VENA CAVA LEIOMYOSARCOMA DEVELOPING BUDD CHIARI SYNDROME: CASE REPORT

• Main Author: Puppo, Mariana Brasileiro Vieira
• Publication Date: 2023
• Other Authors: Rozete, Felício Stenio Schuenck, Tardin, Rovena Campana, Oliveira, Paula Morette de, Rueda-Dantas, Mateus Andre, Storch, Layni, Batista, Danielle Cristina Filgueira Alves, Lirio, Ludmilla Ventura, Wandekoken, Kethleen Gomes, Pinto, Caroline Alcure, Lopes, João Eugênio Loureiro, Gonçalves, Luciana Lofego, Zago-Gomes, Maria da Penha
• Format: Article
• Language: por
• Source: Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online)
• Download full: http://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/986
• topic: Leiomiosarcoma; veia cava inferior; Síndrome de Budd Chiari; dor abdominal; neoplasia retroperitoneal; Leiomyosarcoma; inferior vena cava; Budd Chiari syndrome; abdominal pain; retroperitoneal neoplasms

Vena cava leiomyosarcoma is a rare neoplasm, usually divided into 3 types: Type I- below the renal vein, Type II- between the renal and hepatic veins and Type III - between hepatic veins and right atrium. In Type III venous drainage of the liver is compromised and may be the etiology of Budd Chiari Syndrome. Radiological imaging is very characteristic and may be the key to diagnosis. The aim of this case report is to present a 68-year-old patient, male, with severe abdominal pain associated with ascites, lower limb edema and portal hypertension compatible with Budd Chiari Syndrome. Tomography showed infiltrative image in the inferior vena cava pathway extending to the caudate lobe of the liver and histology showed low-grade leiomyosarcoma, confirmed by immunohistochemistry. Knowing the clinical and radiological picture of vena cava leiomyosarcoma allows doing the diagnosis and guiding the treatment and prognosis, reducing the economic and emotional costs.