Primary venous leiomyosarcoma: A rare but lethal disease

ORIGINAL ARTICLES

Primary venous leiomyosarcoma: A rare but lethal disease C s a b a Dzsinich, M D , P h D , P e t e r Gloviczki, M D , J o n A. v a n H e e r d e n , M D , C h B , D a v i d M. N a g o r n e y , M D , P e t e r C. Pairolero, M D , C. Michael J o h n s o n , M D , J o h n W. Hallett, Jr., M D , T h o m a s C. Bower, M D , and K e n n e t h J. C h e r r y , Jr., M D ,

Pochester, Minn. Primary venous leiomyosarcoma is rare, only 197 patients have been reported. To examine progress in diagnosis, treatment, and clinical outcome , we retrospectively reviewed data of 13 patients, 12 women and one man (mean age, 55 years; range, 19 to 75 years), who in the last 35 years underwent surgical treatment for primary venous leiomyosarcoma at our institution. The tumor arose from the inferior vena cava in eight, iliac vein in two, ovarian vein in one, and greater saphenous vein in two patients. Primary venous hfiomyosarcoma was detected by physical examination in nine patients, symptoms were present in six. The 13 patients underwent 16 surgical procedures to resect primary (12), recurrent (2), or metastatic (2) tumors. Local excision alone was performed in seven, and local excision with reconstruction of the inferior vena cava or iliac vein was performed in six patients. The tumor was greater than 10 cm in eight patients. The perioperative mortality rate was 15% (2 of 13). Median survival of the 11 early survivors was 3.5 years (Jrange, 6 months to 17 years). Currently five patients are alive (four of them free of known ~amor), with a median survival of 3 years (6 months to 17 years) after surgery. O f the eight deceased patients, primary venous leiomyosarcoma recurred after resection in six. Tumor recurrence was not affected by tumor grade, size, or adjuvant treatment. Although early detection with modern imaging techniques could potentially be of benefit, wide local excision with selective venous reconstruction affords the only hope for prolonged survival. (J VASC SURG 1992;15:595-603.)

Primary tumors arising from the smooth muscle cells o f the vessel wall are rare. Soft tissue sarcomas represent less than 1% o f all malignancies, and leiomyosarcomas constitute 6% o f these tumors. 1'2 Vascular leiomyosarcomas constitute 2% o f all leiomyosarcomas and involve veins five times more than arteries. 3 Since Perl 4 described the first case in an autopsy report in 1871, 197 cases o f primary venous leiomyosarcomas (PVLs) have been published as single case reports or as small series. Because venous leiomyosarcomas are generally asymptomatic, diagnosis and treatment are frequently delayed, resulting From the Department of Surgery and the Department of DiagnosrSc Radiology (Dr. Johnson), Mayo Clinic and Mayo Foundation, Rochester, Minn. Presented at the Third Annual Meeting of the American Venous Forum, Fort Lauderdale, Fla., Feb. 21-22, 1991. Reprint requests: Peter Gloviczki,MD, Mayo Clinic, 200 1st St. SW, Rochester, MN 55905. 24/1/34346

in p o o r long-term survival. Recent reports o f radical resection o f P V L followed by successful venous reconstruction have p r o m p t e d us to review our experience, s,6 PATIENTS AND METHODS Between June 1, 1957, and Dec. 31, 1990, 13 patients with P V L were evaluated at the Mayo Clinic (Table I). Age a n d sex d i s t r i b u t i o n Age at the time o f diagnosis ranged from 19 to 75 years (median, 55 years). There were 12 female and 1 male patient. Clinical p r e s e n t a t i o n The t u m o r was discovered incidentally in two o f the 13 patients. An obstructing leiomyosarcoma o f the greater saphenous vein was found during strip595

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Table I. Clinical data of 13 patients with primary venous leiomyosarcoma Patient no.

