Lelomyosarcoma of the Inferior Vena Cava: Report of a Case and Review of the Literature RALPH V. BAILEY, M.D.,* JOSEPH STRIBLING, M.D.,t STANLEY WEITZNER, M.D.,4 JAMES D. HARDY, M.D., F.A.C.S.§

Leiomyosarcoma of the inferior vena cava is a rare and usually fatal disease. Only 45 cases have been reported in the world literature. The authors add one case and review all previous reports. The disease is one primarily of elderly women, arising from the middle third of the inferior vena cava with a wide range of symptomatology, varying from the Budd-Chiari syndrome to a few asymptomatic cases. The tumor may grow very slowly or occasionally very rapidly, shows extensive local invasion, and metastasizes more frequently than previously believed. While radiotherapy and chemotherapy have not been adequately studied, aggressive surgical intervention using the recent advances in vascular surgery appears to offer long term palliation and hopefully cures of this disease in the future.

From the University of Mississippi Medical Center,

Jackson, Mississippi Case Report K.W., a 67-year-old Caucasian woman, was admitted for the first time to the University of Mississippi Medical Center in July, 1975. She had noted a 20 pound weight loss since March, 1974, when she underwent a cholecystectomy in another hospital. At that time exploration of the abdomen was reported to have been negative with the exception of a smooth, rounded liver edge. She complained of early satiety and occasional loose bowel movements of two weeks duration. Evaluation by her referring physician revealed normal barium enema, normal upper G. I. series with the exception of displacement of the stomach to the left by a large mass, and liver scan showing a large defect in the left lobe. Review ofthe percutaneous needle biopsy ofthe mass was interpreted as a low grade malignant tumor of smooth muscle origin. Past history was remarkable only in that she had a partial thyroidectomy at age 23 and an appendectomy at age 37. Review of systems revealed no history of jaundice or alcohol intake. Her hypertension of 15 years duration was controlled by Aldomet. She smoked and had mild symptoms of chronic bronchitis. The patient was

LEIOMYOSARCOMA of the inferior vena cava is a rare tumor which is usually lethal. Though reports of this tumor have been increasing in frequency in the last two decades, we have been able to find only 45 verified cases in the world literature1-43 since it was first described by Perl in 187 1.7 The purpose of this paper is to present a new case, review the literature, and summarize the clinical findings and natural history of leiomyosarcoma of nulliparous. the inferior vena cava. Submitted for publication December 15, 1975. Chief Resident, Department of Surgery. t Assistant Resident, Department of Surgery. t Associate Professor and Director of Surgical Pathology, Department of Pathology. § Professor and Chairman, Department of Surgery. Reprint requests: James D. Hardy, M.D., Professor and Chairman, Department of Surgery, 2500 North State Street, Jackson, Mississippi 39216. *

Physical examination. A large, nontender mass was present in the mid-epigastrum which was irregular in contour and seemed to be attached to the liver. Pelvic examination showed a nulliparous cervix and no palpable abnormalities of the uterus. The extremities showed no cyanosis, varicosities or edema. The remainder of the physical examination was essentially unremarkable. Laboratory Data. Alkaline phosphatase and LDH were slightly elevated at 152 u/L (normal 30-85) and 462 u/L (normal 90-200) respectively. SGOT was normal at 25 u/L (nornal 20-50). Hemoglobin was 10.9 mg/100 ml and trace protein was in her urine. Hospital Course. Aortic arteriography showed a large upper

169

170

BAILEY AND OTHERS

FIG. 1. In situ photograph of the tumor showing its relationship to the right lobe of the liver supported by a thumb (upper left). mid-abdominal mass located anteriorly and primarily supplied by the hepatic artery. The celiac, splenic and hepatic arteries were markedly stretched and displaced inferiorly and anteriorly. Neovascularization was apparent.

At laparotomy, the liver was draped over a large mass (Fig. 1) which appeared to arise from the mid-portion of the inferior vena cava between the renal and hepatic veins. The common bile duct, hepatic artery and

Ann.

Surg. August 1976

FIG. 2. Note the fleshy nodules of tumor and hemorrhagic areas of the cut surface of the

leiomyosarcoma.

portal vein were stretched tightly over the tumor and were "peeled off" by blunt and sharp dissection. The tumor was resected with the anterior wall of the inferior vena cava, which was then repaired. No hepatic metastases of sites of extension were found. No uterine leiomyomata were palpable. Her hospital course was complicated by a common duct stricture, for which a Roux-en-Y choledochojejunostomy was performed with good

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171

LEIOMYOSARCOMA OF THE VENA CAVA

Vol. 184 * No. 2

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