Case Study

Asian Cardiovascular & Thoracic Annals

2014, Vol. 22(7) 858–861
ß The Author(s) 2013
Giant leiomyosarcoma of inferior vena Reprints and permissions:
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cava. A surgical challenge DOI: 10.1177/0218492313487356
aan.sagepub.com

Santosh Kumar, Sudheer Kumar Devana, Sachin Kuthe,

Thakur Deen Yadav and Seema Prasad

Abstract
We describe the case of a 37-year-old woman who presented with an unusually large (26  20  16 cm) extraluminal
leiomyosarcoma arising from segment I (below the renal vessels) of the inferior vena cava. She was successfully managed
with radical surgical excision and reconstruction of the inferior vena cava with synthetic graft.

Keywords
Blood vessel prosthesis implantation, leiomyosarcoma, magnetic resonance imaging, tomography, X-ray computed,
vascular neoplasms, vena cava, inferior

Introduction of appetite. There was no history of bowel or bladder

Primary leiomyosarcoma of vascular origin is a rare
tumor that arises most commonly from the inferior
vena cava (IVC). More than 200 cases of IVC leiomyo-
sarcoma have been reported since 1871.1
Leiomyosarcomas arising from IVC are frequently
seen in the 6th decade of life, and show a female pre-
dominance.2 Presenting symptoms are nonspecific: dull
aching abdominal pain, back ache, vague fullness in the
abdomen, malaise, weight loss, pedal edema, and dys-
pnea. Computed tomography, magnetic resonance ima-
ging, cavography, and echocardiography aid in the
diagnosis and planning of treatment of these retroperi-
toneal tumors.3 Radical surgical excision with negative
margins is the treatment of choice. The excised IVC wall
can be reconstructed by primary repair, cavoplasty with
a patch, or polytetrafluoroethylene (PTFE) graft inter-
position. Adjuvant chemoradiotherapy can be given to
prevent recurrence. In spite of radical surgery and adju-
vant therapy, the 5-year survival is still only in the order
of 50%.4 We describe a case of huge IVC leiomyosar-
coma, in which we performed radical surgical excision
with IVC reconstruction using a PTFE graft.
disturbances. On physical examination, she was frail
and malnourished. A 28  24-cm hard lobulated immo-
bile mass was palpable in the epigastrium, umbilical,
right lumbar, and iliac fossa regions. Contrast-
enhanced computed tomography and magnetic reson-
ance imaging showed a giant 26  20  16-cm lobulated
mass with areas of necrosis, occupying the whole of
right side of the abdomen, encasing the aorta and the
right renal artery. The right kidney was small, none-
nhancing, and compressed by the mass. The IVC was
not seen separately from the level of the confluence
of common iliac veins to the level of left renal vein
(Figure 1). Based on these findings, the infrarenal
IVC was thought to be the probable site of origin of
the tumor. A preoperative Tru-Cut biopsy was suggest-
ive of leiomyosarcoma. In view of the huge size of the
tumor and nonenhancing right kidney, preoperative
embolization of the lumbar vessels feeding the tumor
and the right renal artery was performed to decrease the
vascularity of the tumor and allow peritumoral edema
Department of Urology, Postgraduate Institute of Medical Education and
Research, India

Case report Corresponding author:

Santosh Kumar, MS, FRCS, MCh, Department of Urology, Postgraduate
A 37-year-old woman presented with a 6-month history Institute of Medical Education and Research, Sector 12, Chandigarh
of increasing fullness in the right side of the abdomen, 160012, India.
associated with a dull aching pain, weight loss, and loss Email: santoshsp1967jaimatadi@yahoo.co.in

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Kumar et al. 859

Figure 1. (a) Contrast-enhanced computed tomography showing a giant inferior vena caval mass compressing the right kidney and
displacing the aorta to left side. (b) Contrast-enhanced magnetic resonance image showing a huge vascular mass replacing the
infrarenal inferior vena cava, extending almost to the confluence of the common iliac veins.