Age (years)

Gender

Site of tumor

Resection

Venous reconstruction

Local recurrence

1 2 3 4 5 6 7 8 9 10 11 12 13

75 48 60 63 70 55 70 54 36 30 74 42 19

F F F F F F M F F F F F F

IVC OV IVC IVC GSV IVC IVC GSV IVC IVC IVC IVC IV

Attempted Complete Partial Complete Complete Complete Complete *Complete Complete Complete Complete Complete Complete

ePTFE graft Lateral suture Lateral suture Lateral suture Dacron patch Spiral vein graft

Yes -Yes -

OV, Ovarian vein;/V, iliac vein; GSV, greater saphenous vein. ~Resection performed elsewhere.

Table II. Diagnostic studies in 13 patients with primary venous leiomyosarcoma Diagnostic test Abdominal radiography Intravenous pyelography Ultrasonography Computed tomography Magnetic resonance imaging Arteriography Phlebography

No. of patients studied

No. of positivestudies

11 11

2 8

2 9 4 1 4

2 9 4 1 4

ping, and a leiomyosarcoma of the ovarian vein was discovered on excretory urography during evaluation for genitourinary infection. In 11 patients, however, signs or symptoms related to the PVL were present. In nine patients the tumor mass was palpable, and six patients were symptomatic. The most frequent complaint was abdominal or back pain (4 patients) and only two patients had lower extremity swelling. None of the patients had significant weight loss at the time of the detection of the tumor. Evaluation

The evaluation of patients has evolved during the time span of the study. The diagnostic tests included plain abdominal films, intravenous pyelography, ultrasonography, CT scanning, magnetic resonance imaging, arteriography, and phlcbography. Table II summarizes the characteristic findings. Only two of 11 plain abdominal films were positive, showing stippled calcification of the tumor. Computed tomography and more recently magnetic resonance imaging identified the tumor mass in all patients

Characteristic findings Stippled calcification Dislocation o f ureter and kidney (6 patients) Obstruction of ureter (2 patients) Abdominal or pelvic mass Abdominal or pelvic mass Abdominal or pelvic mass External iliac artery stenosis Obstruction or constriction o f the inferior vena cava (3 patients) Stenosis of external iliac vein (1 patient)

studied (Fig. 1, A through C). Phlebography confirmed caval invasion by the tumor in three patients (Fig. 2) and high-grade narrowing of the lilac vein in another patient (Fig. 3, A). Iliac artery invasion in this patient was visualized by arteriography (Fig. 3, B). Localization The most frequent site of origin of the primary tumor was the inferior vena cava (IVC) (8 patients), followed by the iliac vein (2 patients), the greater saphenous vein (2 patients), and ovarian vein (1 patient) (Fig. 4). Surgical procedures The 13 patients underwent 16 surgical procedures in our institution to resect primary (12 cases), recurrent (2 cases), or metastatic tumor (2 cases) (Table I). Ten of the primary resections were complete, one was partial, and one was attempted resection of the tumor. The seven venous reconstructions performed in

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P~4mary venous leiomyosarcoma 597

Reoperation

Ol~tco~ncof venou~ reconstruction

Clinical Outcome

-

--

--

Intraoperative death

Lung, hip, femur Lung Liver Lung Liver, disseminated -

Lobectomy Local excision Lobcctomy Local excision, ePTFE graft Graft thrombectomy

T h r o m b o s e d at 3 m o P a t e n t at 7 yr P a t e n t at 7.5 yr Patent at 9 mo P a t e n t ~t 1 m o T h r o m b o s e d a t 2 days S t e n o s e d at 3 m o +

Metastases

six patients after resection of the primary (6) or recurrent (1) tumor involved the IVC in six cases and the iliac wfin in one. The IVC was reconstructed by lateral suture repair in three, Dacron patch repair in one, and expanded polytetrafluoroethylene (ePTFE) interposition graft in two cases. The repair involved the suprarenal segment of the IVC in three patients (lateral suture, 2; ePTFE graft, 1). In two of these patients, right nephrectomy was also performed. In the third patient, the left kidney was preserved after resection of the invaded portion of the left renal vein. In three operations the caval repair was infrarenal. Lateral suture repair, Dacron patch repair (Fig. 1, C), and ePTFE interposition grafting were used in one patient each. One patient with PVL of the external iliac vein (Fig. 3,A through B) underwent venous reconstruction with spiral saphenous vein graft. This 19-year-old girl also needed arterial reconstruction because of tumor invasion of the external iliac artery. Adjuvant therapy Postoperative adjuvant therapy was given to five patients. Telecobalt radiotherapy was used prophylactically in one patient and in the treatment of recurrent rumor in four. Three of these patients also received chemotherapy by means of a combination ofmirotic inhibitors (imidazole + carboxamide, cytoxan + adriamycin + cisplatinum, cyclophosphamide + hormone therapy). Pathology In 10 of the 13 patients, the resected tumor was greater thaaa 5 cm. In four patients the size of the tumor was between 5 and 10 cm, in three patients

