Medical and Pediatric Oncology 32:313–314 (1999)
BRIEF REPORT
Successful Treatment of Intracaval and Atrial Extension of Rhabdomyosarcoma
Without Surgical Excision: An Unusual Presentation
Canan Akyüz, MD,1 Volkan Hazar, MD,2* Betül Sevinir, MD,3 Ayşenur Cila, MD,4
and Münevver Büyükpamukçu, MD1
Key words: tumor thrombus; childhood cancer; chemotherapy; radiotherapy
Tumor extension to the inferior vena cava (IVC) by
nonrenal tumors in childhood is rare. Our experience
with such a case may therefore be of value to the others.
Our patient is a 7-year-old girl who initially presented
with a 1-month history of lower abdominal pain, weight
loss, and fatigue. On physical evaluation she was found
to have an abdominal mass (approximately 10 × 5 cm in
size) extending from the mons pubis to the umbilicus.
Other organ systems were normal on examination. Ab-
dominopelvic ultrasonography (US) showed a large, hy-
poechoic, solid tumor filling the whole pelvis causing
total vena caval occlusion by an echogenic tumor throm-
bus starting from the common iliac veins. Echocardiog-
raphy and computed tomography (CT) scans were per-
formed, but the tumor thrombus within the IVC extend-
ing to the right atrium was best outlined by magnetic
resonance (MR) imaging (Fig. 1). Total occlusion of the
right external iliac vein and the IVC was confirmed by
venography. While her biochemical laboratory values
were within normal ranges, thrombosis-associated fac-
tors were as follows: Protein C (PC) 34% (N:60–175%),
protein S (PS) 34% (N:73–210%), antithrombin III (AT
III) 16% (N:18–28%). Excisional biopsy of the pelvic
mass showed an embryonal rhabdomyosarcoma. Six
cycles of chemotherapy consisting of vincristine, ifos-
famide, cisplatin, and doxorubicin were given after
which radiologic evaluation with US and CT showed
shrinking of the thrombus in the IVC and disappearance
of the atrial component. PC, PS, and AT III levels were
within normal ranges. Following 50 Gy radiotherapy to
the pelvic mass and 45 Gy to the IVC, three more cycles
of chemotherapy were given. Subsequent radiologic
evaluation revealed no residual tumor in the pelvis and
punctuate calcification within the thrombosed IVC (Fig.
2). There were no tumor cells in a fine-needle aspiration
from the thrombus. The patient has been relapse-free for
4 years.
Identification of tumor thrombosis is important be-
cause it greatly complicates treatment and alters the
prognosis. It is rare for intravascular tumor thrombi in the
© 1999 Wiley-Liss, Inc.
IVC to break loose and become emboli. In Wilms tumor
patients, at least, either initial surgery or preliminary
chemo-radiation therapy can be used successfully. The
complications are fewer if surgery is delayed [1].
Complications of cancer that occur at a distance from
the primary tumor or from metastases have been studied
for decades. According to current concepts, tumor cells
are involved not only in the activation of blood coagu-
lation and depression of anticoagulant functions, but also
in causing injury to the endothelial lining of blood ves-
sels and activation of platelets [2,3]. Solid tumors grow-
ing outside the blood vasculature regularly deposit fibrin
locally in the tissues. This is thought to lead to micro-
vasculature hyperpermeability and to allow leakage of
fibrinogen and other plasma-clotting factors which initi-
ate clotting [4].
Hemostatic alterations in patients with cancer are
complex. Tumor thrombi usually contain fibrin as well as
tumor cells. Although none are specific for cancer, clot-
ting factors, such as PS, PC, and AT III, may decrease
due to their consumption. Following several of these fac-
tors, rather than one, can be useful in evaluating tumor
remission or progression. Biopsy from the thrombus be-
fore the decision to end therapy may be required in case
the thrombus has not completely resolved. The interest-
1
Department of Pediatrics, Hacettepe University Medical Faculty, An-
kara, Turkey
2
Department of Pediatrics, Akdeniz University Medical Faculty,
Antalya, Turkey
3
Department of Pediatrics, Uludağ University Medical Faculty, Bursa,
Turkey
4
Department of Radiology, Hacettepe University Medical Faculty, An-
kara, Turkey
*Correspondence to: Volkan Hazar, MD, Assistant Professor of Pedi-
atrics, Akdeniz University Medical Faculty, 07070 Antalya, Turkey.
E-mail: neslivolkan@superonline.com
Received 19 May 1998; Accepted 12 August 1998
314 Akyüz et al.

Fig. 2. Follow-up contrast-enhanced abdominal CT after chemo-

radiation therapy. Mid-abdominal image shows a small and throm-
bosed IVC with a centrally located calcification (small arrow). Note
dilated paravertebral veins (arrowheads).

ample [8]. Chemotherapy and radiotherapy alone without

surgical excision of the thrombus may be effective in
such patients.

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Fig. 1. Sagittal T1W image shows thrombus-filled and expanded
IVC (horizontal arrows). Thrombus protrudes into the right atrium
(upper arrow).
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type. IVC tumor thrombosis has been described in chil-
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