MASAKI OKAMOTO, MASARU MORITA, MASAFUMI
OF INFERIOR VENA CAVA
From the Department of Urology, Ehime University School of Medicine, Ehime, Japan
case of leiomyosarcoma
of the inferior uena caua is presented and pertinent clinical
features of 57 reported cases in the English literature are reviewed.
Leiomyosarcoma arising in the inferior vena cava is rare. Since the first description by Per1 in 1871,’ less than 70 cases have been reported in the literature.2 Herein we report another case of leiomyosarcoma of the inferior vena cava and review pertinent clinical features of reported cases in the English literature. Case Report A sixty-four-year-old woman was admitted to the Ehime University Hospital for evaluation of an abdominal mass in September, 1983. She had noted the presence of the mass for about two years prior to this admission. She denied abdominal pain, gross hematuria, or fever. Family history and medical history were noncontributory. On physical examination the patient was found to be a well-developed, wellnourished woman in no distress. The lungs were clear, and there was no heart murmur. The abdomen was soft, and there was a large nontender firm mass palpable in the right side of the abdomen just lateral to the umbilicus. The mass was slightly movable from side to side but not vertically. Both rectal and vaginal examinations were negative. There was no edema of the legs and no evidence of venous engorgement. Results of routine laboratory tests and chest x-ray films were normal.
An excretory urogram showed a soft tissue mass inferior to the right kidney with lateral and superior displacement of the kidney (Fig. 1A). A computerized axial tomogram of the abdomen demonstrated a large mass medial to the right kidney involving the inferior vena cava (Fig. 1B). On arteriography the tumor appeared hypervascular receiving its blood supply from a lumbar artery (Fig. 1C). An inferior venacavogram revealed an intraluminal filling defect and narrowing of the cava (Fig. 1D). Findings on liver and bone scans were negative. From these studies a preoperative diagnosis of retroperitoneal tumor with involvement of the inferior vena cava was made. An operation was performed on October 19, 1983. The peritoneal cavity was entered through a midline incision. A large tumor could be palpated in the retroperitoneal space. There was no evidence of metastatic disease in the abdominal viscera. The retroperitoneum was opened, and circumferential dissection done around the mass, which then appeared to arise from the inferior vena cava below the level of the renal veins. A mass could also be palpated inside the lumen of the inferior vena cava. The inferior vena cava was controlled proximally and distally, and the tumor with a segment of the inferior vena cava was resected en bloc.
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FIGURE 1. (A) Excretory UTOgram shows soft tissue mass inferior to right kidney with lateral and superior displacement of kidney. (B) Computerized axial tomogram shows large mass medial to right kidney involving inferior vena cava. (C) Lumbar arteriogram demonstrates hypervascular tumor. (D) Inferior venacavogram shows narrowing of cava and intraluminal filling deject.
Postoperative course was uneventful. Leg edema did not develop, and the patient was discharged eighteen days after surgery. Two months later she was doing well with no evidence of tumor recurrence. Pathologic
ture. The ratio of female to male patients was 3.4 to 1. Ages ranged from twenty-four to eighty-three years, with the peak incidence in the seventh decade (28 % ) . Presenting signs and symptoms depend on the location and extent of the tumor, degree of venous obstruction, and the presence or absence
The specimen consisted of a bosselated mass measuring 9 by 9 by 5 cm with a segment of inferior vena cava and weighed 310 Gm. The tumor was composed of interlacing bundles of elongated spindle-shaped cells with eosinophilic cytoplasma and blunt-ended nuclei (Fig. 2). There were scattered multinucleated cells, but mitoses were few. Electron microscopy and special stains confirmed tumor cells to be of smooth muscle origin. Histologic diagnosis was a well-differentiated leiomyosarcoma. Comment We collected 57 cases of leiomyosarcoma of the inferior vena cava from the English litera-
FIGURE 2. Microscopic appearance of tumor