Died Died Died Alive Died Died Died Died Alive Alive Alive Alive

at at at at at at at at at at at at

11 y r 5 yr 2 yr 17 yr 3 mo 3.5 y r 9 mo 7 yr 7.5 yr 3 yr 6 mo 6 mo

between 10 and 15 cm, and in three it was 15 crn or more. The largest resected minor weighed 950 gin. All primary, recurrent, and metastatic tumors were evaluated by histology, which confirmed the diagnosis of PVL. Preoperative biopsy for tissue diagnosis was not performed in any of the cases. Histologic grading oFmalignancy is shown in Table HI.7 Mitotic activity was not related to clinical outcome. None of the metastatic tumors were of higher histologic grade than the primary.

RESULTS Early deaths One patient died of hemorrhage during attempted ?esection of a large tumor of caval origin. Another patient who underwent resection of an IVC tumor, right nephrectomy, and caval reconstruction with ePTFE graft died at 3 months after operation because 0f stroke, bronchopneumonia, and renal failure. Afltopsy revealed renal vein and IVC thrombosis, with occlusion of the ePTFE graft. Failure of the graft ~vas not considered to be the cause of death !. in this patient. Early morbidity Early thrombosis after venous reconstruction occurred m two of the seven patients. In one patient thrombectomy was performed. Phlebography at 3 months, however, revealed severe narrowing of the femoroiliac spiral vein graft, in spite of a patent i . femoral agtenovenous fistula (Table I).

Late morbidity Metastatic disease developed in five patients. Metastase~ occurred in the lung (3 patients), in the

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Fig. 1. A, Sagittal section ofa magnetic resonance image shows a huge infrahepatic solid mmor (outlined by arrows) displacing the right kidney and the bowels. B, Axial section of the same magnetic resonance image demonstrates the inhomogeneons rumor mass with an indistinct margin and no cleavage plane between the rumor and the IVC. The minor occupies half of the caval lumen (arrow). C, Postoperative magnetic resonance angiogram of the same patient confirms patency of the IVC (arrow), which was reconstructed with a Dacron patch after resection of the tumor of the lower segment.

liver (2 patients), and in the hip and femur (1 patient). Disseminated metastatic disease developed in one o f the patients. Local recurrence o f the resected t u m o r was documented in two patients. Local recurrence or metastases occurred between 3 months

and 6 years (median, 2.25 years) after resection o f the primary rumor. Local recurrence developed in one o f the seven patients w h o underwent local excision o f the t u m o r without venous reconstruction, and four had metastatic disease. Local recurrence o f the rumor

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Primary venous leiomyosarcoma 599

developed in one of the six patients who underwent excision with reconstruction, and one had disseminated metastases. Late survival

