Medical Image of the Month: Renal Cell Carcinoma with Extensive Tumor Thrombus

Figure 1. CT of the abdomen with contrast (axial image) shows a large right large heterogeneous mass (red arrow), consistent with renal cell carcinoma.

Figure 2. A: CT of the abdomen with contrast (coronal image) shows a large right renal mass (green arrow) and tumor thrombus in the IVC (orange arrow). B: Sagittal image showing extension of the tumor thrombus from the inferior vena cava into the right atrium (blue arrow). C: Axial image showing evidence of tumor thrombus in the right atrium (pink arrow).

A 53-year-old man with a right-sided renal cell carcinoma (RCC) presented with nausea, vomiting, intolerance of oral intake and melena. A contrast enhanced CT of the abdomen and pelvis showed near complete replacement of the right kidney by a large heterogeneous mass, measuring 10 x 16 cm (Figure 1). The mass invaded the renal vein and inferior vena cava (IVC) with extension to the level of the inferior cavo-atrial junction (Figure 2). The mass compressed the duodenum, causing a bowel obstruction. Liver and lung metastases were also found. A duodenal stent was placed with significant improvement in his nausea and vomiting. He was not able to receive anticoagulation due to severe gastrointestinal bleeding. The patient discontinued disease modifying therapy and died four weeks after discharge from the hospital.

Tumor thrombus occurs when a tumor invades a blood vessel. It occurs in approximately 10% of patients with renal cell carcinoma, which is a highly vascular malignancy with a propensity to invade the venous system (1). Extension of the tumor from the inferior vena cava into the right atrium is very uncommon, seen in only about 1% of RCCs (1). The American Joint Committee on Cancer staging system for RCC differentiates between tumor thrombus involving the renal vein (T3a), IVC below the diaphragm (T3b) and IVC above the diaphragm (T3c) (1). The presence of tumor thrombus changes staging, prognosis and surgical options. Surgical treatment may be the approach to tumor thrombus in RCC without metastatic disease. The surgical approach is often complex and requires extensive surgical planning and expertise (2). Perioperative morbidity and mortality appear to be proportional to the height of tumor growth, and tumor thrombus extending above the diaphragm carries increased perioperative risk. Wagner et al. (3) retrospectively studied 1,192 cases, and found reduced long-term survival in patients with any venous involvement. However, they found no significant difference in long-term survival between patients with IVC tumor thrombus below (T3b) or above (T3c) the diaphragm. In this study, the most important prognostic factors in RCC included renal tumor size, the presence of perinephric fat invasion, lymph node involvement and distant metastatic lesions.

David Horn MD, Sue Cassidy ANP-BC and Linda Snyder MD

Departments of Internal Medicine and Pulmonary, Critical Care, Allergy and Sleep Medicine

University of Arizona College of Medicine

Tucson, AZ USA

References

1. Wotkowicz C, Wszolek MF, Libertino JA. Resection of renal tumors invading the vena cava. Urol Clin N Am. 2008; 35: 657-71. [CrossRef] [PubMed]
2. Quencer KB, Friedman T, Sheth R, Rahmi O. Tumor thrombus: incidence, imaging, prognosis and treatment. Cardiovasc Diagn Ther. 2017;7(Suppl 3):S165-77. [CrossRef] [PubMed]
3. Wagner B, Patard JJ, Méjean A, et al. Prognostic value of renal vein and inferior vena cava involvement in renal cell carcinoma. Eur Urol. 2009;55:452-9. [CrossRef] [PubMed]

Cite as: Horn D, Cassidy S, Snyder L. Medical image of the month: renal cell carcinoma with extensive tumor thrombus. Southwest J Pulm Crit Care. 2019;19(3):95-6. doi: https://doi.org/10.13175/swjpcc031-19 PDF 

May 2016 Imaging Case of the Month

Michael B. Gotway, MD

Department of Radiology

Mayo Clinic Arizona

Scottsdale, AZ USA

Imaging Case of the Month CME Information  

Members of the Arizona, New Mexico, Colorado and California Thoracic Societies and the Mayo Clinic are able to receive  0.25 AMA PRA Category 1 Credits™. Completion of an evaluation form is required to receive credit and a link is provided on the last panel of the activity.

0.25 AMA PRA Category 1 Credit(s)™

Estimated time to complete this activity: 0.25 hours

Lead Author(s): Michael B. Gotway, MD. All Faculty, CME Planning Committee Members, and the CME Office Reviewers have disclosed that they do not have any relevant financial relationships with commercial interests that would constitute a conflict of interest concerning this CME activity. 

Learning Objectives:
As a result of this activity I will be better able to:    

1. Correctly interpret and identify clinical practices supported by the highest quality available evidence.
2. Will be better able to establsh the optimal evaluation leading to a correct diagnosis for patients with pulmonary, critical care and sleep disorders.
3. Will improve the translation of the most current clinical information into the delivery of high quality care for patients.
4. Will integrate new treatment options in discussing available treatment alternatives for patients with pulmonary, critical care and sleep related disorders.

Learning Format: Case-based, interactive online course, including mandatory assessment questions (number of questions varies by case). Please also read the Technical Requirements.

CME Sponsor: University of Arizona College of Medicine at the Arizona Health Sciences Center.

Current Approval Period: January 1, 2015-December 31, 2016

Financial Support Received: None.

Clinical History:  A 58-year-old man with hypertension presents for a routine health examination. As part of his routine evaluation, frontal and lateral chest radiography (Figure 1) was performed.  

Figure 1. Frontal (panel A) and lateral (panel B) chest radiography.

