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Carcinoid tumors are low-grade malignant tumors that arise from neuroendocrine cells. Primary renal carcinoid tumors are extremely uncommon. They seem to be more indolent than renal cell carcinomas, although metastases to regional lymph nodes, liver, and bone have been described. The presence of metastases seems to indicate a more malignant course; however, even with metastases a patient might live for 3 or 4 years. Renal carcinoid tumors should be managed by radical or partial nephrectomy, and good outcomes have been obtained for organ-confined disease after radical excision. Conventional methods of imaging are inadequate for detecting smaller carcinoids, so somatostatin receptor scintigraphy should complement computed tomography and magnetic resonance imaging when searching for occult or metastatic disease. Close follow-up after surgery is necessary.

Leiomyosarcoma of the inferior vena cava (IVC) is an extremely rare entity. We present the case of a 62-year-old woman who was found to have a large right upper quadrant mass upon examination by her primary care physician in evaluation for diffuse abdominal pain accompanied by anorexia and weight loss. A computed tomographic scan and magnetic resonance imaging demonstrated a 13-cm retroperitoneal lesion that appeared to stem from the right kidney and yielded a tumor thrombus up to the level of the hepatic venous confluence. The patient underwent a right radical nephrectomy and IVC thrombectomy for treatment of a presumed renal cell carcinoma. Instead, pathology revealed the tumor to be a leiomyosarcoma of the IVC. We document this unusual presentation of an extremely rare tumor entity.