(Radiographics. 1999;19:248-251.)
© RSNA, 1999
Gastrointestinal Case of the Day
Warren L. Reuther, III, MD1,
Cary A. Newman, MD1,
Robert E. Smith, MD1 and
Branko M. Plavsic, MD, PhD1
1 Department of Radiology, SL 54, Tulane University Medical Center, 1430 Tulane Ave, New Orleans, LA 70112.
Index Terms: Hepatic veins, CT, 982.12912 • Hepatic veins, thrombosis, 95.751, 982.659 • Leiomyosarcoma, 982.322 • Liver, CT, 761.12112
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HISTORY
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A 37-year-old woman with no history of medical problems presented to the emergency department with shortness of breath and vague right upper abdominal pain. Physical examination revealed edema of the lower extremities and hepatomegaly. Abdominal ultrasound (US) and computed tomography (CT) were performed.
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FINDINGS
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Doppler US exhibited absence of venous flow within the inferior vena cava (IVC) and hepatic veins (Fig 1). CT of the chest and abdomen with intravenously administered contrast material showed elevation of the right hemidiaphragm and a heterogeneously enhancing mass within the IVC extending into the liver and right atrium (Fig 2). There was mottled enhancement of the liver following contrast material injection with no enhancement of the hepatic veins (Fig 2b). Collateral pathways (the azygos and hemiazygos veins) were enlarged (Fig 2a). In addition, there was blurring of the retroperitoneal vascular structures, mesenteric congestion, and mild anasarca of the abdominal wall soft tissues (Fig 2c).
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Figure 2a. (a) Contrast material–enhanced CT scan of the chest demonstrates a large mass within the right atrium with focal areas of enhancement (arrowheads). The azygos and hemiazygos veins are enlarged (arrows). There are small, bilateral pleural effusions, and the right hemidiaphragm is elevated secondary to an enlarged liver. (b) Contrast-enhanced CT scan of the upper abdomen shows a large, mildly enhancing mass within the hepatic portion of the IVC (arrowheads) extending into the liver. The mass is seen compressing the portal vein and obstructing hepatic venous outflow. The hepatic veins are not enhanced, and there is mottled enhancement of the liver parenchyma. (c) Contrast-enhanced CT scan of the lower abdomen shows a tumor within the IVC and enhancement of the IVC wall (arrowhead). There is an area of soft-tissue attenuation surrounding the retroperitoneal vascular structures as well as mesenteric venous congestion and mild anasarca of the abdominal wall soft tissues (arrows).
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Figure 2b. (a) Contrast material–enhanced CT scan of the chest demonstrates a large mass within the right atrium with focal areas of enhancement (arrowheads). The azygos and hemiazygos veins are enlarged (arrows). There are small, bilateral pleural effusions, and the right hemidiaphragm is elevated secondary to an enlarged liver. (b) Contrast-enhanced CT scan of the upper abdomen shows a large, mildly enhancing mass within the hepatic portion of the IVC (arrowheads) extending into the liver. The mass is seen compressing the portal vein and obstructing hepatic venous outflow. The hepatic veins are not enhanced, and there is mottled enhancement of the liver parenchyma. (c) Contrast-enhanced CT scan of the lower abdomen shows a tumor within the IVC and enhancement of the IVC wall (arrowhead). There is an area of soft-tissue attenuation surrounding the retroperitoneal vascular structures as well as mesenteric venous congestion and mild anasarca of the abdominal wall soft tissues (arrows).
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Figure 2c. (a) Contrast material–enhanced CT scan of the chest demonstrates a large mass within the right atrium with focal areas of enhancement (arrowheads). The azygos and hemiazygos veins are enlarged (arrows). There are small, bilateral pleural effusions, and the right hemidiaphragm is elevated secondary to an enlarged liver. (b) Contrast-enhanced CT scan of the upper abdomen shows a large, mildly enhancing mass within the hepatic portion of the IVC (arrowheads) extending into the liver. The mass is seen compressing the portal vein and obstructing hepatic venous outflow. The hepatic veins are not enhanced, and there is mottled enhancement of the liver parenchyma. (c) Contrast-enhanced CT scan of the lower abdomen shows a tumor within the IVC and enhancement of the IVC wall (arrowhead). There is an area of soft-tissue attenuation surrounding the retroperitoneal vascular structures as well as mesenteric venous congestion and mild anasarca of the abdominal wall soft tissues (arrows).
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DIAGNOSIS: Primary leiomyosarcoma of the IVC with Budd-Chiari syndrome.
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DISCUSSION
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Primary leiomyosarcoma of the IVC is a rare but well-recognized cause of Budd-Chiari syndrome (1). Retroperitoneal leiomyosarcoma exhibits three major growth patterns: extravascular, intravascular, and combined (2). Intravascular leiomyosarcoma may be accompanied by symptoms referable to venous thrombosis.
