Clinical Oncology Letters
https://clinicaloncologyletters.com/article/5c7009940e8825544d8e6fd8
Clinical Oncology Letters
Relato de Caso

Tumor Estromal Extra-Gastrointestinal (E GIST): Relato de caso e revisão da literatura

Extra-gastrointestinal stromal tumor: Case report and review of the literature

Alves GG, Penido BB, Sette CV, Rizzetto EA, dos Santos PE, Manna EF, Camargo EW, Geri A, Palma CG

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Resumo

O tumor estromal gastrointestinal (GIST) é o tumor mesenquimal do trato gastrointestinal mais comum, podendo aparecer por todo o trato, do esôfago ao ânus. É um tumor raro, representando 0.1-3% dos tumores do trato gastrointestinal e tem o estômago como o principal sítio acometido (60-70%). Quando o tumor mesenquimal aparece fora do trato gastrointestinal é então chamado de tumor estromal extra-gastrointestinal (EGIST). Clinicamente se apresentam como grandes massas abdominais, normalmente maiores do que 10 cm, e os principais sintomas são dor abdominal e massa abdominal palpável. A maioria (95%) desses tumores mesenquimais expressa CD117 e 50-80% são CD34 positivos. Relatamos aqui um caso de EGIST de retroperitônio tratado em nosso serviço. Paciente 58 anos, sexo feminino, descobriu massa em retroperitônio devido investigação de anemia. Submetida à laparotomia exploradora. O anátomo-patológico e imunohistoquímica definiu como diagnóstico definitivo de Tumor Estromal Gastrointestinal (GIST) com diferenciação muscular lisa. Paciente iniciou então tratamento com mesilato de Imatinibe 400mg/dia, mantendo-se sem evidência de doença até o momento.

Palavras-chave

Tumores do Estroma Gastrointestinal, GIST, Doenças Raras, Extra-Gastrointestinal, Relatos de Casos

Abstract

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumors of the gastrointestinal tract, it may appear throughout the tract, from the esophagus to the anus. It is a rare tumor, representing 0.1-3% of tumors of the gastrointestinal tract and the stomach has as the main site affected (60-70%). When the mesenchymal tumor appears outside the gastrointestinal tract is so called extra-gastrointestinal stromal tumor (EGIST). Usually present as large abdominal masses, typically greater than 10 cm, and the main symptoms are abdominal pain and palpable abdominal mass. The majority (95%) of these mesenchymal tumors express CD117, and 50-80% are CD34 positive. We report a case of retroperitoneal EGIST treated in our facility. Female patient of 58 year old discovered massive retroperitoneal due investigation of anemia. She underwent exploratory laparotomy. Histopathological and immunohistochemical defined as definitive diagnosis Stromal Tumor Gastrointestinal (GIST) with smooth muscle differentiation. Patients then started treatment with imatinib mesylate 400 mg / day with no evidence of disease so far.

Keywords

Gastrointestinal Stromal Tumors, GIST, Rare Diseases, Extra-gastrointestinal, Case Reports

Referências

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