Download Leiomyosarcoma of the Inferior Vena Cava

Hayley Walker, MSIII
Gillian Lieberman, MD
Leiomyosarcoma of the Inferior Vena Cava
Hayley Walker, Harvard Medical School, MSIII
Gillian Lieberman, MD
Hayley Walker, MSIII
Gillian Lieberman, MD
Overview
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Patient presentation
Pulmonary embolism on CTA
Retroperitoneal mass on CT
Differential diagnosis for retroperitoneal mass
Menu of tests
Approach to IVC masses
Narrowing the differential with imaging
Leiomyosarcoma
Impact of imaging on surgical planning
IVC anatomy review
Intraoperative ultrasound
History continued
Post-surgical follow-up
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Hayley Walker, MSIII
Gillian Lieberman, MD
Our Patient: History of Present Illness
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The patient is a 26-year-old male who developed left leg
swelling and pain in August 2011. Ultrasound revealed a left
occlusive deep vein thrombosis (DVT) from the left common
femoral vein through the posterior tibial vein.
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He was started on warfarin. He had no known DVT risk
factors. Hematology work-up found no evidence of an
acquired hypercoagulable state or inherited thrombophilia.
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In February 2012, he was admitted to an outside hospital
with chest pain, and was found to have a pulmonary
embolism (PE).
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His INR at the time was therapeutic, between 2 and 2.5. He
was therefore begun on enoxaparin for anti-coagulation.
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Hayley Walker, MSIII
Gillian Lieberman, MD
Pulmonary Embolism on CTA
This is the patient’s CTA
image showing a
pulmonary embolism.
-Findings: Filling defect
in right pulmonary
artery
Other structures:
-Aortic outflow tract
-Right ventricle
-Descending aorta
Axial CTA
Outside hospital records
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Hayley Walker, MSIII
Gillian Lieberman, MD
Abnormal mass on CTA
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This is the most caudal
image from the CTA
performed at the outside
hospital when the patient
presented with a PE.
This abnormal
structure was not
mentioned on the
radiologic report.
This reminds us to
always remember to
inspect all images!
Axial CTA
Outside hospital records
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Hayley Walker, MSIII
Gillian Lieberman, MD
Our patient: History continued
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The patient had an outpatient hematology appointment in
February 2012
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Because the patient suffered a pulmonary embolism despite
being on warfarin with a therapeutic INR, a CT abdomen and
pelvis was performed to rule out occult malignancy.
C+ axial CT abdomen & pelvis
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Hayley Walker, MSIII
Gillian Lieberman, MD
Mass on CT abdomen and pelvis
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A large mass with
areas of increased
density can be seen in
the retroperitoneum
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Measured 7.4 x 7.4 x
9.7 cm
The inferior vena cava
(IVC) is not visualized
Multiple abnormal
subcutaneous
collateral vessels have
formed to bypass the
obstructed IVC
C+ axial CT abdomen & pelvis
PACS, BIDMC
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Hayley Walker, MSIII
Gillian Lieberman, MD
Differential diagnosis for
retroperitoneal mass
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Soft tissue sarcoma
Lymphoma
Primary germ cell tumor
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Metastatic testicular cancer
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Check AFP and HCG levels to assess likelihood (both were normal in
this patient)
Perform scrotal ultrasound to assess likelihood (was normal in this
patient)
Neoplasms from duodenum, pancreas, adrenal glands,
or kidneys
Schwannomas, paragangliomas
Benign processes: Castleman’s disease (angiofollicular
lymph node hyperplasia), retroperitoneal fibrosis
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Hayley Walker, MSIII
Gillian Lieberman, MD
IVC mass on CT abdomen & pelvis
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C+ coronal reconstruction
CT abdomen and pelvis
Imaging suggests the
mass is within the IVC
itself (and not simply
retroperitoneal and
externally compressing
the IVC).
Continue to view the
menu of tests for
imaging the IVC.