The acquired type of Budd-Chiari syndrome results from sudden or gradual occlusion of the hepatic veins, the IVC, or both (3). The patient may complain of shortness of breath secondary to decreased cardiac return. At physical examination, the patient may be jaundiced and display lower-extremity edema. Liver function test results are nonspecific and may show moderately elevated bilirubin and alkaline phosphatase levels. The condition later known as Budd-Chiari syndrome was first described by Budd in 1845 (4), and additional information was provided by Chiari in 1899 (5). In nearly two-thirds of patients, the cause cannot be determined. Various factors have been associated with obstruction of hepatic venous drainage, including trauma, medications, congenital abnormalities, and neoplasms. The diagnosis is difficult to make clinically; therefore, radiology plays a critical role.
US, CT, and angiography all provide valuable diagnostic information. Normally, sonographic studies of the IVC and hepatic veins show phasic flow toward the heart. In Budd-Chiari syndrome, blood flow in the IVC and hepatic veins may be absent, reversed, or turbulent depending on the degree of obstruction. Unenhanced CT demonstrates global liver enlargement with diffuse liver hypoattenuation. Contrast-enhanced CT demonstrates a mottled enhancement pattern with typical delayed enhancement of the liver periphery (2). Hepatic congestion prevents contrast material from readily diffusing throughout the liver in a normal fashion. The poorly enhanced portions of the liver are the result of decreased portal flow, venous congestion, and, rarely, infarcts. In addition, the hepatic veins are not well visualized. The caudate lobe has a separate venous drainage directly into the IVC and may appear normal or actually enlarge secondary to shunted portal blood flow. Occasionally, abdominal collateral venous distention and mild splenomegaly may be detected. In our patient, the azygos and hemiazygos veins were enlarged, representing collateral pathways bypassing the IVC. Associated portal hypertension may also cause poor enhancement of the spleen. Hemorrhage from esophageal varices is a known complication. Also, lower-extremity and abdominal soft-tissue lymphedema may be present (5,6). In our patient, contrast-enhanced CT demonstrated a lesion that was suspected to be a neoplasm on the basis of the enhancement pattern seen within the hepatic veins, IVC, and right atrium.
Intraluminal leiomyosarcomas are the most common intravascular venous tumors. Tumor thrombus should be distinguished from nonneoplastic IVC occlusion. Findings that suggest neoplastic thrombus include enlargement of the vessel lumen, enhancement of the mass on CT or magnetic resonance images, and neovascularity. Other tumors that may extend from the IVC into the right atrium include renal cell carcinoma and, less commonly, hepatocellular carcinoma.
The patient subsequently underwent debulking surgery but died during the procedure. Gross specimens of the heart, IVC, and liver obtained at autopsy demonstrated extensive tumor growth obstructing hepatic venous flow at different anatomic levels, which accounted for the classic radiographic manifestation of Budd-Chiari syndrome (Fig 3). Histologic analysis helped confirm the diagnosis of a low-grade leiomyosarcoma (Fig 4).
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Figure 3a. (a) Photograph of a gross autopsy specimen demonstrates leiomyosarcoma occluding the IVC (arrows) and extending into the right atrium. (b) Photograph of the cut surface of the liver demonstrates the "nutmeg" appearance of hepatic venous congestion.
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Figure 3b. (a) Photograph of a gross autopsy specimen demonstrates leiomyosarcoma occluding the IVC (arrows) and extending into the right atrium. (b) Photograph of the cut surface of the liver demonstrates the "nutmeg" appearance of hepatic venous congestion.
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Footnotes
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Address reprint requests to B.M.P.
From the 1998 RSNA scientific assembly.
Received for publication July 29, 1998.
Revision received August 28, 1998. September 10, 1998.
Accepted for publication September 10, 1998.
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References
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Cacoub P, Piette JC, Wechseler B, et al. Leiomyosarcoma of the inferior vena cava: experience with seven patients and literature review. Medicine 1991; 70:293-296.[Medline]
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Hartman DS, Hayes WS, Choyke PL, Tibbetts GP. Leiomyosarcoma of the retroperitoneum and inferior vena cava: radiologic-pathologic correlation. RadioGraphics 1992; 12:1203-1220.[Abstract]
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Mathieu D, Basile N, Menu Y, et al. Budd-Chiari syndrome: dynamic CT. Radiology 1987; 165:409-413.[Abstract/Free Full Text]
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Budd G. On disease of the liver London, England: Churchill, 1845; 141-152.
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Chiari H. Uber die selbstandige phlebitis obliterans der haupstaemme der venae hepaticae als todesursache. Beitr Pathol Anat 1899; 26:1-17.
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Murphy FB, Steinberg HV, Shires GT, III, et al. The Budd-Chiari syndrome: a review. AJR 1986; 147:9-25.[Abstract/Free Full Text]
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HISTORY
FINDINGS
