Therapeutic strategies and clinical evolution of patients with infantile fibrosarcoma: a unique paediatric case series.

BACKGROUND: Infantile fibrosarcoma is the most frequent soft tissue sarcoma in newborns or children under one year of age. This tumour often implies high local aggressiveness and surgical morbidity. The large majority of these patients carry the ETV6-NTRK3 oncogenic fusion. Hence, the TRK inhibitor larotrectinib emerged as an efficacious and safe alternative to chemotherapy for NTRK fusion-positive and metastatic or unresectable tumours. However, real-world evidence is still required for updating soft-tissue sarcoma practice guidelines. OBJECTIVE: To report our experience with the use of larotrectinib in pediatric patients. METHODS: Our case series shows the clinical evolution of 8 patients with infantile fibrosarcoma under different treatments. All patients enrolled in this study received informed consent for any treatment. RESULTS: Three patients received larotrectinib in first line. No surgery was needed with larotrectinib, which led to the rapid and safe remission of tumours, even in unusual anatomical locations. No significant adverse effects were observed with larotrectinib. CONCLUSION: Our case series supports that larotrectinib may be a therapeutic option for newborn and infant patients with infantile fibrosarcoma, especially in uncommon locations.

Validity and reliability of the questionnaire of academic knowledge of teachers of basic general education.

Background: The concern and analysis about the knowledge possessed by teachers of basic general education persists in academic debate and professional practice. It is noteworthy that in the studies consulted, there is no precise evidence that determines with accuracy the configurations of these knowledge factors that function as the basis of the teaching profession. Therefore, the objective of this study is to establish the construct validity and reliability of the questionnaire on the nature or origin of the academic knowledge of teachers of basic general education, adapted from the Pedagogical Content Knowledge (PCK) Competence Model. Methods: A methodological study was established that applies a test to the processes of reliability and internal consistency. The construct validity was performed through (n = 8) expert judges, using Cohen's Kappa. An exploratory factor analysis was performed following the criteria of the Kaiser-Meyer-Olkin Coefficient (KMO), the Bartlett sphericity test and the principal components extraction method in the factor analysis with varimax rotation. The sample consisted of (n = 27) teachers of basic general education of the Ibarra Canton. Results: The results show a reliability analysis for the instrument obtained a Cronbach's alpha (α = 0.901), estimated to be an excellent level. Conclusions: The questionnaire is relevant, valid and reliable, adapting to the needs of teachers of basic general education to determine the nature or origin of the academic knowledge in a fast and reliable manner.