Cross-Cultural Adaptation and Validation of the Romanian Musculoskeletal Tumor Society Scoring System for Patients with Extremity Bone Sarcomas.

Background and Objectives: Primary malignant bone tumors are rare lesions, and their complex treatment can lead to functional impairment. It is important to have a postoperative assessment tool for patients' functional outcomes to be evaluated and to consequently adapt future treatments in the pursuit of a continuous improvement of their quality of life. The Musculoskeletal Tumor Society Score (MSTS) is a validated specific system score that is used frequently in the follow-up of these patients. We found no information about a valid translated Romanian version of this score neither for the upper limb nor for the lower limb. We proposed in this study to translate the original version of the MSTS Score into Romanian and to perform validation analysis of the Romanian-language MSTS Score. Materials and Methods: We selected 48 patients who underwent limb-salvage surgery after resection of bone sarcomas. Patients were interrogated twice according to the translated Romanian version of the MSTS Score during their follow-up. The translation was performed according to the recommended guidelines. A total number of 96 questionnaires were valid for statistical analysis. Results: Internal consistency and reliability were good for both sets of questionnaires' analytic measurements, with Cronbach's alpha values of 0.848 (test) and 0.802 (retest). The test-retest evaluation proved to be statistically strong for reproducibility and validity with Spearman's rho = 0.9 (p < 0.01, 95% CI). Conclusions: This study permitted the translation of this score and the validation of psychometric data. Our results showed that the Romanian version of the MSTS is a reliable means of assessment of the functional outcome of patients who received limb-salvage surgery for the upper and lower extremities.

Therapeutic results in children with brain tumors - a single center experience over 18 years.

Background and aims: Tumors of the central nervous system represent the main cause of death by cancer in children. The diagnosis and molecular classification of these neoplasms have seen great improvement in the past years, due to ongoing genomic advances. In general, the treatment consists of surgery, radiation therapy and chemotherapy. However, the currently available pharmacological treatment options have limited effectiveness due to the particular characteristics of the blood-brain barrier. Methods: We decided to study the therapeutic results in children treated for brain tumors in the Cluj-Napoca "Prof. dr. Ion Chiricuta" Oncology Institute, between 2001 and 2018, in order to provide a more accurate understanding of the disease and the available therapeutic options in our center. Results: Out of the 207 cases included in this study, we recorded 98 deaths (47.3%). This is significantly less than the 5-year survival rate recorded in the US between 2012 and 2018 (74.9%). There are many factors that could explain the low survival rate, such as a very late diagnosis, the inability to implement innovative radiation therapy techniques until 2018, and the fact that between 2001 and 2010 the chemotherapy regimens in our center were not as effective as the more recent ones. Conclusions: The therapeutic results recorded in this study are similar to those in other middle-income countries, however, the available treatment options for pediatric brain tumors are not as effective as those currently in use for other pediatric and adult malignancies.

Early Deaths in Childhood Cancer in Romania-A Single Institution Study.

(1) Background: Survival in childhood cancer has improved significantly over the last decades. However, early deaths (EDs) represent an important number of preventable deaths. Our aim was to provide more insight intoEDs in developing countries. (2) Methods: We conducted a retrospective analysis of patients aged 0-18 years with childhood cancer diagnosed between 1996 and 2008 and admitted in the Institute of Oncology "Prof. Dr. Ion Chiricuta" Cluj-Napoca (IOCN), Romania. After exclusion of patients (pts) older than 18 years at diagnosis, pts with a missing personal identification number and pts with unconfirmed diagnosis of malignancy, we included 783 pts in the final analysis. We defined ED as survival of less than one month after cancer diagnosis. We divided pts in groups according to age, major tumour categories and treatment time periods. (3) Results: ED was registered in 20 pts (2.55%). A total of 16EDs were registered in haematologic malignancies and 4 in solid tumours. Statistical analysis was performed on pts diagnosed with haematological malignancies. A statistically significant higher proportion of patients with performance status (PS) 3 and 4 died within one month after diagnosis (24.1%) than patients admitted with PS 0-2 (1%)-p < 0.01. We found no statistically significant difference regarding ED when comparing male versus female (p = 0.85), age at diagnosis or between the threeperiods of diagnosis (p = 0.7). (4) Conclusions: PS at admission is an important risk factor associated with ED in pts with haematologic malignancies. ED in our institution reflects frequent late presentation for medical care, late diagnosis and referral to specialised centres.

