.

Introduction: The clinical value of lymph node sampling in Wilms tumor (WT) lies in its ability to accurately determine lymph node (LN) involvement. LN yield (LNY) is used as a valuable tool to measure LN retrieval, and a minimum of 6 LNs is one of the current recommendations. In patients who are managed with the SIOP strategy, preoperative chemotherapy decreases the retrieval of LN during surgery resulting in lower LNY values.

Desmoid-type fibromatosis in children. Clinical features, treatment response, and long-term follow-up.

Desmoid-type fibromatosis (DF) is a tumor with high local recurrence rate. Sixteen patients (18 desmoid tumors) were retrospectively evaluated. Initial surgery was performed in 13/18 tumors, with complete resection in 6 (one with free margin and five with microscopic residual disease); 10/13 had local relapse.

Neuroblastoma in patients under 18 months. Single institution experience in Argentina.

The purpose of the study was to evaluate the outcome of patients under 18 months diagnosed with neuroblastoma. Between April 2006 and December 2013, 45 consecutive patients followed in Hospital de Pediatría Garrahan, were retrospectively reviewed. With a median age of 9.

Laparoscopic total nephrectomy for Wilms tumor: Towards new standards of care.

Introduction: Laparoscopic total nephrectomy (LN) in malignant pediatric tumors remains controversial. For selected patients undergoing pre-operative chemotherapy in referral centers, LN has so far shown comparable results to the standard open technique.

Purpose: To describe the inclusion criteria and preliminary results of laparoscopic nephrectomies (LN) for the treatment of unilateral Wilms tumors (WT).

Beliefs and Determinants of Use of Traditional Complementary/Alternative Medicine in Pediatric Patients Who Undergo Treatment for Cancer in South America.

Purpose The use of traditional complementary/alternative medicine (TCAM) among children with cancer has been well documented. South America has a rich history of traditional healers and medicinal resources; however, little is known about the use of TCAM among children with cancer. We sought to investigate patterns, beliefs, and determinants of TCAM use among South American children with cancer.

Ambulatory High-dose Methotrexate Administration in Pediatric Osteosarcoma Patients at a Single Institution in Argentina.

Background: The purpose of this study was to evaluate the feasibility and safety of ambulatory high-dose methotrexate (HDMTX) administration with oral hydration, alkalinization, and leucovorin rescue. HDMTX (12 g/m) was given intravenously over 4 hours after urine alkalinization. Families and patients were instructed to continue ambulatory oral hydration and alkalinization to monitor urine pH and to adjust bicarbonate according to our institution's treatment algorithm.

Results of a randomized, prospective clinical trial evaluating metronomic chemotherapy in nonmetastatic patients with high-grade, operable osteosarcomas of the extremities: A report from the Latin American Group of Osteosarcoma Treatment.

Background: Metronomic chemotherapy (MC) consists of the administration of a low dose of chemotherapy on a daily or weekly basis without a long break to achieve an antitumoral effect through an antiangiogenic effect or stimulation of the immune system. The potential effect of MC with continuous oral cyclophosphamide and methotrexate in patients with high-grade operable osteosarcomas (OSTs) of the extremities was investigated.

Methods: Patients with high-grade OSTs who were 30 years old or younger were eligible for registration at diagnosis.

Malignant extra-cranial germ cell tumors in children and adolescents. Results following the guidelines of SFOP/SFCE 95 Protocol.

Between September 1995 and December 2010, 99 new consecutive assessable patients with extra-cranial MGCT were treated according to SFOP/SFCE TGM95 Protocol. A "watch and wait" strategy for completely resected stage I-II was observed in cases with preoperative high tumor markers levels. Metastatic disease or alpha fetoprotein levels > 15 000 ng/ml cases were treated by VIP chemotherapy (etoposide, ifosfamide and CDDP) 4-6-courses.

Outcome of Nephroblastoma Treatment According to the SIOP-2001 Strategy at a Single Institution in Argentina.

Objective: Wilms tumor (WT) is a disease with a good prognosis. The aim of this study was to evaluate the outcome of patients with WT, treated according to the SIOP-2001 strategy.

Methods: A retrospective analysis of 141 consecutive patients with WT diagnosed at our institution between December 2001 and 2013 was performed.

Childhood Neuroblastoma: Incidence and Survival in Argentina. Report from the National Pediatric Cancer Registry, ROHA Network 2000-2012.

Background: There are reports indicating a low incidence of neuroblastoma (NB) in some developing countries but no conclusive data are available from population-based studies at a national level.

Purpose: To describe the incidence and survival of 971 patients with NB in Argentina with data from the National Pediatric Cancer Registry (ROHA), and the impact of age, gender, stage, regional, and socioeconomic indicators on outcome.

Methods: All cases of NB reported to ROHA (2000-2012) were the subject of the analysis.

[Multi-parametric Flow Cytometry for Neuroblastoma, a new and possible diagnostic tool: case report].

Neuroblastoma is the most frequent extracranial solid tumor in childhood, representing 5.6% according to the "Registro Oncopediatrico Hospitalario Argentino". For its diagnosis, several complementary methods (radiological, biological and biochemical) are required, and Multi-parametric Flow Cytometry (MFC) arises as a potential diagnostic method, despite not having been so far extensively explored.

Racotumomab for treating lung cancer and pediatric refractory malignancies.

Introduction: Racotumomab (originally known as 1E10 mAb) is an anti-idiotype murine IgG1 directed to membrane glycoconjugates expressed in aggressive solid tumors. It was developed as a mirror image of the idiotype of another antibody against N-glycolyl-containing molecules, such as the NeuGcGM3 ganglioside. After a successful phase II/III study, racotumomab formulated in alum was conditionally approved in Latin American countries as maintenance therapy for advanced non-small cell lung cancer.

A Phase I Study of the Anti-Idiotype Vaccine Racotumomab in Neuroblastoma and Other Pediatric Refractory Malignancies.

Background: Pediatric neuroectodermal malignancies express N-glycolylated gangliosides including N-glycolyl GM3 (NeuGcGM3) as targets for immunotherapy.

Procedure: We evaluated the toxicity and maximum tolerated dose and immunological response of racotumomab, an anti-idiotype vaccine targeting NeuGcGM3 through a Phase I study enrolling children with relapsed or resistant tumors expressing NeuGcGM3.

Materials And Methods: Drug dose was escalated to three levels (0.

[Pediatric adamantinoma. Case report].

Adamantinoma is a primary tumor of long bones, which affects mainly the shaft of the tibia, and is extremely rare in pediatrics. It frequently presents during the second decade of life, with a slight predominance in males. It is a low grade tumor, with local aggressiveness and low rate of metastasis and recurrence once it is completely removed.

Improving our understanding of the use of traditional complementary/alternative medicine in children with cancer.

Background: Children with cancer in high-income and low-income countries often use traditional complementary/alternative medicine (TCAM). With efforts by the World Health Organization and international twinning programs improving access to conventional care for patients with childhood cancer, understanding the global use of TCAM is important because reliance on TCAM may affect time to presentation, adherence, and abandonment of care. In the current study, the authors describe the process and validation of an international survey documenting the use of TCAM among children with cancer.

Phase II study of high-dose cyclophosphamide in relapsing and/or resistant hepatoblastoma in children: a study from the SIOPEL group.

The study sought to evaluate the response to cyclophosphamide (CPM) in hepatoblastoma (HB). Patients with a refractory or relapsing HB after first-line therapy as per SIOPEL 2 and 3 protocols were eligible. All patients were to receive two courses of CPM 2 g/m(2) on days 1 and 2 at 3-week intervals.