Leiomyosarcoma of the inferior vena cava: resection and vascular reconstruction using a dacron graft and an Adam DeWeese clip-three-year follow-up.
ABSTRACT Leiomyosarcomas are rare malignant tumors that particularly affect women. In 2% of all cases, they involve the veins, and in 60% of the cases affecting veins, an involvement of the inferior vena cava (IVC) has been demonstrated. We report a case of IVC leiomyosarcoma operated by resection and reconstruction with a Dacron bypass and apposition of an Adams-DeWeese IVC filter. The latter procedure has never been reported before in association with a graft applied for this disease. Technical and clinical details are described.
- SourceAvailable from: ncbi.nlm.nih.gov[Show abstract] [Hide abstract]
ABSTRACT: Leiomyosarcoma of the inferior vena cava (IVCL) is a rare retroperitoneal tumor. We report two cases of level II (middle level, renal veins to hepatic veins) IVCL, who underwent en bloc resection with reconstruction of bilateral or left renal venous return using prosthetic grafts. In our cases, IVCL is documented to be occluded preoperatively, therefore, radical resection of tumor and/or right kidney was performed and the distal end of inferior vena cava was resected and without caval reconstruction. None of the patients developed edema or acute renal failure postoperatively. After surgical resection, adjuvant radiation therapy was administrated. The patients have been free of recurrence 2 years and 3 months, 9 months after surgery, respectively, indicating the complete surgical resection and radiotherapy contribute to the better survival. The reconstruction of inferior vena cava was not considered mandatory in level II IVCL, if the retroperitoneal venous collateral pathways have been established. In addition to the curative resection of IVCL, the renal vascular reconstruction minimized the risks of procedure-related acute renal failure, and was more physiologically preferable. This concept was reflected in the treatment of the two patients reported on.World Journal of Surgical Oncology 06/2012; 10:120. · 1.20 Impact Factor
Leiomyosarcoma of the Inferior Vena Cava:
Resection and Vascular Reconstruction Using
a Dacron Graft and an Adam De Weese
Domenico Angiletta, Martinella Fullone, Luigi Greco, Davide Marinazzo, Piero Frontino, and
Guido Regina, Bari, Italy
Leiomyosarcomas are rare malignant tumors that particularly affect women. In 2% of all cases,
they involve the veins, and in 60% of the cases affecting veins, an involvement of the inferior
vena cava (IVC) has been demonstrated. We report a case of IVC leiomyosarcoma operated
by resection and reconstruction with a Dacron bypass and apposition of an Adams-DeWeese
IVC filter. The latter procedure has never been reported before in association with a graft applied
for this disease. Technical and clinical details are described.
Leiomyosarcoma is a rare but lethal disease. Radical
resection, associated with different types of recon-
struction, has been proposed for the treatment of
leiomyosarcoma. Pulmonary embolism is the major
complication of such surgeries, contributing largely
to the mortality rate in these patients.
A 39-year-old woman was hospitalized for edema of the
right leg, that had appeared 3 months earlier. Physical
examination showed an abdominal mass associated with
pain. An echo duplex examination showed inferior vena
cava (IVC) and iliac vein bifurcation thrombosis.
Computed tomography (CT) scan demonstrated the
presence of a retroperitoneal mass measuring about 10 cm
in diameter, involving the vena cava and the iliac bifurca-
tion, with iliac vein thrombosis.
A biopsy was performed and the histology revealed
a G3 (poorly differentiated) leiomyosarcoma.
The patient underwent surgical resection of the mass
and reconstruction of the caval and iliac veins with
a Dacron graft (18 ? 9 mm2) (Fig. 1). Before exclusion
of the venous segment, the patient received intravenous
heparin and because of the presence of an iliac thrombus,
we decided to apply the Adams-DeWeese clip (3 mm
channels, 1 3/8?3.5 cm length) below the renal veins to
protect against intraoperative thromboembolic events.
