Dados do Trabalho

Título

CLINICAL AND PATHOLOGICAL CHARACTERIZATION OF DISSEMINATED PILOCYTIC ASTROCYTOMAS: A LONG-TERM RETROSPECTIVE ANALYSIS FROM A SPECIALIZED ONCOLOGICAL CENTER

Resumo

ABSTRACT

BACKGROUND: Among pediatric CNS tumors, pilocytic astrocytoma (AP) is the most frequent tumor, presenting non-malignant features, like slow growth and non-infiltrative traits. Rearrangements in the BRAF gene are the main molecular alteration. Up to 10% may show dissemination. In such cases, radiation therapy, chemotherapy and targeted therapies can be used. AIM: Our aim is to describe clinical and pathological aspects of patients with disseminated pilocytic astrocytoma at the Barretos Cancer Hospital from January 2000 to December 2019. METHODS: It’s a longitudinal cohort study, with retrospective data collection and analysis of clinical and molecular data from pediatric patients diagnosed with disseminated pilocytic astrocytoma from 2000 to 2019. RESULTS: From 93 patients diagnosed with AP during this time, 10 (10,7%) had disseminated disease at diagnosis or follow-up. Half (5/10, 50,0%) were in the hypothalamus/optical pathway region. Treatment modalities varied according to age and clinical conditions. BRAF V600E mutation was detected in 3 tumors from 8 analyzed (3/8, 37,5%) and one had KIAA1549: BRAF fusion (1/5, 20,0%). At this moment, 60% of patients are alive and the overall 3-year survival was 65%. CONCLUSION: This study provided clinical and epidemiological data of patients with disseminated AP from a specialized group in pediatric neuro-oncology. Findings are similar to those described in the literature regarding the analyses described (clinical, radiological, morphological, molecular, therapeutic and survival).

Key words: pilocytic astrocytoma, central nervous system, pediatrics, proto-oncogene proteins BRAF.

Área

Neuro-oncologia