Prepared by Tung V. Huynh, MD and Thomas B. Kinney, MD of
the Department of Radiology, and John B. Welsh, MD of the
Department of Pathology, at the University of California San
Diego Medical Center, San Diego, CA.
A 52-year-old man presented with worsening left lower extremity
swelling despite adequate anticoagulation for a deep venous
thrombosis diagnosed 2 months earlier. Physical examination
revealed bilateral lower extremity edema, worse on the left. Venous
duplex exam (not shown) revealed bilateral thrombi within the
superficial femoral and common femoral veins.
Work-up included laboratory evaluation for hypercoagulable state
along with imaging workup for occult malignancy as a possible cause
of Trousseau syndrome. Initial ultrasonographic abdominal survey
reveals right hydronephrosis (not shown) and CT was recommended
(figure 1). Figure 2 is a vena cavagram at the time of IVC filter
placement and figure 3 is microscopic section of an aspiration
Leiomyosarcoma of the inferior vena cava (IVC) (IVC sarcoma)
The IVC is patent at the level of the renal vein. Right
hydronephrosis is demonstrated (figure 1A). More inferiorly, a
large 5 cm heterogeneously enhancing retroperitoneal mass extends
from the renal hilar level to the iliac bifurcation (figure 1B),
centered within and obliterating the IVC. The intraluminal filling
defect surrounded by contrast above the iliac bifurcation (figure
1C) could represent an intraluminal component of the tumor mass or
The patient underwent successful biopsy of the mass
percutaneously with CT guidance. The aspirates and cell block
(figure 3) demonstrate pleomorphic and cohesive groups of spindle
cells, which are positively stained with vimentin and muscle
specific actin (special stains not shown) indicating spindle cell
neoplasm of smooth muscle differentiation, highly suspicious for
Laboratory evaluation confirmed antiphospholipid antibody
syndrome, which, in combination with the IVC sarcoma, contributed
to his recurrent and progressive DVT despite appropriate coumadin
therapy. Because of retroperitoneal bleeding related to the biopsy
procedure and the concern of possible pulmonary embolism, a
suprarenal Greenfield stainless steel IVC filter was inserted. The
vena cavogram. at the time of filter placement (figure 2) shows
complete occlusion of the IVC just below the renal vein
Leiomyosarcoma of the IVC is an uncommon tumor first reported by
Perl in 1871.
Data as of December 1993 from the International Registry of IVC
reported a total of 218 cases, inclusive of comprehensive
literature review and authors' personal communication. Since then,
35 additional cases have been described, bringing the total number
of reported cases to 254, including the current case.
Histologically, leiomyosarcoma show a typical pattern of
interlacing bundles of spindle-shaped cells with varying degrees of
mitotic activity, and cellular and nuclear polymorphism.
Differentiation from other types vascular tumors
(hemangioendothelioma, angiosarcoma, and fibrosarcoma) is possible
by different histologic features and determination of smooth muscle
origin by immunohistochemical methods or electron microscopy.
Generally, there is a poor correlation between the histologic
appearance of leiomyosarcoma and clinical outcome; the size and
location of the tumor are the most important prognostic factors.
IVC sarcoma is predominantly a female affliction, with a female
to male ratio of approximately 4:1.
The average age of the patients in the International Registry of
IVC Leiomyosarcoma is 54 years (range 15 to 85 years). The
presenting symptoms depend somewhat on both the size and location
of the tumor and include abdominal pain (67%), palpable mass (43%),
leg edema (35%), weight loss (22%), and Budd-Chiari's syndrome
(17%). Other less common symptoms (<12%) reported include:
weakness, nausea, dyspnea, fever, anorexia, vomiting, jaundice, and
night sweat. This tumor has a very poor prognosis and the optimal
treatment regimen has not been established. The role of radiation
and chemotherapy is not well defined; while surgical resection,
when possible, seems to offer the best chance for survival.
Comprehensive analysis of the clinical outcome of the 218 patients
in the International Registry of IVC leiomyosarcoma (180 with
follow-up data) showed a mean survival of 4.7 ± 1.1 months (median
2 months) in patients who were not operated on for curative
resection (38.5%). The 5- and 10-year survival rates for patients
undergoing radical resection (61.5%) were 49.4% and 29.5%
respectively. More than half of these patients had disease
recurrence (13.4% with local recurrence, 31.3% with metastasis, and
9% with both) with overall 5- and 10-year disease-free rates of
31.4% and 7.4% respectively.
A statistically significant increased risk of death occurred in
patients with tumor involving the upper IVC segment (hepatic vein
origin to right atrium) with or without Budd-Chiari's syndrome, IVC
occlusion, intraluminal growth, and limb edema. These poor
prognostic indicators reflect the critical location of the tumor
behind the liver, obligating intraluminal growth and making radical
resection of the tumor technically difficult or impossible. In
fact, only 14.6% of patients with upper IVC involvement underwent
radical resection of the tumor as compared with 70% to 75% of
patients with mid IVC (involving renal vein but below hepatic vein)
or lower IVC (infrarenal) involvement.
In patients with IVC sarcomas involving the mid and lower
segments, good prognostic indicators include presence of abdominal
pain and absence of palpable mass at the time of presentation.
These parameters likely correlate with earlier detection of the
tumor as well as surgical resectability. Tumors in the middle
segment of the cava generally cause symptoms earlier due to the
rich sensory innervation of the surrounding organs, allowing
earlier diagnosis and better 5- and 10-year survival rates (56.7%
and 47.3% respectively) compared to tumors in lower IVC segment
(37.8% and 14.2%).
Cross-sectional imaging plays a significant role in the early
detection and in defining resectability of this tumor. As in the
case presented here, heterogeneous contrast uptake on CT is
characteristic and allows differentiating tumor mass from bland
thrombus. Similar tumor vascularity could be seen with Doppler
ultrasound and gadolinium-enhanced MR imaging. These features,
along with the pattern of growth (i.e., intraluminal versus
extravascular extension), are non-specific and accurate
preoperative diagnosis still relies on pathologic examination of
the biopsy specimen. Transvenous biopsy, an alternative biopsy
technique, may be useful in such cases as this, it might have
avoided the bleeding complication this patient developed.
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3. Detailed list of published case reports available from
authors upon requests.
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superior vena cava syndrome: Diagnosis with percutaneous
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