Leiomyosarcoma of the Inferior Vena...

Leiomyosarcoma of the Inferior Vena Cava

Hayley Walker, Harvard Medical School, MSIII

Gillian Lieberman, MD

Hayley Walker, MSIII


Overview

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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Our Patient: History of Present Illness

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.

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.

In February 2012, he was admitted to an outside hospital with chest pain, and was found to have a pulmonary embolism (PE).

His INR at the time was therapeutic, between 2 and 2.5. He was therefore begun on enoxaparin for anti-coagulation.



3

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

4

Outside hospital records

Axial CTA

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!



Abnormal mass on CTA

5

Axial CTA

Outside hospital records

The patient had an outpatient hematology appointment in

February 2012

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.



Our patient: History continued

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C+ axial CT abdomen & pelvis

Mass on CT abdomen and pelvis

A large mass with

areas of increased

density can be seen in

the retroperitoneum

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



7 PACS, BIDMC

C+ axial CT abdomen & pelvis

Soft tissue sarcoma

Lymphoma

Primary germ cell tumor Check AFP and HCG levels to assess likelihood (both were normal in

this patient)

Metastatic testicular cancer 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



Differential diagnosis for

retroperitoneal mass

8

IVC mass on CT abdomen & 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.



9

C+ coronal reconstruction

CT abdomen and pelvis

PACS, BIDMC

Imaging the IVC: Menu of Tests Conventional venography

Historical gold standard; now rarely used

Ultrasonography 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.

MR T2-weighted images as well as pre- and post-IV contrast injection T1-

weighted images



10

The menu of tests for imaging the IVC has been presented.

Please proceed to view a general approach to IVC masses.



11

Approach to IVC masses: Step 1

The first step is to differentiate between bland thrombus (clot) and tumor thrombus Tumor thrombus suggested by:

Expansion of lumen by the thrombus

Enhancement of filling defect

Direct continuity between tumor in another organ and thrombus

Please proceed to the next slide to view an image of a companion patient which illustrates the characteristic features of tumor thrombus



12

Companion patient: tumor thrombus

on CT

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.



13

From Sheth Sheila, Fishman EK. Imaging of the Inferior Vena Cava

with MDCT. AJR 2007; 189: 1243-51.


CT abdomen & pelvis

Once a mass is identified as tumor thrombus and not clot, the second step is to differentiate between primary and secondary IVC tumors

Primary IVC Tumors

Leiomyosarcoma is the most common malignant primary tumor of the IVC

Secondary IVC Tumors

Please proceed to the next slide to view a list of secondary IVC tumors



14

Approach to IVC masses: Step 2

Approach to IVC masses: Secondary IVC tumors

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



15

You have now seen a general approach to IVC masses

In this patient, imaging helped to narrow the differential diagnosis for IVC masses

Please proceed to see images which caused the radiologist to favor a primary IVC tumor over a secondary tumor



16

Left adrenal gland (y-

shaped)appears normal

Both kidneys are unremarkable



Narrowing the differential with imaging:

Ruling out renal or adrenal tumor

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PACS, BIDMC PACS, BIDMC


CT abdomen & pelvis C+ coronal reconstruction

CT abdomen & pelvis

Right adrenal gland is difficult

to visualize, but appears normal

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.

PACS, BIDMC C+ coronal reconstruction CT

torso

Pathologic diagnosis: Leiomyosarcoma

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 Budd-

Chiari 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.



19

Impact of imaging on surgical

planning

Imaging can play a role in planning surgery for resection of an IVC tumor.

Thrombus extension into the supradiaphragmatic IVC requires cardiopulmonary bypass surgery

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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The patient underwent surgery in February 2012.

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.

The next slide will review relevant anatomy.

Following the anatomy review, please proceed to view

intraoperative ultrasound images.



21

IVC anatomy



From Standring: Gray’s Anatomy, 39e. www.graysanatomyonline.com

22

The surgeon wished

to determine whether

the IVC was involved

at the level of the

hepatic veins

Intraoperative ultrasound:

Locating the mass

The IVC mass was located on

intraoperative ultrasound

Findings:

IVC superior to mass

Superior aspect of IVC mass

Liver



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PACS, BIDMC

PACS, BIDMC

Ultrasound with color Doppler

imaging

Ultrasound

Mass on intraoperative ultrasound

The mass shows

mixed echogenicity

Doppler reveals

blood flow to the

mass



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PACS, BIDMC

Ultrasound with color Doppler

imaging

IVC is patent at level of hepatic veins on

intraoperative ultrasound



•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 demonstrated 3.5 cm

between the superior tumor edge and

the confluence of the hepatic veins.

Therefore, resection of the liver was

not necessary.

25 PACS, BIDMC

Ultrasound

Ultrasound

Image 1

Image 2

Our patient: History continued

The mass was removed,

including a portion of the

IVC

Possible because of

extensive collateral

circulation

Pathology showed negative

margins, and confirmed

diagnosis of

leiomyosarcoma (high-

grade)



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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.

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



27

Gross leiomyosarcoma

specimen

From Sheth Sheila, Fishman EK. Imaging

of the Inferior Vena Cava with MDCT. AJR

2007; 189: 1243-51.

Follow-up for patients with

leiomyosarcoma

Lung nodules on follow-up CT

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

December 2012

-New bilateral pulmonary nodules noted

on follow-up CT, very concerning for

progression of metastatic disease

-Evidence of prior resection can be

seen



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PACS, BIDMC PACS, BIDMC

C+ axial CT chest C+ axial CT chest

Staging of Leiomyosarcoma

Unfortunately, this

patient has stage IV

disease

Five-year survival

estimates for stage

IV disease range

from 0-17%

29



From 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-of-retroperitoneal-soft-tissue-

sarcoma?source=search_result&search=leiomyosarcoma&selectedTitle=5%7E66#H3

5. UpToDate. Accessed December 7, 2012.

References

Sheth, Sheila, Eliot K Fishman. Imaging of the Inferior Vena Cava with MDCT. AJR 2007;189: 1243-51

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 mutliple-modality demonstration of causes, manifestation, and collateral pathways. Radiographics 1992; 12: 309-322.

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-of-retroperitoneal-soft-tissue-sarcoma?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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Acknowledgements

Dr. Gunjan Senapati

Dr. Gillian Lieberman

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