Histology demonstrated negative resection margins. The
postoperative course was uneventful and the patient
was discharged on the seventh postoperative day. Heparin
was continued postoperatively and then oral anticoagu-
lant therapy was prescribed at discharge.
After 6 months, CT scan showed patency of the graft,
a correct position of the clip, and a local recurrence
involving only the abdominal wall which was resected.
A further course of chemotherapy was administered.
After 36 months, the graft was found to be patent and
the patient is in good clinical conditions. She is still taking
anticoagulant therapy and has not suffered thromboem-
bolic events or leg edema (Figs. 2 and 3).
Leiomyosarcomas are rare tumors affecting veins in
2% of cases. Primary IVC leiomyosarcomas account
for 0.5% of all soft-tissue sarcomas. Mingoli et al.
suggest a subdivision of the IVC into three regions,
each of which has a different grade of involvement
and prognosis. The worst prognosis is associated
with the involvement of the IVC from the right
atrium to the hepatic vein (20% of cases).1
ment with autogenous or prosthetic materials is
Vascular Surgery Unit Policlinico Universitario Bari, Bari, Italy.
Correspondence to: Guido Regina, MD, Vascular Surgery Unit,
Policlinico Universitario Bari, Piazza G. Cesare 11, Bari, Italy, E-mail:
Ann Vasc Surg 2011; 25: 557.e5-557.e9
? Annals of Vascular Surgery Inc.
generally considered to be the best choice of treat-
ment. Because of the rarity of the reported cases
(about 300), several techniques have been used by
different surgeons over the years, but there is no
consolidated evidence about the best surgical
approach. Surgical reconstruction can be accom-
plished by simple repair, patch repair, or segmental
synthetic materials, pericardium or peritoneum
fascial grafts, whereas segmental replacement can
be achieved with a polytetrafluoroethylene (PTFE),
teflon, Dacron, autogenous vein graft or an IVC allo-
graft, and an aortic homograft. These procedures
carry an increased risk of pulmonary embolism.
The mortality rate in patients undergoing this
surgical technique is about 50.6% at 5 years and
70.5 % at 10 years; metastases develop in a maxi-
mum of 57.3% cases.
Several studies havedemonstrated that prognosis
is strictly dependent on the condition of the resec-
are associated to a mortality rate of approximately
IVC ligation has also been advocated, but these
patients may present venous circulation problems
of the legs or a thrombus may form in the blind
portion of the IVC and may extend above or cause
pulmonary infarction. We did not use the saphe-
nous vein or superficial femoral vein because of
their small size, demonstrated at the preoperative
control. A Dacron graft was preferred to a ringed
PTFE graft because of the danger of producing
a caval-enteric fistula caused by the compression
of the rings on the contiguous structure and also
because of the need to administer radiotherapy.
PTFE, however, is associated to a reduced patency
rate in the long term.5,6
Patency rates similar to PTFE grafts were also
reported by Cho et al. and results comparable with
PTFE were obtained by Schwarzbach.5,7-9
The use of arteriovenous fistulas offers another
possibility to optimize venous patency. However,
the effect of this procedure is still controversial
because the potential benefits might not outweigh
the risks. By contrast, cases of persistence of edema
in the lower limbs despite graft patency have been
attributed to the presence of the arteriovenous
of caval grafts after surgery seems to be good. Bower
et al. reported a graft occlusion rate of 10.7%, and
one occlusion was related to recurrent tumor.10
Kuehnl et al. had similar results, describing 15% of
graft occlusion.11Huguet et al. and Kieffer et al.
