RESUMO
Angola, a country grappling with prevalent tropical diseases such as malaria, is witnessing an alarming rise in cancer-related deaths. Despite the escalating significance of cancer globally and in Angola, the nation's medical schools lack compulsory oncology disciplines in their curricula. This absence compromises the comprehensive training of medical students, preventing the development of integrated perspectives and skills crucial for addressing the growing cancer burden. This article, authored by the Angolan Oncology Research Group (AORG), proposes an oncology program for undergraduate medical students in Angola, aiming to bridge the educational gap. The program outlines discipline objectives, topics to be covered, class formats, and workload considerations.
RESUMO
Introdução: O sarcoma de Ewing extraósseo (SEE) de pâncreas representa 0.3% dos tumores que acometem o pâncreas nessa faixa etária pediátrica, sendo tão raro quanto outros tumores pancreáticos em crianças. Objetivo: Relatar caso raro de SEE primário de pâncreas em criança. Relato do caso: Paciente de sexo feminino, branca, 10 anos de idade, com história de dor abdominal, náuseas e icterícia. O estudo de imagem com ressonância magnética revelou massa em cabeça de pâncreas medindo 6.3 x 5.3 x 5.7cm, volume tumoral de 99,53ml. A biópsia por trucut da tumoração revelou neoplasia de pequenas células redondas azuis. O laudo histopatológico foi compatível com Sarcoma de Ewing/PNET, e o exame molecular para a pesquisa de translocação pelo método FISH, foi positivo para presença de translocação envolvendo o gene EWSR1 (22q12,1-q12,2). O estadiamento mostrou doença não metastática ao diagnóstico. A paciente recebeu tratamento quimioterápico com vincristina, doxorrubicina e ciclofosfamida alternados com ifosfamida e etoposide, de acordo com o protocolo GALOP. Na semana 14 foi submetida à duodeno gastropancreatectomia parcial com margens livres, com resseção de 11 linfonodos. Na histopatologia apresentava 80% de células viáveis no tumor primário e todos os linfonodos foram negativos para neoplasia. Devido ao baixo índice de necrose, a paciente recebeu radioterapia a partir da semana 21 na dose de 4500cGy + 500cGy de reforço no leito tumoral. Atualmente a paciente encontra-se em controle da doença há 9 meses após o término de tratamento oncológico. Conclusão: Devido a rara apresentação é necessário alto índice de suspeita de SEE de pâncreas. Com o tratamento multimodal realizado por equipe multidisciplinar especializada, foi possível obter bom controle de doença, sendo necessário mais tempo de acompanhamento
Introduction: Extraosseous Ewing sarcoma (EES) of the pancreas represents 0.3% of tumors that affect the pancreas in this pediatric age group, being as rare as other pancreatic tumors in children. Objective: To report a rare case of primary EES of the pancreas in a child. Case report: Female patient, white, 10 years old, with a history of abdominal pain, nausea and jaundice. The magnetic resonance imaging study revealed a mass in the head of the pancreas measuring 6.3 x 5.3 x 5.7cm, tumor volume of 99.53ml. Trucut biopsy of the tumor revealed small round blue cell neoplasm. The histopathological report was compatible with Ewing Sarcoma/PNET, and the molecular examination for translocation research using the FISH method was positive for the presence of a translocation involving the EWSR1 gene (22q12.1- q12.2). Staging showed non-metastatic disease at diagnosis. One patient received chemotherapy treatment with vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide, according to the GALOP protocol. At week 14 he underwent partial duodenal gastropancreatectomy with free margins, with resection of 11 lymph nodes. Histopathology showed 80% viable cells without primary tumor and all lymph nodes were negative for neoplasia. Due to the low rate of necrosis, one patient received radiotherapy from week 21 at a dose of 4500cGy + 500cGy booster to the tumor bed. Currently, the patient has been in control of the disease for 6 months after the end of oncological treatment. Conclusion: Due to the rare presentation, a high index of suspicion for pancreatic EES is necessary. With multimodal treatment carried out by a specialized multidisciplinary team, it was possible to obtain good control of the disease, requiring longer follow-up time