Central nervous system embronic tumors and prognostic properties treated in our center

Objective: The aim of this study is to reveal the demographic and clinical features, to examine the complaints and the time to first diagnosis, to evaluate the characteristics of the tumor, to examine the results of the treatments and treatments with general life and uneventful life rates, to determine late side effects and sequelae in Dokuz Eylül University Faculty of Medicine, Department of Pediatrics, Department of Pediatric Oncology in the years 1987-2018 admitted to childhood central nervous system embryonal tumors diagnosed patients. Patient and methods: The files of patients with central nervous system embryonal tumor diagnosed in 1987-2018 were evaluated retrospectively. A total of 77 patients' files were reached. Clinical characteristics, multimodal treatment regimens, side effects and sequelae were evaluated. Six patients were separated from our observation without our knowledge. While overall survival (OS) and event-free survival (EFS) were calculated, these patients were excluded from the study. The overall survival rate of the patients was obtained by subtracting the date of diagnosis from the last follow-up date, and the event-free life rate was obtained by subtracting the date of diagnosis from the date of the first event. The diagnosis of cancer was demonstrated histopathologically in all cases. The complaints of the patients were examined. The time from admission to diagnosis was evaluated. Clinical status at the time of diagnosis was evaluated. Treatment regimens and responses to these regimens were evaluated with OS and EFS. Patients were grouped according to tumor subgroups. Patients were compared according to POG risk score. Neurological and endocrine sequelae were evaluated in the surviving cases. Results: In our study, 77 cases were evaluated. 44 of the cases were male and 33 were female. The female to male ratio was 1.33 / 1. The most common clinical findings were vomiting and headache. Vomiting on admission in 50 patients (64.9%), headache in 36 (46.8%), ataxia in 26 (33.8%), cranial nerve paralysis in 17 (22%), and 10% (10%). 13) had seizures, 3 (3.9%) had personality changes, and 2 (2.6%) had dizziness. The mean time from clinical complaints to diagnosis was 1 month (2 weeks-2 months). There were 16 (20,7%) patients under 3 years of age. There were 31 (40,2%) patients between the ages of 0-5, 31 (40,2%) patients between the ages of 5-10 and 15 (19,6%) patients above 10 years of age. All cases had histopathological diagnosis. There were 65 patients (84.4%) with medulloblastoma, 10 patients (13%) with PNET / CNS neuroblastoma, and 2 patients (2.6%) with atypical rhabdoid teratoid tumors. All patients were pathologically examined in the grade 4 group. The mean tumor size was 42mm (10-80mm). There were 15 (19.4%) patients with tumor size smaller than 3 cm and 62 (80.6%) patients with tumor size larger than 3 cm. 20 (33.3%) of the medulloblastoma cases (n: 60) were evaluated at standard risk and 40 (66.6%) were at high risk. Patients were treated with treatment protocols consisting of surgery, CT and RT regimens. Tumor surgery was performed with 84% gross total and 16% subtotal excision. Craniospinal radiotherapy (RT) was applied to 75.3% of the patients and chemotherapy (CT) was applied to 57.1% of the patients. Over the years, different KT regimens have been applied, mainly consisting of platinum group, alkylating agents and etoposide-based combinations. The mean follow-up was 81 months (1-326 months). One-year OS was 64%, 5-year OS was 56%, 10-year OS was 54% in all CNS embryonic tumor cases. In the same cases, 1-year EFS was 60%, 5-year EFS 54%, 10-year EFS 51%. Five-year OS and EFS were 62% / 60% of patients with medulloblastoma. Five-year OS and EFS values were 24% / 18% in patients with non-medulloblastoma embryonal tumors. The 5-year OS and EFS values of the patients at standard risk were 85% / 80%. Five-year OS and EFS values of high-risk cases were 50% / 40%. Sequelae were mostly due to surgical treatments. Cranial nerve palsies, motor dysfunction and ataxia were frequently observed. 23 patients (30%) had CSF flow regulators. 8 patients (10.3%) had ototoxicity due to platinum use and 1 patient had VCR polyneuropathy. A total of 32 patients (45.2%) died. Twenty-one (65.6%) died of progression, 7 (21.8%) died of relapse, 2 (6.2%) died of treatment toxicity, and 2 (6.2%) died of other causes. Discussion: SSS embryonal tumors are a topic that has been discussed in recent years. Despite advances in technology and innovations in treatment, refractoriness, relapse and deaths are still visible. In our study, anti-cancer treatment varied over the years. The survival rates of our treatments are similar to the current literature. Multimodal approaches to treatment increase the uneventful survival of patients. Early recognition of clinical symptoms and widespread use of imaging methods have enabled early diagnosis of these patients. With appropriate treatments for risk groups, especially in patients diagnosed with medulloblastoma, results were good. Non-medulloblastoma CNS embryonal tumors still have poor survival rates. Management of sequelae due to the increase in the number of living patients is important for the comfort of life of the patients. Keywords: Childhood, CNS Embryonal Tumors, Clinical Features, Treatment, Survival Rates