reported zero graft occlusions in their series.12,13
The Adams-DeWeese clip was applied in our case
to prevent intraoperative and postoperative throm-
boembolic events because the CT scan documented
the presence of a thrombus in both the IVC and the
iliac vein bifurcation.14
Moreover, pulmonary tumor embolism occurs
more frequently than is clinically recognized and is
more common in tumors that invade veins or arise
from the vessel wall, as in thepresentcase. The find-
ings of macroscopic venous invasion at the time of
surgery and histologic confirmation of intravascular
leiomyosarcoma emboli therefore support pulmo-
nary tumor embolism.15
A literature review retrieved few cases of pulmo-
nary embolism. Hollenbeck et al. (25 cases) noted
two cases of perioperative mortality from pulmo-
nary embolism in the setting of preoperative IVC
thrombosis. In a series of nine patients, Cho et al
described one patient with pulmonary embolism at
diagnosis, in whom a temporary IVC filter was
Additionally, in cases of IVC thrombosis, surgery
is not contraindicated, but many authors agree with
the placement of a caval filter before tumor extirpa-
tion, defined as an optimal therapeutic option.16-18
Fig. 1. Surgical resection of the mass and reconstruction
of the caval and iliac veins with a Dacron graft and
557.e6 Case reports
Annals of Vascular Surgery
A review of 161 patients who underwent caval
interruption (92 filters and 69 clips) for both ther-
apeutic and prophylactic reasons showed that the
surgical mortality and morbidity rates were 0%
and 3.3% for filter patients and 8.7% and 2.9%
for clip patients; no procedure-related mortalities
occurred. The late caval patency rate, as docu-
mented by duplex ultrasonography and/or venog-
raphy, was 100% for filter patients and 88% for
clip patients (p ¼ 0.011). Late limb swelling
occurred in 7% of the filter patients and 20% of
the clip patients (p ¼ 0.05). The incidence of
recurrent late pulmonary embolism was 2.5% in
the filter group and 1.9% in the clip group. In
the filter group, 10% of patients experienced
postoperative thrombosis at the femoral vein
insertion site and 0% at the jugular vein insertion
Introduction of the filter through the jugular
vein up to the IVC below the renal veins seemed
hazardous because of the compression of this vas-
cular segment by the tumor, with probable throm-
botic matter coating the vessel walls, whereas
positioning through the iliac vessels was impossible
because of the presence of a thrombus. After tumor
resection and prosthetic graft placement, the clip
was left because of the potential risk of IVC occlu-
sion and of the beneficial effects resulting from
a high blood flow above, thereby reducing the risk
of upstream thrombus formation. Besides, in our
view, additional procedures, such as placement
and subsequent percutaneous removal of a remov-
able filter even if they are considered to be safe and
easy to perform, carry a certain morbidity rate19
which we tried to avoid by leaving the external
filter. Moreover, Blute et al. recommended that
Fig. 2. Pre operative CT scan.
Vol. 25, No. 4, May 2011
preoperative placement of a filter should be avoided
in all instances as the filter can be incorporated into
the thrombus and can complicate surgery.20
Even if there is no evidence in the previously
published data about a thromboembolic risk after
prosthetic replacement, long-term anticoagulation
therapy is still under debate because of the potential
for hemorrhage, presence of foreign material in low
flow venous segments with potential embolization,
or to prevent postoperative graft occlusions. The
risk of thromboembolism is higher than that in
other patient populations. Chemotherapy may also
promote venous thrombosis by causing the release
of procoagulants and cytokines, following toxic
damage to endothelial cells and a decrease inendog-
enous anticoagulants, such as protein C, protein S,
and antithrombin. Moreover, thromboembolism
may be induced by asymptomatic thrombus deposi-
tion or late graft occlusion.
In our experience, resection of the tumor
together with graft replacement and use of a clip
can offer a more favorable outcome to patients in
such severe conditions, in absence of thromboem-
bolic pulmonary complications.
1. Mingoli A, Cavallaro A, Sapienza D, et al. International
registry of inferior vena cava leiomyosarcoma: analysis of
a world series of 218 patients. Anticancer Res 1996;16(15B):
2. Therajima H, Yamaoka Y. Resection and reconstruction of
the inferior vena cava for major hepatic resection. Nippon
Geka Gakkai Zasshi 2001;102:810-814.
