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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 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 2 Hayley Walker, MSIII Gillian Lieberman, MD 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 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 4 Hayley Walker, MSIII Gillian Lieberman, MD Abnormal mass on 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! Axial CTA Outside hospital records 5 Hayley Walker, MSIII Gillian Lieberman, MD Our patient: History continued 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. C+ axial CT abdomen & pelvis 6 Hayley Walker, MSIII Gillian Lieberman, MD 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 C+ axial CT abdomen & pelvis PACS, BIDMC 7 Hayley Walker, MSIII Gillian Lieberman, MD Differential diagnosis for retroperitoneal mass Soft tissue sarcoma Lymphoma Primary germ cell tumor Metastatic testicular cancer 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 8 Hayley Walker, MSIII Gillian Lieberman, MD IVC mass on CT abdomen & pelvis 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 9 Hayley Walker, MSIII Gillian Lieberman, MD Imaging the IVC: Menu of Tests Conventional venography Ultrasonography 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. MR T2-weighted images as well as pre- and post-IV contrast injection T1weighted images 10 Hayley Walker, MSIII Gillian Lieberman, MD The menu of tests for imaging the IVC has been presented. Please proceed to view a general approach to IVC masses. 11 Hayley Walker, MSIII Gillian Lieberman, MD 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 Hayley Walker, MSIII Gillian Lieberman, MD 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. C+ coronal reconstruction CT abdomen & pelvis From Sheth Sheila, Fishman EK. Imaging of the Inferior Vena Cava with MDCT. AJR 2007; 189: 1243-51. 13 Hayley Walker, MSIII Gillian Lieberman, MD Approach to IVC masses: Step 2 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 Hayley Walker, MSIII Gillian Lieberman, MD 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 Hayley Walker, MSIII Gillian Lieberman, MD 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 Hayley Walker, MSIII Gillian Lieberman, MD Narrowing the differential with imaging: Ruling out renal or adrenal tumor C+ coronal reconstruction CT abdomen & pelvis PACS, BIDMC Left adrenal gland (yshaped)appears normal Both kidneys are unremarkable C+ coronal reconstruction CT abdomen & pelvis PACS, BIDMC Right adrenal gland is difficult to visualize, but appears normal 17 Hayley Walker, MSIII Gillian Lieberman, MD Primary IVC tumor seemed most likely based on CT findings 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 18 Hayley Walker, MSIII Gillian Lieberman, MD 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 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. 19 Hayley Walker, MSIII Gillian Lieberman, MD 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 20 Hayley Walker, MSIII Gillian Lieberman, MD 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 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 22 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 23 Hayley Walker, MSIII Gillian Lieberman, MD Mass on intraoperative ultrasound The mass shows mixed echogenicity Doppler reveals blood flow to the mass Ultrasound with color Doppler imaging PACS, BIDMC 24 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 25 Hayley Walker, MSIII Gillian Lieberman, MD 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 (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. 26 Hayley Walker, MSIII Gillian Lieberman, MD Follow-up for patients with leiomyosarcoma 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. 27 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 28 seen Hayley Walker, MSIII Gillian Lieberman, MD Staging of Leiomyosarcoma 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. 29 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 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. 30 Hayley Walker, MSIII Gillian Lieberman, MD Acknowledgements Dr. Gunjan Senapati Dr. Gillian Lieberman 31