PACS, BIDMC
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Hayley Walker, MSIII
Gillian Lieberman, MD
Imaging the IVC: Menu of Tests
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Conventional venography
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Ultrasonography
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Historical gold standard; now rarely used
Color Doppler flow imaging is used to assess for blood flow
MDCT
Different phases of IV contrast administration are used
Arterial phase: Identify hypervascular tumors. Assess for pulmonary
emboli or lung metastases.
Portal venous phase: 60-70 seconds after contrast injection. Typically
used to evaluate IVC. Can also evaluate liver parenchyma.
 IVC heterogeneously enhances in this phase. Non-opacified blood
from lower extremities mixes with opacified blood from renal veins.
Can make IVC thrombus assessment difficult.
Three-minute delay phase: Provides more homogenous enhancement
of IVC lumen; allows better assessment of superior and inferior
extension of tumor.
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MR
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T2-weighted images as well as pre- and post-IV contrast injection T1weighted images
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Hayley Walker, MSIII
Gillian Lieberman, MD
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The menu of tests for imaging the IVC has been
presented.
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Please proceed to view a general approach to IVC
masses.
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Hayley Walker, MSIII
Gillian Lieberman, MD
Approach to IVC masses: Step 1
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The first step is to differentiate between bland
thrombus (clot) and tumor thrombus
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Tumor thrombus suggested by:
 Expansion of lumen by the thrombus
 Enhancement of filling defect
 Direct continuity between tumor in another organ and
thrombus
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Please proceed to the next slide to view an
image of a companion patient which illustrates
the characteristic features of tumor thrombus
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Hayley Walker, MSIII
Gillian Lieberman, MD
Companion patient: tumor thrombus
on CT
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This companion patient was found to
have tumor thrombus in the IVC
secondary to renal cell carcinoma
The thrombus obstructs and expands
the IVC
There is direct continuity between the
renal mass and the IVC thrombus
Neovascularity in the main thrombus
confirms the finding is tumor thrombus
and not bland thrombus
 This image was obtained in the
arterial phase.
C+ coronal reconstruction
CT abdomen & pelvis
From Sheth Sheila, Fishman EK. Imaging of the Inferior Vena Cava
with MDCT. AJR 2007; 189: 1243-51.
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Hayley Walker, MSIII
Gillian Lieberman, MD
Approach to IVC masses: Step 2
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Once a mass is identified as tumor thrombus and not clot,
the second step is to differentiate between primary and
secondary IVC tumors
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Primary IVC Tumors
 Leiomyosarcoma is the most common malignant
primary tumor of the IVC
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Secondary IVC Tumors
 Please proceed to the next slide to view a list of
secondary IVC tumors
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Hayley Walker, MSIII
Gillian Lieberman, MD
Approach to IVC masses:
Secondary IVC tumors
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Secondary IVC Tumors
 Tumors extending contiguously from a primary tumor
 Renal cell carcinoma (most common)
 Hepatocellular carcinoma
 Adrenocortical carcinoma
 Wilms’ tumor (children)
 Leiomyosarcoma arising in retroperitoneum can
secondarily invade IVC
 Rarely, renal angiomyolipoma and pheochromocytoma
involve the IVC. Metastatic disease in retroperitoneal
lymph nodes can also extend into the IVC.
 Females: Intravenous leiomyomatosis
 Smooth muscle tumor; either arises in uterine veins or
represents extension of uterine fibroma into the IVC
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Hayley Walker, MSIII
Gillian Lieberman, MD
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You have now seen a general approach to IVC
masses
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In this patient, imaging helped to narrow the
differential diagnosis for IVC masses
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Please proceed to see images which caused the
radiologist to favor a primary IVC tumor over a
secondary tumor
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Hayley Walker, MSIII
Gillian Lieberman, MD
Narrowing the differential with imaging:
Ruling out renal or adrenal tumor
C+ coronal reconstruction
CT abdomen & pelvis
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PACS, BIDMC
Left adrenal gland (yshaped)appears normal
Both kidneys are unremarkable
C+ coronal reconstruction
CT abdomen & pelvis
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PACS, BIDMC
Right adrenal gland is difficult
to visualize, but appears normal
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Hayley Walker, MSIII
Gillian Lieberman, MD
Primary IVC tumor seemed most likely
based on CT findings
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The liver, spleen, and bowel
appeared normal. The pancreas
was deviated anteriorly by the
mass, but was otherwise
unremarkable. Lymph nodes
were not enlarged.