Median survival of the 11 patients surviving the initial operation was 3.5 years (range, 6 months to 17 years). Median survival of the seven patients with tumor recurrence or metastases was also 3.5 years (range, 9 months to 11 years). Currently only one of these patients is alive after resection of a recurrent rumor with replacement of the IVC. The other four patients, who are alive without local tumor recurrence or metastasis, have a median survival of 4 years (range, 6 months to 17 years). Four of the five patients who received adjuvant r' ~rapy died within 1.5 and 5 years (median, 2.25 years) after initiating the treatment. The only early survivor from this group received prophylactic radiotherapy after tumor resection and venous and arterial reconstruction. Five patients are alive at present between 6 months and 17 years (median, 3 years) after resection of the primary tumor, and four of these patients are free of known tumor. O f the eight dead patients, PVL recurred after resection either locally or as metastatic disease in six. Late pateney o f venous reconstructions Uttrasonography, phlebography, or magnetic resonance imaging documented postoperative patency of the reconstructed IVC or iliac vein in all five survivors who underwent venous reconstruction after rumor excision. Patency in three patients was confirmed at 1 month, 7 years, and 7.5 years, respectively, after surgery. The fourth patient had a recurrent tumor at 2.25 years after tumor resection and lateral suture of the IVC. The narrowed cava with recurrent tumor was removed and replaced with ePTFE interposition graft. This graft was patent at 9 months after surgery. In the fifth patient, a spiral vein graft, used for iliac vein replacement needed early thrombectomy. The graft was patent, but was severely narrowed at 3 months after operation. DISCUSSION

A review of the literature revealed that 197 patients with PVL have been reported between 1871 and 1991. aa6 Including our 13 patients, the total number of published cases is 210. The youngest reported patient was a 31/2-year-old girl with a femoral vein PVL, 8 and the oldest was an 83-year-old woman with an IVC tumor.9 One half of the patients

Fig. 2. Ascending phlebography of the IVC. The large curvilinear filling defect (arrow) in the infi'arenal segment of the cava was caused by a recurrent primary caval leiomyosarcoma. were in their sevcnth decade of life when the tumor was detected. The mean age was estimated to be 61 years by Bailey et al.,n although the mean age was 55 years in more recent reports sa2 and in our series. Although women and men are almost equally affected by PVL of the extremities, 14 a remarkable female preponderance occurs among patients with leiomyosarcoma of t h e I V C ) 3 All but one of our patients and almost 80% of the reported cases with caval tumors were women. The site of the primary tumor was the IVC in 127 of the 210 reported cases (60%). Retroperitoneal veins with the IVC were affected in about 75% and included the renal, the iliac, the ovarian and spermatic veins, the portal, the splenic, and the inferior mesenteric vein) In the lower extremity the greater saphenous vein is the most frequent site of origin. Including our 2 cases, 19 patients with saphenous PVL have been reported) 4 Despite the remarkable size of many of these tumors, they are often asymptomatic and thus remain undetected for long periods. The most frequent clinical signs and symptoms of retroperitoneal PVL include a palpable mass (39% to 81%), back pain (69%), or abdominal pain (61%). If the suprarenal

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600 Dz:inich etal.

Fig. 3. Ascendingphlebographyofa 19-year-old female patient with primary leiomyosarcoma of the left external iliac vein. Arrow indicates high-grade narrowing, caused by the tumor. B, Arteriography of the same patient shows a moderate stenosis of the external iliac artery (arrow) caused by invasion of the adventitia by the tumor.

segment of the IVC or the renal veins are invaded, renal failure may develop, z6,27 However, because of the rich collateral network of the left renal vein, renal insufficiency is infrequent. None of our patients with renal vein invasion had renal failure. Although our series did not include patients with obstruction of the hepatic veins, this has been reported in 36 patients, ls,25 In these cases the clinical symptoms of both acute or chronic form of BuddChiari syndrome may be present. 1°'1s'23'24 In 17 of these patients the right heart cavities were also invaded. The patients presented with arrhythmia, syncope, or with signs and symptoms of pulmonary embolism, pulmonary hypertension, or right heart failure. Because tumor growth is slow, adequate venous collateral circulation frequently develops, and clinical presentation with signs and symptoms of acute deep venous thrombosis is rare. Lower extremity swelling or chronic venous insufficiency is more frequent if the iliac or femoropopliteal veins are invaded. Primary

tumor of the superficial leg veins usually presents as a painless mobile mass, whereas those arising from deep veins can be fixed to the surrounding tissues. 16.17 Biochemical and hematologic investigations are generally unremarkable, s O f the imaging studies, plain abdominal radiography is seldom helpful, although it may show calcification of the retroperitoneal tumor. Intravenous pyelography frequently reveals displacement of the kidney and ureter or secondary hydronephrosis. Ultrasonography may have fimitations in the retroperitoneum because of overlying intestine, but in the extremity it easily detects the tumor mass and confirms venous thrombosis. If retroperitoneal tumor is suspected, our preference more recently has been to use magnetic resonance imaging for diagnosis. Computed tomography, however, is a suitable alternative. These examinations display the anatomic and structural details of the tumor and adjacent structures with high accuracy. Phlebography is helpful to visualize the extent and degree of venous invasion, to confirm

