Primitive neuroectodermal tumor of the kidney with inferior vena cava tumor thrombus during pregnancy response to sorafenib.

Abstract

A 26-year-old primigravida in the 37th week of gestation was admitted to the West China Hospital at the end of December 2007 with severe hematuria and pain in the right flank that had persisted for about one month. Color Doppler sonography demonstrated a large substantive lump on the right kidney. Routine blood tests showed severe anemia, while other laboratory parameters and her medical history were uneventful. An urgent Caesarian section was performed. The newborn was checked and found to have no abnormalities. One week later, a contrast enhanced computed tomography of the abdomen confirmed a large heterogenous mass of 11 cm × 10 cm × 9 cm in size with multiple areas of necrosis and hemorrhage involving two thirds of the right kidney except the lower pole (Figure 1). A venous thrombus was reported to distend the right renal vein and extend into the IVC. Thoracic and pelvic CT scan did not reveal any metastasis. From a history we learned that from the medial duration of pregnancy, the patient began to feel the right flank pain. Unfortunately, she presumed that the flank pain was due to pregnancy, so she did have any urological related examination until she was in labor. Radical nephrectomy was performed two weeks after the Caesarian section. In the operation, the mass resection was successful, and exploration confirmed a level III (infra-diaphragmatic IVC) tumor thrombus 7 cm in length. Fortunately, the wall of the IVC was not involved. The venous thrombus was extracted carefully and entirely.