Updates on the 2016 World Health Organization Classification of Pediatric Tumors of the Central Nervous System - a systematic review.

Tumors of the central nervous system (CNS) represent the main cause of death through solid tumors in children and the second most frequent neoplasm in this patient group. The poor survival rate is due to many factors, such as the large diversity of morphological features, the particular micro-environmental characteristics of the nervous tissue, the relative rareness in relation to other childhood diseases, which leads to late diagnosis and the limited effectiveness of the available treatment options. Up until 2016, brain tumors were classified according to their histologic features. The new 2016 World Health Organization (WHO) Classification of CNS tumors incorporates molecular features, alongside the immunohistology, in order to provide a more accurate understanding of the disease. The treatment consists of surgery, radiation therapy and chemotherapy. We decided to review the literature on this pathology, in order to show the importance of the recent discoveries in this field.

Pleuropulmonary blastoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations.

Pleuropulmonary blastoma (PPB) is a rare cancer occurring mainly during early childhood and often associated with germline DICER1 mutations. It is classified by the macroscopic appearance into three interrelated clinico-pathologic entities on a developmental continuum. Complete tumor resection is a main prognostic factor and can be performed at diagnosis or after neoadjuvant treatment that includes chemotherapy and in some cases radiotherapy. Optimal modalities of neo- or adjuvant treatments can be challenging taking into account potential long-term toxicities in this young population. This paper presents the recommendations for diagnosis and treatment of children and adolescents with PPB elaborated by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the European Union-funded project PARTNER (Paediatric Rare Tumours Network - European Registry).

Genetic predisposition in pediatric oncology.

Identifying patients with a genetic predisposition for developing malignant tumors has a significant impact on both the patient and family. Recognition of genetic predisposition, before diagnosing a malignant pathology, may lead to early diagnosis of a neoplasia. Recognition of a genetic predisposition syndrome after the diagnosis of neoplasia can result in a change of treatment plan, a specific follow-up of adverse treatment effects and, of course, a long-term follow-up focusing on the early detection of a second neoplasia. Responsible for genetic syndromes that predispose individuals to malignant pathology are germline mutations. These mutations are present in all cells of conception, they can be inherited or can occur de novo. Several mechanisms of inheritance are described: Mendelian autosomal dominant, Mendelian autosomal recessive, X-linked patterns, constitutional chromosomal abnormality and non-Mendelian inheritance. In the following review we will present the most important genetic syndromes in pediatric oncology.

Post-Treatment Thyroid Diseases in Children with Brain Tumors: A Single-Center Experience at "Prof. Dr. Ion Chiricuța" Institute of Oncology, Cluj-Napoca.

AIM OF STUDY: The purpose of the study was to evaluate the association of thyroid dysfunction occurring in pediatric patients treated for brain tumors. PATIENTS AND METHODS: A total of 255 patients with brain tumors were treated between 2001 and 2018 at the "Prof. Dr. Ion Chiricuța" Institute of Oncology, Cluj-Napoca. Due to a minimum follow-up of 4 years, we studied 184 out of the 255 patients. The cohort included 69 girls (37.5%) and 109 boys (62.5%), with a median age of 8.4 years. The evaluated tumors included medulloblastomas (47 patients), astrocytomas (44 patients), ependymomas (22 patients), gliomas (20 patients), germ cell tumors (12 patients), primitive neuroectodermal tumors (4 patients), as well as other types of tumors (15 patients); in 20 of the cases, biopsy could not be performed. RESULTS: There was a 60% overall survival rate; among the 120 surviving patients, 11 (9.1%) were diagnosed with iatrogenic thyroid disease. We observed an important number of iatrogenic thyroid disease cases in this group of patients, thus revealing the importance of long-term thyroid function evaluation in all children who finalized their treatment for brain tumors. Through this study, we aimed to provide an accurate image of the methodology of monitoring for thyroid dysfunction in childhood brain tumor survivors. CONCLUSION: Given the fact that the probability of developing thyroid dysfunction in the pediatric population treated for brain tumors is not rare, we recommend that childhood brain tumor survivors be monitored for iatrogenic thyroid disease, in order to provide early diagnosis and treatment.