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Primary venous leiomyosarcoma 601

April 1 9 9 2

L.g

Table III. Histologic grading, local recurrence, and metastatic disease in 13 patients with primary venous leiomyosarcoma 21/£alignancy grade

No. of patients

No. of patients with local recurrence~metastasis

1

2

1

I

1-2 2

l 8

-1

4

2b 3

1 1

-

-

venous thrombosis, to assess collateral venous circulation, and to plan venous reconstruction after radical resection. Although phlebography was performed in only four of our patients in this retrospective study, ""e believe that because of the advantages mentioned, phlebography should be performed if venous reconstruction is planned. Arteriography may occasionally show invasion of the adjacent arterial wall by the rumor. Preoperative tissue diagnosis of the primary tumor was not obtained in any of our patients. Preoperative biopsy by the percutaneous or the transvenous route has been reported only in a small number of patients. Surgical treatment of PVL includes wide local excision of the tumor with free margins. Large size and invasion of adjacent organs, such as the kidneys, adrenal gland or liver, can make the operation technically challenging and may be responsible for increased perioperative morbidity and mortality rates. Nephrectomy, cholecystectomy, or liver resection may be required to obtain free tumor margins, s We performed right nephrectomy in two of the three patients who had suprarenal invasion of the IVC by the rumor. Excision of the segment of the left renal vein close to the IVC can be performed without left nephrectomy, if the adrenal and gonadal veins remain intact. Advances in venous reconstruction have increased the number of patients who may undergo wide resection of the minor with free margins. 5,6 If the IVC is patent and only a portion of the wall is invaded, patency of the cava can be maintained by longitudinal lateral suture or patch angioplasty. If circumferential resection of the cava or iliac vein is required, autologous or prosthetic graft can be used for replacement. 5,6 The suprahepatic and suprarenal IVC requires immediate reconstruction in most patients. If the tumor extends into the right atrium, extracorporeal circulation is necessary for repair. 6 Although we prefer reconstruction of the infrarenal

)V

0per,, ")

2

I

,

-/,o772 .....

)

2/external iliac vein

SVC = Superior v~na cava HVs = Hepatic veins IVC = Inferior vena cava RV = Renal vein G S V = Great saphenoua vein

2

G V

GSV

Fig. 4. Localization of primary venous leiomyosarcoma in 13 patients. The lower (I) segment of the IVC is the infrarenal part, the middle (H) segment involves the entry of renal veins and the cava up to the hepatic veins. The upper (/H) segment extends from the hepatic veins to the right atrium. IVC with ringed ePTFE graft at the time of tumor resection, ligation of the IVC at this level and delayed reconstruction of veins in symptomatic patients is an accepted alternative. ~ We used externally supported PTFE grafts in two patients with caval tumor and a spiral saphenous vein graft in another with PVL of the iliac vein. A femoral arteriovenous fistula, with use of the technique described by us previously, a7was used in one patient only. Two early thromboses occurred. Thrombectomy in one patient resulted in partial early salvage of the graft, which was, however, severely narrowed 3 months later. The natural history of PVL is that of slow but definite progression. Although only one of our patients had evidence of metastases at the time of the initial operation, almost half of the patients probably have metastatic disease at the time of resection of the

602

Dzsinich et al.

primary tumor.11,~3 Solitary metastasis in the lung or liver or late local recurrence of the tumor should be treated surgically. Disseminated metastases occur rarely, and they represent the terminal phase of the disease, n