Figure 2. (a, b) Pre-embolization angiograms of the lumbar arteries. (c) Post-embolization angiogram of the lumbar arteries.

to develop, to aid surgical resection and decrease
intraoperative blood loss (Figure 2). Intraoperatively,
the mass was found arising from the infrarenal IVC,
encasing and invading the right renal artery
(Figures 3a, 3b). Excision of the mass along with a
segment of the IVC (extending from the infrarenal
IVC to the confluence of common iliac veins) and a
right nephrectomy were carried out (Figure 3c).
The resected IVC segment was reconstructed with a
PTFE ring graft (Figures 3d, 3e). Histopathology
revealed a high-grade spindle-cell tumor suggestive of
leiomyosarcoma, arising from the IVC. The patient was

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860 Asian Cardiovascular & Thoracic Annals 22(7)

Figure 3. Intraoperative pictures showing (a) the giant inferior vena caval mass with the left renal vein and infrahepatic inferior vena
cava looped in vascular slings, (b) the lower limit of the tumor with both the common iliac veins looped in vascular slings, (c) the
resected inferior vena cava with the ring graft, (d) the completed inferior vena caval reconstruction, and (e) the resected specimen of
the giant leiomyosarcoma.

extubated on postoperative day 1. She was allowed oral
feeding after 3 days, due to postoperative paralytic
ileus. She had persistent right subhepatic drain output
for 10 days. She was discharged from the hospital on
postoperative day 12, and kept on oral anticoagulants
at the time of discharge. She was advised adjuvant
radiotherapy following surgery.
Discussion
Approximately 2% of leiomyosarcomas are vascular in
origin, half of which are of IVC origin, and 75% to 90%
occur in women. IVC leiomyosarcomas arise from the
tunica media of caval wall. They demonstrate 3 growth
patterns: extraluminal, intraluminal, or both.
Depending on the site of origin from the IVC, they are
anatomically divided into 3 types: lower or segment I
(below the renal vessels), middle or segment II (renal
vessels to retrohepatic IVC), and upper or segment III
(suprahepatic IVC).2 The International Registry of
Inferior Vena Cava Leiomyosarcoma collected 218
cases in 1992; of these, 80 tumors arose from segment
I, 94 from segment II, and 41 from segment III.1 Surgical
management depends on the site of origin of the tumor.

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Kumar et al.
Clinical features are nonspecific and may precede the
diagnosis by several years. The 3 most common symp-
toms are abdominal pain (52%), distension (20%), and
deep vein thrombosis (12%).5 Other features such as
Budd-Chiari syndrome, cerebral embolism, and new
heart murmurs may be found.6 Modern imaging mod-
alities using Doppler ultrasound, computed tomog-
raphy, magnetic resonance imaging, cavography,
echocardiography, and positron-emission tomography
can used for accurate preoperative diagnosis. In some
cases, despite these imaging modalities, the actual site
of origin of the tumor cannot be confirmed. These ima-
ging modalities also help in planning the surgical strat-
egy. Biopsy is indicated for a formal diagnosis.
Histopathology of leiomyosarcoma reveals spindle
cells that are positive for vimentin, smooth muscle
actin, and desmin.7
The optimal management of IVC leiomyosarcoma is
still unknown due to limited experience of the disease.
Aggressive surgical excision with negative margins
(1 cm) is the treatment of choice because the tumor
has a slow growth pattern and relatively low malignant
potential. After tumor excision, the IVC can be mana-
ged by ligation in the case extensive collaterals, primary
repair, patching, or interposition grafting with a syn-
thetic conduit.8 Primary repair is the method of choice
where applicable, as it is associated with the lowest
complication rate. Patients who undergo IVC grafting
should be kept on oral anticoagulation. Tumors invol-
ving the lower segment of the IVC are amenable to
complete resection, in contrast to tumors involving seg-
ments II and III. Neoadjuvant therapy can be given to
downstage the tumor and increase resectability.
Complete surgical resection or palliative debulking of
the tumor with adjuvant chemoradiotherapy can be
applied to reduce local and systemic recurrence and
increase survival.9 The 5-year cumulative survival rate
was reported to be 53% for patients with IVC leiomyo-
sarcoma, suggesting that radical surgical excision and
adjuvant therapy offer the best outcome.
Funding
This research received no specific grant from any funding
agency in the public, commercial, or not-for-profit sectors.
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861
Conflicts of interest statement
None declared.
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