3. Mingoli A, Sapienza P, Cavallaro A, et al. The effect of extent
of caval resection in the treatment of inferior vena cava leio-
myosarcoma. Anticancer Res 1997;17(5B):3877-3881.
4. Spinelli F, Stilo F, La Spada M, et al. Surgical treatment of
tumors involving the inferior vena cava. Personal experi-
ence. J Cardiovasc Surg 2008;49:323-328.
Fig. 3. Thirty-six months CT scan showing graft patency in absence of local recurrence.
557.e8 Case reports
Annals of Vascular Surgery
5. Hardwingsen J, Balandraud P, Anania P, et al. Leiomyosar-
coma of the retrohepatic portion of the inferiori vena cava:
clinical presentation and surgical management in five
patients. J Am Coll Surg 2005;200:57-63.
6. Hirohashi K, Shuto T, Kubo S. Asymptomatic thrombosis as
a late complication of the retrohepatic vena cava caval graft
performed for primary leiomyosarcoma of the inferior vena
cava: a case report. Surg Today 2002;32:1012-1015.
8. Sapienza P, Edwards JD, Mcgregor PE, et al. Dacron graft
replacement with bilateral renal vein reimplantation for
inferior vena cava leiomyosarcoma. A case report. Vasc
Endovasc Surg 1996;30:163-168.
9. Cho SW, Marsh JW, Geller DA, et al. Surgical management
of leiomyosarcoma of the inferior vena cava. J Gastrointest
10. Bower TC, Nagirney DM, Cherry KJ Jr, et al. Replacement of
the inferior vena cava for malignancy: an update. J Vasc
11. Kuehnl A, Schmidt M, Horning HM, et al. Resection of
malignant tumors invading the vena cava: perioperative
complications and long-term follow-up. J Vasc Surg
12. Huguet C, Ferry M, Gavelli A. Resection of the suprarenal
inferior vena cava: the role of prosthetic replacement.
Arch Surg 1995;130:793-797.
13. Kieffer E, Bahnini A, Koskas F. Non-thrombotic disease of
the inferior vena cava: surgical management of 24 patients.
In: Bergan JJ, Yao JST eds. Venous Disorders. Philadelphia,
PA: WB Saunders, 1991. pp 501-516.
14. Bower TC, Stanson AW. Tumors of the inferior vena cava;
diagnosis and management. In: Rutherford RB ed. Vascular
Surgery. 5th ed. Philadelphia, PA: WB Saunders, 2000.
15. Gentle S, Fisher C, Soni N, et al. Pulmonary tumour embo-
lism complicating a case of leiomyosarcoma. Sarcoma
16. Hollenbeck ST, Grobmyer JR, Kent KC, et al. Surgical treat-
ment of patients with primary vena cava leiomyosarcoma.
J Am Coll Surg 2003;197:575-579.
17. Illuminati G, Calio FG, D’Urso A, et al. Prosthetic replace-
ment of the infrahepatic inferior vena cava for leiomyosar-
coma. Arch Surg 2006;141:919-924.
18. Sartori A, Vigna S, Dal Pozzo A, et al. Leiomyosarcoma of
the inferior vena cava. A case report and review of the liter-
ature. Chir Ital 2009;61:503-505.
19. AbuRahma AF, Robinson PA, Boland JP, et al. Therapeutic
and prophylactic vena caval interruption for pulmonary
embolism: caval and venous insertion site patency. Ann
Vasc Surg 1993;7:561-568.
20. Blute ML, Boorjian SA, Leibovich BC, et al. Results of infe-
rior vena cava interruption by greenfield filter, ligation or
resection during radical nefrectomy and tumor thrombec-
tomy. J Urol 2007;178:440-445.
Vol. 25, No. 4, May 2011