The sensitivity of the tumor to radiotherapy or to antimitotic agents has never been well proven. In a similar manner we were not able to show clear benefit. Because of the poor prognosis of these patients, however, combined postoperative adjuvant treatment is frequently used and is expected to prevent progression or recurrence of the disease, s'6'12 With the exception of a few cases, PVL in all locations and size is likely to return and will ultimately be the cause of death of the patient. Our patient with tumor free survival 17 years after resection of a saphenous PVL is a rare experience. The longest reported survival after resection ofa caval tumor was 13 years. 36 The median survival of our 11 patients who survived the initial operation was 3.5 years. Survival was not affected by tumor size, location, malignancy grade, and adjuvant therapy. In conclusion, primary leiomyosarcoma of the veins is a rare but frequently lethal disease. The tumor has a slow growing pattern, and the patient may be free of symptoms for a long period. Modern imaging techniques provide earlier and more accurate diagnosis. Wide local excision with selective venous reconstruction appears to offer the only hope for prolonged survival. The authors acknowledge Henry D. Tazelaar, MD, for reviewing the slides of the histologic specimens and Marcia Simonson for her editorial assistance. REFERENCES 1. Lawrence W Jr, Donegan WL, Natarajan N, Mettlin C, Beart R, Winchester D. Adult soft tissue sarcomas. A pattern of care survey of the American College ofSurgeons. Ann Surg 1987;205:349-59. 2. Lefall LD Jr. Soft tissue sarcomas. In: Sabiston DC Jr, ed. Textbook of surgery. The biological basis of modern surgical practice. 12th ed. Philadelphia: WB Saunders, 1981:614-21. 3. Kevorkian J, Cento DP. Leiomyosarcoma of large arteries and veins. Surgery 1973;73:390-400. 4. Perl L. Ein Fall yon Sarkom der Vena Cava Inferior. Virchow's Arch [A] 1871;53:378-83. 5. Kieffer E, Berrod JL, Chomette G. Primary tumors of the inferior vena cava. In: Bergan JJ, Yao JST, eds. Surgery of the veins. New York: Grime and Stratton, 1985:423-43. 6. Kieffer E, Bahnini A, Koskas F. Nonthrombotic disease of the inferior vena cava: surgical management of 24 patients. In: Bergan JJ, Yao JST, eds. Venous disorders. Philadelphia: WB Saunders, 1991:501-16.

Journal of VASCULAR SUP,G~V.Y"

