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The key role of surgery in the management of retroperitoneal sarcomas : a case report of a rare undifferentiated pleomorphic sarcoma
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Resumo(s)
Os tumores retroperitoneais são neoplasias raras e complexas que constituem 15% dos sarcomas de tecidos moles e menos de 1% das neoplasias em adultos. Estes tumores frequentemente invadem órgãos adjacentes e estruturas vasculares major. Devido à sua raridade, é essencial que a abordagem a este tipo de doentes seja realizada em contexto multidisciplinar em centros de referência com um elevado volume de casos. A resseção cirúrgica completa é, até ao momento, o único fator relacionado com o tratamento que exerce uma forte influência na sobrevida global. Pretende-se, então, descrever o caso de um doente com um raro sarcoma pleomórfico indiferenciado com com envolvimento vascular e ureteral, tendo sido necessária resseção tumoral bem como ressecções vasculares major e reconstrução arterial. Pretende-se também avaliar o papel chave da cirurgia e o papel de outras opções terapêuticas na abordagem a estes doentes. Em conclusão, a resseção cirúrgica en bloc do massa tumoral é o gold-standard na gestão dos sarcomas retroperitoneais e não existe ainda consenso acerca de qual extensão da resseção completa e se a radioterapia pode ou não ter um papel complementar à cirurgia.
Retroperitoneal sarcomas are rare, complex neoplasms that comprise approximately 15% of all soft tissue sarcomas and less than 1% of all adult cancers. These rare tumors often involve adjacent organs and major blood vessels. Due to its rarity, it is essential that the approach to these patients must be performed in the multidisciplinary setting of high-volume sarcoma reference centers. Complete surgical resection is so far the only treatment-related factor that exert a strong influence on overall survival. Here, we report the case of a patient with a large, rare undifferentiated pleomorphic sarcoma with vascular and ureteral involvement that required resection as well as major vascular resections and arterial reconstruction. We address the key role of surgery and the role of other treatment options in the approach of these patients. In conclusion, complete en bloc surgical resection of the tumor is the gold-standard for the management of retroperitoneal sarcomas and there is still no consensus about the extension of resection and whether radiotherapy can complement or not the role of surgery.
Retroperitoneal sarcomas are rare, complex neoplasms that comprise approximately 15% of all soft tissue sarcomas and less than 1% of all adult cancers. These rare tumors often involve adjacent organs and major blood vessels. Due to its rarity, it is essential that the approach to these patients must be performed in the multidisciplinary setting of high-volume sarcoma reference centers. Complete surgical resection is so far the only treatment-related factor that exert a strong influence on overall survival. Here, we report the case of a patient with a large, rare undifferentiated pleomorphic sarcoma with vascular and ureteral involvement that required resection as well as major vascular resections and arterial reconstruction. We address the key role of surgery and the role of other treatment options in the approach of these patients. In conclusion, complete en bloc surgical resection of the tumor is the gold-standard for the management of retroperitoneal sarcomas and there is still no consensus about the extension of resection and whether radiotherapy can complement or not the role of surgery.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2018
Palavras-chave
Sarcomas retroperitoneais Sarcoma pleomórfico indiferenciado Radioterapia Resseção cirúrgica