It seems the mass is arising
from the IVC itself, and not from
another retroperitoneal organ or
structure.
 This suggests primary IVC
tumor, with leiomyosarcoma
being most common.
C+ coronal reconstruction CT
torso
PACS, BIDMC
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Hayley Walker, MSIII
Gillian Lieberman, MD
Pathologic diagnosis: Leiomyosarcoma
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CT-guided core needle biopsy was performed to confirm the diagnosis
 Pathologic diagnosis: Leiomyosarcoma
IVC Leiomyosarcoma
 Arises from smooth muscle cells in vessel wall
 Often presents late; occasionally with leg swelling, ascites, or BuddChiari syndrome
 Most often affects lower 2/3 of IVC. Can be entirely intraluminal or
extend outside lumen
 Usually seen in women in 5th and 6th decades.
 Poor prognosis: 14% survival at 10 years
Treatment of IVC Leiomyosarcoma
 Surgery. Complete resection with microscopically negative margins
offers best hope for cure.
 Role for radiation and chemotherapy is debated.
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Hayley Walker, MSIII
Gillian Lieberman, MD
Impact of imaging on surgical
planning
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Imaging can play a role in planning surgery for resection
of an IVC tumor.
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Thrombus extension into the supradiaphragmatic IVC
requires cardiopulmonary bypass surgery
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It is important to identify superior extension of tumor prior to
surgery with CT, MRI or intraoperative ultrasound
If tumor invades vessel wall, segmental resection of IVC
is necessary
In this patient, involvement of IVC at confluence of
hepatic veins would likely necessitate partial liver
resection
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Hayley Walker, MSIII
Gillian Lieberman, MD
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The patient underwent surgery in February 2012.
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The surgeon wanted to determine whether the IVC was
involved at the level of the hepatic veins. Involvement of
the hepatic veins would necessitate partial liver resection.
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The next slide will review relevant anatomy.
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Following the anatomy review, please proceed to view
intraoperative ultrasound images.
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Hayley Walker, MSIII
Gillian Lieberman, MD
IVC anatomy
The surgeon wished
to determine whether
the IVC was involved
at the level of the
hepatic veins
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From Standring: Gray’s Anatomy, 39e. www.graysanatomyonline.com
Hayley Walker, MSIII
Gillian Lieberman, MD
Intraoperative ultrasound:
Locating the mass
The IVC mass was located on
intraoperative ultrasound
Findings:
 IVC superior to mass
 Superior aspect of IVC mass
 Liver
Ultrasound
PACS, BIDMC
Ultrasound with color Doppler
imaging
PACS, BIDMC
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Hayley Walker, MSIII
Gillian Lieberman, MD
Mass on intraoperative ultrasound
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The mass shows
mixed echogenicity
Doppler reveals
blood flow to the
mass
Ultrasound with color Doppler
imaging
PACS, BIDMC
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Hayley Walker, MSIII
Gillian Lieberman, MD
IVC is patent at level of hepatic veins on
intraoperative ultrasound
Image 1
•Image 1 shows hepatic veins
draining into a patent IVC
•Image 2, taken from a more inferior
level, shows increased echogenicity
within the IVC due to the mass
Ultrasound
Image 2
• Ultrasound demonstrated 3.5 cm
between the superior tumor edge and
the confluence of the hepatic veins.
Therefore, resection of the liver was
not necessary.
Ultrasound
PACS, BIDMC
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Hayley Walker, MSIII
Gillian Lieberman, MD
Our patient: History continued
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The mass was removed,
including a portion of the
IVC
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Possible because of
extensive collateral
circulation
Pathology showed negative
margins, and confirmed
diagnosis of
leiomyosarcoma (highgrade)
Example of collateral circulation that
allows blood to bypass the IVC
From Sonin Andrew, Mazer MJ, Powers TA. Obstruction of the
inferior vena cava: a mutliple-modality demonstration of causes,
manifestation, and collateral pathways. Radiographics 1992; 12:
309-322.