7. Varela-Duran J, Oliva H, Rosai J. Vascular leiomyosarcoma. Cancer 1979;44:1684-91. 8. Allison MF. Leiomyosarcoma of the femoral vein. Report of a case in a child. Clin Pediat 1965;4:28-31. 9. Onerheim WO, Tesluk H. Leiomyosarcoma of the inferior vena cava. Arch Surg 1961;83:395-9. 10. Nagayoshi M, Umetsu K, Tanae E, Nishihara S, Ishibashi K, Ninomura N. A case of leiomyosarcoma of the inferior vena cava. Kyobu Geka 1989;42:155-9. 11. Bailey RV, Stribling J, Weitzner S, Hardy JD. Leiomyosarcoma of the inferior vena cava. Ann Surg 1976; 184:169-73. 12. Griffin AS, Sterchi JM. Primary leiomyosarcoma of the inferior vena cava: a case and review of the literature. J Surg Oncol 1987;34:53-60. 13. Goze A, Bousquet JC, Grellet J, Kieffer E. Les leiomyosarcomes de la veine cave inferieure. J Radiol 1986;67:897910. 14. Humphry M, Neff J, Lin F, Krishnan L. Leiomyosarcoma of the saphenous vein. J Bone Joint Surg 1987;69:282-6. 15. Gareau R. Extraordinary "thrombophlebitis" of the fem%~ vein. Union Med Can 1986;115:53-4. 16. Berlin O, Stener B, Kindblom L-G, Angervall L. Leiomyosarcomas of venous origin in the extremities. Cancer 1984; 54:2147-59. 17. Basu SK, Scott TD, Wilmhurst CC, MacEachem AG, Clyne CA. Leiomyosarcomata of the popliteal vessels: rare primary tumors. Eur J Vase Surg 1988;2:423-5. 18. Leu HI, Makek M. Intramural venous leiomyosarcomas. Cancer 1986;57:1395-400. 19. Pietri J, Abet D, Gamain J, Rakotoariveilo Z, Gruel C. Malignant venous tumors of the limbs. Apropos of 6 cases (in French). J Chir 1988;125:575-81. 20. Payen L, Canlet T, Diebold MD, Pluot M, Patey M, Bauquel J. Leiomyosarcome de la paroi de la veine femorale profonde. Arch Anat Cyt Path 1983;3:233-6. 21. Vaillant A, Tournigand P, Dussarat G, et al. Giant leiomyoma of the tight femoral vein extending to the right heart cavities via the inferior vena cava. Ann Clair 1988;42:620-4. 22. Demoulin JC, Sambon Y, Baudinet V, Beaujean M, Jeukens JM, Delvigne J. Leiomyosarcoma of the inferior vena cava: an unusual cause of pulmonary embolism. Chest 1974;66: 597-9. 23. Mandelbaum I, Pauletto FJ, Nasser WK. Resection of a leiomyoma of the inferior vena cava that produced tricuspid valvular obstruction. J Thorac Cardiovasc Surg 1974;67: 561-7. 24. Hoffbrand AV, Lloyd-Thomas HG. Leiomyosarcoma of the inferior vena cava leading to the obstruction of the tricuspid valve. Br Heart J 1964;26:709-15. 25. Kracht M, Becquemin JP, Anglade MCh, Mathieu D, Hillion ML, Teboul IL. Acute Budd-Chiari syndrome secondary to leiomyosarcoma of the inferior vena cava. Ann Vase Surg 1989;3:268-72. 26. Martin B, Roche A, Menu Y. Diagnostic radiologique des leiomyosarcomes de la veine renale: role de l'angiographie. J Radiol 1986;67:789-95. 27. Farah MC, Shirkoda A, Ellwood RA, Bernacki E, Farah J. Leiomyosarcoma of the renal vein: radiologic pathologic correlation. Clin Imaging 1989;13:323-6. 28. Pelissier E, Sarraz-Bournet B, Monticuquet P, Didier D, Poulat R, Etievent JP. Tumeurs de la veine cave inferieure.

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Diagnostic et traitment. Apropos d'un cas avec revue de la literature. Chirurgie 1985;11!:641-9. Guedon J, Mesnard J, Poisson J, Kuss R. Hypertension renovasculalrepar leiomyosarcomede la veine cave inferieure. Guerison de l'hypertension et survive de 2 ans apres l'intervention chirurgicale. Ann Med Int (Paris) 1970;121: 905-12. Gaudemar M, Dauge MC, Grossin M, Bourgeois P, Bocquet L. Leiomyosarcomes vasculaires peripheriques. Apropos de deux cas. Ann Path 1987;7:56-63, Schildberg FW, Kuntz RM. Leiomyosarkome der Vena Cava Inferior. Thoraxchirurgie 1977;25:28-35. Bousquet J-C, Goze A, Hassan M, Kieffer E, Curet P, Grellet J. Leiomyosarcoma of the inferior vena cava. Ultrasonographic: appearance (In French). J Radiol 1986;67:897910.

33. van Zanten TEG, Golding RP. CT andMR demonstration of leiomyosarcoma of inferior vena cava. J Comput Assist Tomogr 1987;11:670-4. 34. Pollanen M, Butany J, Chiasson D. Leiomyosarcoma of the inferior vena cava. Arch Pathol Lab Med 1987;111:1085-7. 35. Dalton RR, Donohue JH, Mucha PJr, van Heerden JA, Reiman HM, Chen S. Management of retroperitoneal sarcomas. Surgery 1989;106:725-33. 36. KalsbeckHL. Leiomyosarcomaof the inferior vena cava.Arch Chir Neerl 1974;26:35-40. 37. Gloviczki P, Pairolero PC, Cherry KJ, Hallett JW. Reconstruction of the vena cava and its primary, tributaries: a preliminary report. J Vasc SURG1990;11:373-81.

Submitted July 17, 1991; accepted Oct. 11, 1991.

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