Case Study

Asian Cardiovascular & Thoracic Annals

20(6) 715–717
ß The Author(s) 2012
Primary inferior vena cava Reprints and permissions:
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leiomyosarcoma: reconstruction DOI: 10.1177/0218492312440266
aan.sagepub.com
is not mandatory

Emiliano A Rodrı́guez-Caulo, Carlos Velázquez, Bella Ramı́rez

and José M Barquero

Abstract
Computed tomography in an 81-year-old woman revealed obstruction of the inferior vena cava by a large primary
vascular leiomyosarcoma, and involvement of the right renal vein. She underwent successful en-bloc resection of
the tumor, right kidney, hepatic segments IV and VI, and inferior vena cava, without caval reconstruction. A renal
vein-to-remaining infrahepatic inferior vena cava saphenous vein graft bypass was performed for left renal venous
drainage. The need for vascular reconstruction is not always mandatory.

Keywords
Leiomyosarcoma, vascular neoplasms, vascular surgical procedures, vena cava, inferior

Introduction Case report

Although rare, primary vascular leiomyosarcoma
(PVL) is the most common primary malignancy of
the inferior vena cava (IVC).1 Anatomically, the IVC
is divided into 3 levels: level I extends from the
hepatic veins up to the right atrium, level II com-
prises the area between the renal and hepatic veins,
and level III includes the area below the renal veins.
Level II is most frequently affected by PVL.2–5 This
tumor has an extremely poor prognosis. According
to the international register established by Mingoli
and colleagues,1 the 5- and 10-year actuarial malig-
nancy-free survival rates after wide surgical resection
are 31.4% and 7.4%, respectively. PVL arising in
the IVC are most frequently seen in the 6th
decade, with a female predominance.2 Clinical symp-
toms are nonspecific and may precede the diagnosis
by several years.1 Diagnosis is often challenging
because patients may present with nonspecific com-
plaints such as dyspnea, weight loss, abdominal
pain, back pain, and lower extremity edema.
Computed tomography, magnetic resonance imaging,
and ultrasound allow early and accurate preoperative
diagnosis.
An 81-year-old woman presented with a history of
abdominal pain and weight loss. Past medical history
included obesity, hypertension, and varicose veins.
Physical examination was normal except for abdominal
tenderness and a palpable mass in the right flank.
Computed tomography revealed a dense heterogeneous
retroperitoneal mass at IVC level II, without evidence
of pulmonary emboli or metastases (Figure 1). A per-
cutaneous biopsy showed fascicular fibers positive
for desmin and smooth muscle actin. A diagnosis of
pleomorphic PVL was established. Through a median
laparotomy and Kocher maneuver of the colon and
duodenum, radical en-bloc surgical resection was per-
formed, with partial IVC ligation and resection, and a
right nephrectomy. The resected tumor weighed 1320 g
Cardiovascular Surgery Department, Virgen Macarena University
Hospital, UGC Heart Area, Seville, Spain
Corresponding author:
Emiliano A Rodrı́guez-Caulo, Cardiovascular Surgery Department, Virgen
Macarena University Hospital, UGC Heart Area, Av Dr Fedriani S/N
41009, 3aD, Seville, Spain.
Email: erodriguezcaulo@hotmail.com

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716 Asian Cardiovascular & Thoracic Annals 20(6)

Figure 1. Preoperative images showing the leiomyosarcoma (*)

of the inferior vena cava (IVC). (A) Axial contrast-enhanced
computed tomography scan. (B) Coronal magnetic resonance
imaging.

and measured 16  13  10 cm (Figure 2A) with a

thickened IVC wall (Figure 2B). A left renal vein-
to-remaining infrahepatic IVC saphenous vein bypass
graft was constructed (Figure 2C) to achieve adequate
left renal venous drainage. Intraoperative pathological
findings confirmed the preoperative diagnosis of PVL
that had infiltrated the right kidney and renal vein.
Surgical margins of the resected specimen were
tumor-free. The patient was discharged on 17th post-
operative day with preserved renal function, and has
remained asymptomatic after 12 months.
Discussion
Due to the absence of early symptoms, retroperitoneal
tumors are often not diagnosed until the disease is at an
advanced stage and involves the surrounding
Figure 2. Intraoperative findings. (A) The giant leiomyosarcoma
(*) emerging from the inferior vena cava. (B) Operative
photograph showing the tumor (*) and a thickened inferior vena
caval wall with the appearance of fish meat (#). (C) The left renal
vein-to-inferior vena caval saphenous vein bypass graft.
structures. Curative radical en-bloc resection of the
affected IVC segment remains the current treatment
of choice for PVL, and is associated with prolonged
survival of 50%–62% at 5 years and 22% at 10
years.3,5,6 In PVL, the major surgical issue is the need
for venous reconstruction, basically ligation and resec-
tion of the affected IVC and cavoplasty, or graft
replacement. At IVC level III, simple ligation and

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Rodrı́guez-Caulo et al.
resection achieves good functional results due to previ-
ous development of collateral veins. Tumors that
involve level I or II raise special challenges for operative
treatment with respect to vascular reconstruction. The
topographic relationship to the renal or hepatic veins is
critical to the surgical strategy, for maintaining venous
outflow, complete tumor control, and prevention of
recurrence.
Currently, there is considerable controversy about
the need for reconstruction of the IVC level II.
Several reports recommended prosthetic replacement,
but others have described cavoplasty or ligation and
resection without complications.2 Sometimes, ligation
and resection may be precarious because of impairment
of renal venous outflow, resulting in renal dysfunction,
particularly in the right kidney. In cases where the right
renal vein needs to be sacrificed because of tumor inva-
sion, the kidney has to be resected, as occurred in this
case. In contrast, the left renal vein provides more col-
laterals. However, even on the left side, renal functional
impairment was described after sacrifice of the left
vein;7 thus we reconstructed it with an inverted autol-
ogous saphenous vein graft, which should be tried
whenever possible, although we emphasize that it is
not essential to reconstruct IVC continuity in all
cases. To improve the outcome of patients with PVL
of the IVC, recent adjuvant and neoadjuvant treatment
protocols have been applied, with promising results
including 100% local control without recurrence at 3
years.8
Surgery, performed alone or in combination with
adjuvant therapy constitutes the only chance of pro-
longed survival. Aggressive management using
modern vascular surgical techniques is currently recom-
mended. The type of IVC resection or reconstruction
should be individualized depending on tumor expan-
sion. Therefore, IVC ligation and resection requiring
graft replacement is not always mandatory. This con-
cept was reflected in the treatment of our patient.
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717
Funding
This research received no specific grant from any funding
agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement
None declared.
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