Leiomyosarcoma of the Inferior Vena Cava: Report of Two Cases and Review of the Literature

Abstract

Leiomyosarcoma of vascular origin is a rare and usually lethal tumor. It arises more commonly from large veins than arteries, and more than one half occur in the inferior vena cava.1 Most of these tumors are diagnosed at autopsy or laparotomy. Surgical resection offers the best prospect of cure; however, the majority of patients die from metastatic lesions into the liver and lungs.The purpose of this report is to describe two cases of leiomyosarcoma of the inferior vena cava. Case Reports Case 1A 35-year-old Saudi woman was admitted with a 14-year history of an intermittent dull ache in the right hypochondrium. The pain had no relationship to meals, and there was no history of weight loss or other relevant complaints. Physical examination revealed a large, nontender, firm abdominal mass in the right flank. Results of routine hematological investigations, including complete blood cell count, erythrocyte sedimentation rate, urea, and electrolytes, and liver function tests were all normal. Ultrasound examination of the abdomen revealed a 9 × 5 × 9-cm solid mass in the right flank and a single stone in the right kidney. The liver and spleen appeared normal, and there were no other masses in the abdomen or pelvis. Findings on subsequent radiologic investigations, which included chest x-ray, liver and spleen isotope scan, intravenous urogram, and barium enema, were all reported normal.At laparotomy the mass was retroperitoneal, arising from the inferior vena cava at the level of the right renal vein and extending superiorly to within 2.5 cm of the most inferior hepatic vein. The left renal vein was not involved in the tumor mass. The inferior vena cava was completely occluded. The mass was separated from surrounding structures by sharp and blunt dissection, then completely resected together with the right kidney. The two ends of the inferior vena cava were closed with a continuous suture. There were no other abnormal findings in the abdomen. Postoperative recovery was uneventful. Histopathologic examination of the resected mass showed leiomyosarcoma arising from the inferior vena cava.The patient is well and alive 23 months later with no signs of metastases or lower limb edema, and renal function is normal. Case 2A 55-year-old Saudi woman was admitted for further investigations of right flank pain, which was vague in nature and associated with indigestion and increasing constipation of 7 months' duration. Three months before admission, there was increasing puffiness of the face and swelling of both lower limbs. There was no history of jaundice or shortness of breath, nor was there any history of gynecologic problems. The patient was a known diabetic and had been treated with oral hypoglycemic drugs for the last 9 years. She was moderately obese, and apart from the puffiness of the face and minor edema of the lower limbs, the physical examination was normal.Laboratory studies showed normal blood cell count, but the ESR was high at 66 mm/h. Blood urea and creatinine were elevated at 12 mmol/L and 185 mmol/L, respectivel...