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DOI: 10.1055/s-0044-1785523
Partial Sacrectomy in the Management of Sacral Chordoma: Case Report and Literature Review
Sacrectomia parcial no manejo do cordoma sacral: Relato de caso e revisão da literatura
Abstract
Chordomas are rare neoplasms of low to intermediate grades, which arise from ectopic remnants of notochordal tissue, presenting a slow growth pattern and locally aggressive behavior. Due to their insidious course, the diagnosis is late, requiring immediate therapeutic intervention. The main prognostic factor is total surgical resection with wide margins. This therapeutic objective is only achieved in 40% to 55.6% of the cases, since chordoma tends to present an aggressive behavior, invading adjacent tissues and neurovascular structures. Currently, the main challenge of sacrectomy is to balance a wide resection with the preservation of the neurological function of the patient. Despite cases of successful gross total resection, local recurrence is an inevitable reality, and the overall survival is relatively low. The indication of adjuvant therapies is not well stablished in the literature, since the response to radiotherapy is not satisfactory for these tumors. The aim of the present study is to present a report the case of a patient with sacral chordoma (SC) who underwent partial sacrectomy and to carry out a brief review of the literature on sacrococcygeal chordomas.
Resumo
Cordomas são neoplasias raras de graus baixo a intermediário, que surgem de remanescentes ectópicos de tecido notocordal, e apresentam padrão de crescimento lento e comportamento localmente agressivo. Devido ao seu curso insidioso, o diagnóstico é tardio, e necessita de intervenção terapêutica imediata. O principal fator prognóstico é a ressecção cirúrgica total com margens amplas. Esse objetivo terapêutico somente é atingido em 40% a 55,6% dos casos, pois o cordoma tende a apresentar um comportamento agressivo, pois invade tecidos adjacentes e estruturas neurovasculares. Atualmente, um dos principais desafios da sacrectomia é equilibrar uma ampla ressecção com a preservação da função neurológica do paciente. Apesar dos casos de ressecção total bem-sucedida, a recorrência local é uma realidade inevitável, e a sobrevida global é relativamente baixa. A indicação de terapias adjuvantes não está bem estabelecida na literatura, uma vez que a resposta à radioterapia não é satisfatória para esses tumores. O objetivo deste trabalho é relatar o caso de um paciente com cordoma sacral (CS) submetido à sacrectomia parcial e fazer uma breve revisão da literatura sobre cordomas sacrococcígeos.
Publication History
Received: 29 April 2023
Accepted: 26 January 2024
Article published online:
26 April 2024
© 2024. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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