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Primary and secondary tumors of the vena cava are rare and often malignant. They either originate in the vein wall, invade the walls secondarily, or grow within the vena cava as tumor thrombus. They can cause extrinsic compression of the vein lumen. Most patients have advanced local, regional, or distant metastatic disease at the time of diagnosis. Despite advances in adjuvant therapy, survival is measured in months unless surgical resection can be performed. Similar to most cancers and sarcomas, unless a better understanding of the tumor biology is found, development of effective adjuvant therapies will remain problematic.
The most common primary tumor of the inferior vena cava (IVC) and superior vena cava (SVC) is leiomyosarcoma. Primary venous leiomyosarcoma involves the IVC in 60% of patients, with three fourths of those involving the suprarenal and infrarenal segments ( Figure 1 ). These tumors are polypoid or nodular, are firmly attached to the vessel wall, and show less intratumor hemorrhage or necrosis than other sarcomas. The most common growth pattern is intraluminal, but the tumor can grow through the vein wall into adjacent structures in advanced cases. Such biologic behavior makes it difficult to differentiate a primary venous leiomyosarcoma from other retroperitoneal sarcomas. Metastatic disease to the lung, liver, kidney, bone, pleura, or chest wall occurs in nearly one half of patients by the time diagnosis is made.
Secondary tumors involve the IVC more often than primary tumors. They can affect any segment of the vena cava, and they often infiltrate its wall ( Box 1 ). Some malignancies exhibit intraluminal tumor thrombus as part of their biologic behavior, with renal cell cancer being the most common. Such renal cell cancers tend to be large (>4.5 cm), and they more often involve the right kidney. Thrombus is isolated to the renal vein–caval confluence in 50%; another 40% have thrombus in the suprarenal IVC, and 10% have thrombus extending into the right heart.
Primary leiomyosarcoma
Secondary superior vena cava tumors
Lung cancer with mediastinal adenopathy
Lymphoma
Follicular or medullary thyroid cancer
Teratoma
Thymoma
Angiosarcoma
Synovial cell carcinoma
Secondary inferior vena cava tumors
Retroperitoneal soft tissue tumors
Liposarcoma
Leiomyosarcoma
Malignant fibrous histiocytoma
Hepatic tumors
Cholangiocarcinoma
Hepatocellular carcinoma
Metastatic (e.g., colorectal)
Pancreaticoduodenal cancers
Secondary inferior vena cava tumors that might have tumor thrombus
Renal cell carcinoma
Pheochromocytoma
Adrenocortical carcinoma
Sarcomas of uterine origin
Leiomyomatosis
Endometrial stromal cell
Germ cell tumors
Embryonal
Teratocarcinoma
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