José Antonio López-Ruiz1, Luis Tallón-Aguilar2, Beatriz Marenco-de la Cuadra2, José López-Pérez2, Fernando Oliva-Mompeán2, Javier Padillo-Ruiz3. 1. Unidad de Cirugía de Urgencias y Cirugía Oncológica Multidisciplinar, Unidad de Gestión Clínica de Cirugía General y Aparato Digestivo, Hospital Universitario Virgen Macarena, Sevilla, España. Electronic address: wurdalach@gmail.com. 2. Unidad de Cirugía de Urgencias y Cirugía Oncológica Multidisciplinar, Unidad de Gestión Clínica de Cirugía General y Aparato Digestivo, Hospital Universitario Virgen Macarena, Sevilla, España. 3. Unidad de Gestión Clínica de Cirugía General y del Aparato Digestivo, Hospital Universitario Virgen del Rocío, Sevilla, España.
Abstract
BACKGROUND: Large vessel sarcomas are rare tumours. Leiomyosarcoma of the inferior vena cava is the most common. About 300 cases have been reported in the literature. They tend to be large, and not develop metastasis. The prognosis of these tumours is poor. CLINICAL CASE: An 81 year-old woman who complained of pain in the right flank, with no other symptoms. Abdominal computed tomography showed a large retroperitoneal mass, which affected the inferior vena cava, with signs of thrombosis inside. It also encompassed the right renal vein and the right kidney. Excision of the tumour was performed in block, performing an autologous saphenous vein bypass between left the renal vein and proximal segment of inferior vena cava. DISCUSSION: Leiomyosarcomas of the inferior vena cava are classified according to their relationship with adjacent structures. The clinical signs and symptoms are generally non-specific. Diagnosis is made using computed tomography or magnetic resonance imaging, and biopsy of the retroperitoneal mass. Surgery is the only treatment capable of providing prolonged survival. The surgical management is determined by: the level of involvement, the extension, and the presence or absence of collateral veins. The role of adjuvant therapy is controversial. CONCLUSIONS: Inferior vena cava leiomyosarcomas remain a challenge for surgeons. At present, radical resection with negative margins, offers the highest survival rate. The best results are obtained with a multidisciplinary approach by experienced teams in the management of these tumours.
BACKGROUND: Large vessel sarcomas are rare tumours. Leiomyosarcoma of the inferior vena cava is the most common. About 300 cases have been reported in the literature. They tend to be large, and not develop metastasis. The prognosis of these tumours is poor. CLINICAL CASE: An 81 year-old woman who complained of pain in the right flank, with no other symptoms. Abdominal computed tomography showed a large retroperitoneal mass, which affected the inferior vena cava, with signs of thrombosis inside. It also encompassed the right renal vein and the right kidney. Excision of the tumour was performed in block, performing an autologous saphenous vein bypass between left the renal vein and proximal segment of inferior vena cava. DISCUSSION: Leiomyosarcomas of the inferior vena cava are classified according to their relationship with adjacent structures. The clinical signs and symptoms are generally non-specific. Diagnosis is made using computed tomography or magnetic resonance imaging, and biopsy of the retroperitoneal mass. Surgery is the only treatment capable of providing prolonged survival. The surgical management is determined by: the level of involvement, the extension, and the presence or absence of collateral veins. The role of adjuvant therapy is controversial. CONCLUSIONS: Inferior vena cava leiomyosarcomas remain a challenge for surgeons. At present, radical resection with negative margins, offers the highest survival rate. The best results are obtained with a multidisciplinary approach by experienced teams in the management of these tumours.