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Hayley Walker, MSIII
Gillian Lieberman, MD
Follow-up for patients with
leiomyosarcoma
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Leiomyosarcoma most commonly
metastasizes to lung and liver
 Follow-up after resection should
involve imaging of chest, abdomen,
and pelvis on a regular schedule
 Imaging is recommended every 3-6
months for 2-3 years; however, there
have been no randomized trials
comparing different surveillance
strategies
Gross leiomyosarcoma
specimen
From Sheth Sheila, Fishman EK. Imaging
of the Inferior Vena Cava with MDCT. AJR
2007; 189: 1243-51.
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Hayley Walker, MSIII
Gillian Lieberman, MD
Lung nodules on follow-up CT
C+ axial CT chest
PACS, BIDMC
August 2012
-Left lower lobe nodules noted on
follow-up CT
-Underwent VATS left lower lobe
wedge resection in August
2012. Nodules proved to be
leiomyosarcoma on pathology
C+ axial CT chest
PACS, BIDMC
December 2012
-New bilateral pulmonary nodules noted
on follow-up CT, very concerning for
progression of metastatic disease
-Evidence of prior resection can be
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seen
Hayley Walker, MSIII
Gillian Lieberman, MD
Staging of Leiomyosarcoma
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Unfortunately, this
patient has stage IV
disease
Five-year survival
estimates for stage
IV disease range
from 0-17%
From Mullen, John T, Thomas F DeLaney. Clinical features, evaluation, and treatment
of retroperitoneal soft tissue sarcoma. http://www.uptodate.com/contents/clinicalfeatures-evaluation-and-treatment-of-retroperitoneal-soft-tissuesarcoma?source=search_result&search=leiomyosarcoma&selectedTitle=5%7E66#H3
5. UpToDate. Accessed December 7, 2012.
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Hayley Walker, MSIII
Gillian Lieberman, MD
References

Sheth, Sheila, Eliot K Fishman. Imaging of the Inferior Vena Cava with MDCT. AJR 2007;189: 1243-51
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Kandpal, Harsh, Raju Sharma, Shiva Gamangatti, Deep N Srivastava, Sushma Vashisht. Imaging the
Inferior Vena Cava: A road less traveled. Radiographics 2008; 28: 669-689.
Sonin, Andrew H, Murray J Mazer, Thomas A Powers. Obstruction of the inferior vena cava: a mutliplemodality demonstration of causes, manifestation, and collateral pathways. Radiographics 1992; 12: 309322.
Kaufman, Lauren B, Benjamin M Yeh, Richard S. Breiman et al. Inferior Vena Cava Filling Defects on CT
and MRI. AJR 2005;185:717-726.
Cuervas, Carlos, Molly Raske, William H. Bush et al. Imaging primary and secondary tumor thrombus of
the inferior vena cava: multi-detector computer tomography and magnetic resonance imaging. Curr Prob
Diagn Radiol 2006;35:90-1001.
Mullen, John T, Thomas F DeLaney. Clinical features, evaluation, and treatment of retroperitoneal soft
tissue sarcoma. http://www.uptodate.com/contents/clinical-features-evaluation-and-treatment-ofretroperitoneal-soft-tissuesarcoma?source=search_result&search=leiomyosarcoma&selectedTitle=5%7E66#H35. UpToDate.
Accessed December 7, 2012.
Catalano, Onofrio A, Anandkumar H. Singh et al. Vascular and Biliary Variants in the Liver: Implications
for Liver Surgery. RadioGraphics 2008; 28:359 –37
Netter, Frank H, John Craig, Carlos Machado. Netter’s Atlas of Human Anatomy 5e online.
www.netterimages.com. Accessed 12/5/12.
Standring, Susan. Gray’s Anatomy 39e online. www.graysanatomyonline.com. Accessed 12/6/12.
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Hayley Walker, MSIII
Gillian Lieberman, MD
Acknowledgements
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Dr. Gunjan Senapati
Dr. Gillian Lieberman
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