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Identifier 000449815
Title Νεότερα βιβλιογραφικά δεδομένα στην ταξινόμηση - θεραπεία του Νευροβλαστώματος στα παιδιά και αποτύπωση 20ετούς εμπειρίας της Κλινικής Αιματολογίας-Ογκολογίας Παίδων του ΠαΓΝΗ
Alternative Title Current data in the classification and treatment of neuroblastoma in children and depiction of 20-year experience in the Department of Pediatric Hematology and Oncology of the University General Hospital of Heraklion (PAGNI)
Author Παναγιώτου, Γεωργία
Thesis advisor Στειακάκη, Ευτυχία
Reviewer Ραϊσάκη, Μαρία
Δράκος, Ηλίας
Abstract Introduction: Neuroblastoma is a fetal tumor of the peripheral sympathetic nervous system and is the most common extracranial solid tumor in childhood, accounting for 8% of malignancies and 15% of childhood cancer deaths. The manifestations of neuroblastoma vary depending on the location of the tumor and the presence or absence of paraneoplastic syndromes, while special features of these neuroendocrine tumors are the early age of onset, the high frequency of metastatic disease at diagnosis and the tendency for automatic healing in infancy. The age of the patient, the stage of the tumor according to the staging systems INRG and INSS, the histopathological classification and the amplification of the oncogene MYCN are the most documented prognostic indicators. Nevertheless, several new gene lesions have been identified in sporadic and familial cases of neuroblastoma that show an association with adverse outcome. Regarding the outcome of children with neuroblastoma, it shows great heterogeneity, ranging from complete cure to a 5-year survival rate of over 90% in patients with low-risk disease, to less than 50% of 5-year survival, in patients with high-risk disease, who are likely to be resistant even to high doses of chemotherapy regimens. Therefore, it is necessary to identify new targets and new immunotherapeutic approaches, in order to increase the survival rates of patients belonging to high-risk categories. Purpose: The purpose of the study is to review the literature on the latest data in the classification and treatment of patients with neuroblastoma. At the same time, there will be a retrospective recording of children with neuroblastoma at the Pediatric Hematology-Oncology Clinic PAGNI in the years 1999-2021 in order to capture the experience of a center in clinical practice and to correlate the demographic and clinical data, available histological and molecular data. and data on the biological background of the disease, the diagnosis, the distinction of risk groups, the staging, the treatment and the final outcome of the patients in relation to the international experience during this time period. Methodology: The review of the international literature was carried out in the Pubmed database and articles from the last 10 years were used. All children with neuroblastoma who were diagnosed, hospitalized and monitored at the Pediatric Hematology-Oncology Clinic PAGNI from 1999 to 2021 were retrospectively studied. The data used were extracted from patients' medical records and all available information on clinical characteristics of patients, location of primary lesion and metastatic localization, biopsy result, laboratory and cytogenetic test results where available, as well as type of treatment (based on current treatment protocol during hospitalization) and outcome of the disease. End Results: During the period between 1999 and 2021, from which this study gathered data, 28 patients with neuroblastoma were diagnosed in the Pediatric Hematology - Oncology Clinic of the Pancretan General Hospital of Herakleion, with the mean age of diagnosis being 28 months of age and when distributed based on sex, males outweighed females 2.1/1. The most common primary sitewas the adrenal glands and regarding clinical presentation most patients were asymptomatic. Metastatic disease was present in 15 out of 28 patients at the time of diagnosis (53,5%), with the most common site of metastases being the bones (75%), and followed by bone marrow, liver, lungs and distal lymph nodes. Recurrence occurred in 8 children (28,5%), out of which 5 passed away. In patients with neuroblastoma, statistically important negative predicting factors were found to be the stage of the disease, the age of diagnosis (>18 months), the adverse histological findings according to the INPC classification, elevated ferritin levels and the excess of the 17q chromosome (p<0,05). Of the 5 patients who died, all of them exhibited a relapse, were in M-stage disease, had unfavorable histological findings and high risk, drug resistant neuroblastomas. Conclusions: From the results of the retrospective study, many agree with the data of the literature, while the differences are due, on the one hand to the small sample of patients and on the other hand to the medical development and changes in treatment protocols that have occurred in recent years. Continuing to record children with neuroblastoma will be very important to lead to safer results. Despite the bounds in the treatment of neuroblastoma over the past two decades, the prognosis in children at high risk remains poor. The aim is to find new therapeutic approaches with targeted molecular therapies as well as new immunotherapeutic regimens, which are integrated into existing treatment protocols, in order to improve the survival rate of those patients who belong to high-risk groups.
Language Greek
Subject Infants
Risk groups
Βρέφη
Νευροβλάστωμα
Issue date 2022-07-29
Collection   School/Department--School of Medicine--Department of Medicine--Post-graduate theses
  Type of Work--Post-graduate theses
Permanent Link https://elocus.lib.uoc.gr//dlib/f/e/3/metadata-dlib-1657621128-887136-29651.tkl Bookmark and Share
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