of thrombosis.3 Based on the highest extension of tumor, 23 tumors involved the upper third of the inferior vena cava (from the hepatic veins to the right atrium). Budd-Chiari syndrome with ascites, hepatomegaly, and progressive liver failure due to hepatic vein occulsion was noted in 20 cases (87 % ). mmor extension into the right atrium causing tricuspid valve obstruction or into the pulmonary artery with pulmonary emboli, has been reported.4*5 Eighteen tumors were located in the middle third of the inferior vena cava (from the renal to the hepatic veins). Abdominal pain was the most frequent symptom (61%) and an abdominal mass was palpable in 7 cases (39 % ). Although several authorse-g reported importance of signs and symptoms of renal vein thrombosis in tumors located in the middle third, this appears to be of theoretical possibility only, and proteinuria, nephrotic syndrome, or renal failure were not well documented in reported cases. Sixteen tumors involved the lower third of the inferior vena cava (from the bifurcation to the renal veins). The majority of tumors at this level were palpable (81% ), and abdominal or back pain was also a frequent symptom (69%). Preoperative diagnosis of leiomyosarcoma of the inferior vena cava is difficult unless one has a high index of suspicion. Excretory urography and ultrasonography usually suggest the presence of a solid retroperitoneal tumor. Inferior venacavography appears to be the most useful diagnostic procedure, and it will reveal the anatomic location and extent of the tumor.3,10 Computerized axial tomography is also useful and will demonstrate the tumor size and its relationship to surrounding organs. l1 If arteriography is performed, hypo- to moderately vascular tumors will be demonstrated. The best treatment is complete surgical excision. In 30 of 57 reported cases tumors were surgically resected (53 % ): in 28 of 30 cases the tumor was completely resected, and in the remaining 2 cases resection was partial. All of these tumors were located either in the middle or lower third of the inferior vena cava. Unfortunately no tumor originating in the upper third was resected. When the middle third of the inferior vena cava is removed along with the tumor, the right kidney must be resected or autotransplanted to the iliac fossa. The left kidney has sufficient collateral channels to survive. Infrarenal vena cava can be resected safely since an abundant collateral venous supply will have developed in most cases. Of 28
completely resected cases, 15 (54 % ) were alive with no evidence of tumor in the follow-up period ranging from two to twenty-seven months. In 9 cases (32 % ) either local recurrence or distant metastases developed, usually to the liver or the lung, in five to forty-eight months. No follow-up information was available in the remaining 4 cases. Of 2 partially resected cases, 1 patient was alive at six months and the other died of liver failure at three months postoperatively. Varela-Duran, Oliva, and Rosai13 believe that the mitotic index is the most important pathologic feature on which prognostic evaluation for leiomyosarcoma can be based. The higher the mitotic count per ten high-powered fields, the greater the possibility of me&static disease developing. Although the place of radiotherapy and chemotherapy has not been well established in the treatment of leiomyosarcoma of the inferior vena cava,2 the adjunctive use of postoperative radiotherapy to the tumor bed or chemotherapy to prevent local recurrence or distant metastasis may be indicated in those patients whose tumors show high mitotic count. Ehime, Japan (DR. OCHI) References 1. Per1 L: Ein Fall von Sarkom der Vena cava inferior, Virchows Arch Path01 Anat 53: 378 (1871). 2. Skooe ST. McLeod DG. Stutzman RE. and Bloom DA: Leiomyos&o”&a of the inferior vena cava l&senting as a suprarenal mass, J Urol 130: 760 (1983). 3. Brewster DC, Athanasoulis CA, and Darling C: Leiomyosarcoma of the inferior vena cava, Arch Surg 111: 1081 (1976). 4. Hoffbrand AV, and Lloyd-Thomas HG: Leiomyosarcoma of the inferior vena cava leading to obstruction of the tricuspid valve. Br Heart 126: 709 (1964). 5. Demoulin jC, et al: ‘Leiohyosarcoma of the inferior vena cava; an unusual cause of pulmonary embolism, Chest 66: 597 (1974). 6. Halloch P, Watson CJ, and Berman L: Primary tumor of inferior vena cava, with clinical features suggestive of Chiari’s disease, Arch Intern Med 66: 50 (1940). 7. Kalsbeek HL: Leiomyosasrcoma of the inferior vena cava, Arch Chir Neerl 26: 35 (1974). 8. llxner AR: Leiomyosarcoma of the inferior vena cava, Clin Oncol4: 187 (1978). 9. Pate1 JK, and Englander LS: Leiomyosarcoma of the inferior vena cava, J Surg Oncol21: 238 (1982). 10. Wray RC, and Dawkins H: Primary smooth muscle tumors of the inferior vena cava, Ann Surg 174: 1009 (1971). 11. Chauhan MA, et al: Leiomyosarcoma of the inferior vena cava; angiographic and computed tomography findings, Cardiovasc Intervent Radio1 4: 2009 (1981). 12. Nyman U, Hellekant C, ahd Jdnsson K: Angiography in leiomyosarcoma of the inferior vena cava-report of two cases, Br J Radio1 52: 273 (1979). 13. Varela-Duran J, Oliva H, and Rosai J: Vascular leiomyosarcoma: the malignant counterpart of vascular leiomyosarcoma, Cancer 44: 1684 (1979).