Which of the following statements regarding the chest radiograph is most accurate? (Click on the correct answer to proceed to the second of eight panels)

1. The frontal chest radiograph shows a small lung nodule
2. The frontal chest radiograph shows a small metallic focus just posterior to the inferior sternum
3. The frontal chest radiograph shows an unusual right-sided mediastinal contour
4. The frontal chest radiograph shows asymmetrically increased attenuation of the left thorax compared with the right
5.  The frontal chest radiograph shows normal findings

Cite as: Gotway MB. May 2016 imaging case of the month. Southwes J Pulm Crit Care. 2016 May;12(5):180-91. doi: http://dx.doi.org/10.13175/swjpcc040-16 PDF 

Medical Image of the Week: Scimitar Syndrome

Figure 1. Axial CT in lung windows at the level of the right atrium shows a dilated anomalous vein (arrow) coursing in close proximity to the major fissure (star).

Figure 2. Coronal CT in soft tissue windows at the level of the right hemi diaphragm shows evidence of post surgical repair with the anomalous vein draining into the left atrium (arrow).  Note the right hemi-diaphragm is elevated suggesting some degree of pulmonary hypoplasia.

A 38 year-old woman presented for evaluation of palpitations and chronic progressive dyspnea on exertion accompanied by chest tightness and fatigue. Chest radiograph was normal except for low lung volumes. An echocardiogram revealed normal left ventricular size and function with an ejection fraction of 60%, normal right ventricular size and function and moderate tricuspid insufficiency with an estimated right ventricular systolic pressure of 36 mm Hg plus central venous pressure and a mildly enlarged right atrium. Computed tomography (CT) of the heart with contrast showed normal coronary arteries, enlarged right atrium and partial anomalous pulmonary venous return from the right lung to the inferior vena cava (IVC) (Figure 1). Cardiac catheterization with selective angiography confirmed anomalous pulmonary venous drainage from the right upper and right lower lobe to the hepatic portion of the inferior vena cava with obstruction (8 mm Hg gradient between the anomalous vein and the right atrium). The calculated pulmonary to systemic flow ratio (Qp:Qs) was 1.3:1. The pulmonary vascular resistance was 7.6 Woods units and the mean pulmonary artery pressure was 24 mmHg.  The diagnosis of Scimitar syndrome was made.

The patient underwent surgical repair of partial anomalous pulmonary drainage and pulmonary vein stenosis as well as ligation of an aortopulmonary collateral artery found intraoperatively. The anomalous pulmonary vein was divided from the IVC, the caval end was oversewn and the anomalous vein was anastomosed to the left atrium with a CorMatrix patch (Figure 2). The patient recovered from surgery, however, her symptoms continued and Doppler pattern on a follow-up transthoracic echocardiogram suggested residual obstruction at the site of anastomosis to the left atrium.  Catheterization confirmed obstruction of the anastomosed vein at the level of insertion into the left atrium.  The patient underwent repeat sternotomy and repair of pulmonary vein obstruction using a CorMatrix patch and sutureless reconstruction of the right-sided pulmonary vein obstruction.  She recovered from this procedure with some improvement of her symptoms.

Scimitar syndrome is a rare congenital anomaly characterized by partial anomalous pulmonary venous connection of the right pulmonary veins to the IVC, anomalous systemic arterial supply to the right lung and a variable degree of right lung hypoplasia with or without sequestration (1).  Partial anomalous pulmonary venous return (PAPVR) is an uncommon congenital anomaly and accounts for less than 1% of congenital heart lesions. Scimitar syndrome comprises 3-5% of PAPVR (2). The term refers to the distinctively shaped vein, which resembles a short, curved Turkish sword called a scimitar (Figure 2). Most cases present in infancy and childhood and diagnosis in adulthood is quite rare. In adults, the clinical presentation is variable and the lesion may be discovered incidentally or patients can present with dyspnea, pulmonary hypertension and recurrent right-sided lung infection (1). The treatment of isolated PAPVR in adults is controversial due to rare occurrence of the disease, complexity of surgical repair and risk of re-stenosis. Surgical repair involves re-implantation of the anomalous vein into the left atrium and can be a complex and difficult procedure, however it can be accomplished with low morbidity and mortality at specialized centers (3). Thrombosis or stenosis of the scimitar vein is a serious complication of surgery. Our patient demonstrates some of the challenges in diagnosis and treatment and illustrates some of the post-operative complications of this rare disease.

Jamie Colombo, DO¹

Linda Snyder, MD²

Veronica Arteaga, MD³

Daniela Lax, MD⁴

¹Department of Pediatrics

²Department of Medicine, Division of Pulmonary/Critical Care Medicine

³Department of Medical Imaging

⁴Department of Pediatrics, Division of Pediatric Cardiology

University of Arizona

Tucson, AZ

References

1. Yehia BR, Bachmann JM, Traill TA. Scimitar syndrome: a rare cause of dyspnea in adults. South Med J. 2010;103:578-580. [CrossRef] [PubMed]
2. Haest RJ, van den Berg CJ, Goei R, Baur LH. Scimitar syndrome; an unusual congenital abnormality occasionally seen in adults. Int J Cardiovasc Imaging. 2006;22:565-568. [CrossRef] [PubMed]
3. Majdalany DS, Phillips SD, Dearani JA, Connolly HM, Warnes CA. Isolated partial anomalous venous connections in adults: twenty-year experience. Congenit Heart Dis. 2010;5:537-545. [CrossRef] [PubMed]

Reference as: Colombo J, Snyder L, Arteaga V, Lax D. Medical image of the week: scimitar syndrome. Southwest J Pulm Crit Care. 2014;9(2):137-9. doi: http://dx.doi.org/10.13175/swjpcc111-14 PDF