Cas clinique

DOI of or

Vascular S

CorrespondPoliclinico Unig.regina@chirv

Ann Vasc SurgDOI: 10.1016/� Annals of V�Edit�e par ELS

L�eiomyosarcome de la veine cave inf�erieure :R�esection et reconstruction vasculaireutilisant une proth�ese en Dacron et un clipd’Adams De Weese : suivi �a trois ans

Domenico Angiletta, Martinella Fullone, Luigi Greco, Davide Marinazzo, Piero Frontino,

Guido Regina, Bari, Italie

Les l�eiomyosarcomes sont des tumeurs malignes rares qui affectent en particulier les femmes.Dans 2% des cas, elles impliquent les veines, et dans 60% des cas affectant les veines, uneparticipation de la veine cave inf�erieure (IVC) a �et�e d�emontr�ee. Nous rapportons un cas del�eiomyosarcome de la VCI trait�e par r�esection et reconstruction avec une pontage en Dacronavec mise en place d’un filtre d’Adams-DeWeese. La derni�ere proc�edure n’a jamais n’a �et�erapport�ee avant en association avec une proth�ese pour cette maladie. Des d�etails techniqueset cliniques sont d�ecrits.

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.

CASE REPORT

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

bifurcation, with iliac vein thrombosis.

iginal article: 10.1016/j.avsg.2010.12.015.

urgery Unit Policlinico Universitario Bari, Bari, Italie.

ence : Guido Regina, MD, Vascular Surgery Unit,versitario Bari, Piazza G. Cesare 11, Bari, Italie, E-mail:asc.uniba.it

2011; 25: 557.e5-557.e9j.acvfr.2012.04.006ascular Surgery Inc.EVIER MASSON SAS

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 anticoagulant

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 involv-

ing only the abdominal wall whichwas 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).

DISCUSSION

Leiomyosarcomas are rare tumors affecting veins in

2% of cases. Primary IVC leiomyosarcomas account

599.e5

Fig. 1. Surgical resection of the mass and reconstruction

of the caval and iliac veins with a Dacron graft and

Adams-DeWeese clip.

599.e6 Cas cliniques Annales de chirurgie vasculaire

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

Resection of the neoplastic mass and vein repla-

cement with autogenous or prosthetic materials is

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 accomplis-

hed by simple repair, patch repair, or segmental

replacement.2 Patch grafting may be performed

using synthetic materials, pericardium or

peritoneum fascial grafts, whereas segmental

replacement can be achieved with a polytetra-

fluoroethylene (PTFE), teflon, Dacron, autogenous

vein graft or an IVC allograft, and an aortic homo-

graft. 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 have demonstrated that prognosis

is strictly dependent on the condition of the resec-

tionmargins. Positivemicroscopic resectionmargins

are associated to a mortality rate of approximately

100%.3,4

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 por-

tion of the IVC and may extend above or cause pul-

monary infarction. We did not use the saphenous

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 fis-

tula itself.

In the absence of tumor recurrence, the durabi-

lity 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, des-

cribing 15% of graft occlusion.11 Huguet 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

thromboembolic events because the CT scan docu-

mented 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 the present case. The fin-

dings 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

Fig. 2. Pre operative CT scan.

Vol. 25, No. 4, 2011 Cas cliniques 599.e7

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 pla-

ced preoperatively.9

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

A review of 161 patients who underwent caval

interruption (92 filters and 69 clips) for both thera-

peutic 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

venography, 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 throm-

bosis at the femoral vein insertion site and 0% at the

jugular vein insertion site.19

Introduction of the filter through the jugular vein

up to the IVC below the renal veins seemed hazar-

dous because of the compression of this vascular

segment by the tumor, with probable thrombotic

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 occlusion and

Fig. 3. Thirty-six months CT scan showing graft patency in absence of local recurrence.

599.e8 Cas cliniques Annales de chirurgie vasculaire

of the beneficial effects resulting from a high blood

flow above, thereby reducing the risk of upstream

thrombus formation. Besides, in our view, additio-

nal procedures, such as placement and subsequent

percutaneous removal of a removable 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 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 pro-

mote venous thrombosis by causing the release of

procoagulants and cytokines, following toxic

damage to endothelial cells and a decrease in

endogenous anticoagulants, such as protein C,

protein S, and antithrombin. Moreover, throm-

boembolism may be induced by asymptomatic

thrombus deposition or late graft occlusion.

In our experience, resection of the tumor toge-

ther with graft replacement and use of a clip can

offer a more favorable outcome to patients in such

severe conditions, in absence of thromboembolic

pulmonary complications.

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