Aggressive Resection for a Primary Leiomyosarcoma of the Vena Cava Masquerading as a Pancreatic Head Tumor.

Smooth muscle is a normal component of the inferior vena cava (IVC) wall. Although uncommon, the smooth muscle component may undergo neoplastic change. Benign neoplasms are termed leiomyomas, and when there is a malignant change, the nomenclature is changed to an IVC leiomyosarcoma. Leiomyosarcomas of the IVC are rare, with less than 150 cases reported in medical literature. Unfortunately, the majority of IVC leiomyosarcomas are diagnosed at advanced disease stages. Surgical resection of locally advanced lesions is technically challenging, but complete resection is the mainstay of treatment as leiomyosarcomas respond poorly to chemo-radiotherapy. Due to the advanced disease stage at diagnosis and the technical complexity of IVC resection and reconstruction, most patients are transferred to high-volume centers in developed nations. We report a case of a patient with a locally advanced leiomyosarcoma masquerading as a pancreatic head tumor. This patient could not access care in a high-volume center and required aggressive maneuvers to resect the IVC leiomyosarcoma in a resource-poor, low-volume center. We present this case to highlight the steps in operative management and also to show that these procedures can be carried out in resource-poor environments once there is meticulous planning, appropriate equipment, and multidisciplinary care.

Xanthogranulomatous pyelonephritis

Xanthogranulomatous pyelonephritis (XGP) is a severe form of renal infection which may be associated with diagnostic problems. Pathologically, it is characterized by renal enlargement, hydronephrosis, lithiasis and xanthomatous masses, which replace the kidney parenchyma, accompanied by granulomatous inflamation. This presentation describes a review of 15 cases of XGP, seen by our surgical pathology unit over a 20 year period (1972-1992). Eleven cases were in females and 4 in males. The preoperative diagnosis included pyelonephritis (11 cases, 4 associated with calculi); 2 other cases were diagnosed as tuberculosis and 2 as cell carinoma. Left-sided lesions predominated (11:4). The youngest patient was 24 years old, and the eldest a woman of 67 years. The weight of the nephrectomy specimens ranged from 120g - 2060g. The largest kidney measured 23 x 18 x 10 cm. Microscopically, there was extensive destruction of renal parenchyma with interstitial infiltrates of acute and chronic inflammatory cells, cholesterol clefts, numerous foreign body type giant cells and foamy histocytes. Because of the possible confusion with even more serious disease states such as tuberculosis and renal carcinoma, this distinctive lesion is worthy of attention. A literature review indicates that a good result is likely following nephrectomy. (AU)