Abstract |
Introduction
Bone sarcomas and soft-tissue sarcomas, including rhabdomyosarcoma, is a heterogeneous group of tumors of mesenchymal origin that can present at any age. The most common bone sarcoma is osteosarcoma, followed by Ewing sarcoma and chondrosarcoma. Incidence increases with increasing age until 15 years and then diminishes. These patients treated with a combination of surgery, chemotherapy and radiotherapy, mostly for Ewing sarcoma, as osteosarcoma is not a radiosensitive tumor. They often carry dismal prognosis. Rhabdomyosarcoma is the most common soft-tissue sarcoma of children and is a highly aggressive tumor. It is treated with a combination of surgery, chemotherapy and radiotherapy. Prognosis for low and intermediate risk rhabdomyosarcoma is usually good with high overall survival. On the other hand, prognosis for high risk and relapsed rhabdomyosarcoma is poor.
This diploma thesis will review the latest data on treatment and prognosis of osteosarcoma, Ewing sarcoma and rhabdomyosarcoma. A retrospective study of the 25-year experience of the Pediatric Hematology-Oncology Department of the University Hospital of Heraklion will be also performed.
Hematology - Oncology of Childhood and Αdolescence
School of Medicine – University of Crete
5
Objective
This study will review the literature for the latest data on treatment and prognosis of bone sarcomas and rhabdomyosarcoma. At the same time a retrospective study of children, adolescents and young adults will be performed in the Pediatric Hematology-Oncology Department of the University Hospital of Heraklion in the last 25 years (1996-2021) aiming to capture a single-center experience and show the effect of new strategies in the diagnosis, treatment and disease outcome.
Methods
Literature review was performed using Pubmed database and retrieved clinical trials, reviews, systemic reviews, meta-analyses of the last 5 years (2016 to 2021). The retrospective study comprised all children, adolescents and young adults with osteosarcoma, Ewing sarcoma and rhabdomyosarcoma, diagnosed, hospitalized, treated and followed up in the Pediatric Hematology-Oncology Department of the University Hospital of Heraklion from 1996 to 2021. Data were collected from their medical files and included available information on clinical-epidemiological patient characteristics, biopsy results and genetic features where available, treatment protocols, disease course and patient outcome. A data sheet was created in Microsoft excel using an identification number for each patient and then statistical analysis was performed using Microsoft Excel and IBM SPSS Statistics 25
Results
During the study period (1996 to 2021) 14 patients with osteosarcoma, 13 with Ewing sarcoma and 11 with rhabdomyosarcoma were diagnosed in the Pediatric Hematology-Oncology Department of the University Hospital of Heraklion. Their median age was 13.9 for osteosarcoma patients, 12.1 for Ewing sarcoma patients and 6.5 for rhabdomyosarcoma patients. At time of diagnosis 4 patients with Ewing sarcoma and 1 with rhabdomyosarcoma had metastatic disease. Three patients with osteosarcoma, 3 patients with Ewing sarcoma and 4 patients with rhabdomyosarcoma relapsed. The 3-year overall survival of osteosarcoma patients was 88,9%, of Ewing sarcoma patients was 50% and of rhabdomyosarcoma patients was 87.5%. The 3-year event free survival was 64.8% for osteosarcoma patients, 45.5% for Ewing sarcoma patients and 50% for rhabdomyosarcoma patients.
Statistically significant poor prognostic factor for osteosarcoma and Ewing sarcoma patients was relapse (p=0.012 and p=0.048 respectively). In rhabdomyosarcoma patients unfavorable primary tumor site, tumor size and raised LDH were statistically significant poor prognostic factors (p=0.048, p=0.024 and p=0.019 respectively).
Hematology - Oncology of Childhood and Αdolescence
School of Medicine – University of Crete
6
Conclusions
Despite existing progress in treatment of bone sarcomas and rhabdomyosarcoma, prognosis of patients with refractory, relapsed and metastatic disease remains poor. Future goals include on one hand new treatment strategies, targeted treatments or combinations in order to improve outcomes for these patients and on the other hand, to reduce the intensity of treatment in low-risk patients.
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