Inferior vena cava leiomyosarcoma

Primary
leiomyosarcoma of inferior vena cava
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IVC
leiomyosarcoma. CECT coronal reformatted image of abdomen showing inhomogenously enhancing lesion in IVC with minimal extension into both renal veins.
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Pleomorfic
leiomyosarcoma of the inferior vena cava. A-D)The MR demonstrated that the lobulated mass presented partial invasion of the right renal artery (black arrow), the right suprarenal gland (white arrow head) and the caudate lobe (asterisk).
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Pleomorfic
leiomyosarcoma of the inferior vena cava. A-D)The MR demonstrated that the lobulated mass presented partial invasion of the right renal artery (black arrow), the right suprarenal gland (white arrow head) and the caudate lobe (asterisk).
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Pleomorfic
leiomyosarcoma of the inferior vena cava. A-D)The MR demonstrated that the lobulated mass presented partial invasion of the right renal artery (black arrow), the right suprarenal gland (white arrow head) and the caudate lobe (asterisk).
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Pleomorfic
leiomyosarcoma of the inferior vena cava. A-D)The MR demonstrated that the lobulated mass presented partial invasion of the right renal artery (black arrow), the right suprarenal gland (white arrow head) and the caudate lobe (asterisk) .
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Pleomorfic
leiomyosarcoma of the inferior vena cava. B) Coronal projection. Large mass that affects the inferior vena cava (white arrow). The mass extends from the right renal vein to the liver.
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Pleomorfic
leiomyosarcoma of the inferior vena cava. Abdominal CT with intravenous contrast. A) Axial projection. B) Coronal projection. Large mass that affects the inferior vena cava (white arrow). The mass extends from the right renal vein to the liver.
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IVC
leiomyosarcoma. CT thorax lung window axial image shows multiple parenchymal nodular lesions of variable sizes in both lung fields.
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IVC
leiomyosarcoma. CECT axial image
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IVC
leiomyosarcoma. CECT coronal reformatted image of abdomen reveals an inhomogenously enhancing lesion in midlle and lower segment of IVC with bland thrombus at its cranial end.
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Primary
leiomyosarcoma of inferior vena cava. Lobulated mass within the suprarenal and intrahepatic segments of IVC, distending the vein. The mass stops short of the hepatic veins.
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Retroperitoneal
leiomyosarcoma • Leiomyosarcoma of the inferior vena cava - Ganzer Fall bei Radiopaedia
Hartung M, Leiomyosarcoma of the inferior vena cava. Case study, Radiopaedia.org (Accessed on 06 Jan 2023) https://doi.org/10.53347/rID-73588 • CC by-nc-sa 3.0 (modified)
Primary
leiomyosarcoma of inferior vena cava. Lobulated, enhancing mass in the IVC with extraluminal component anteriorly. A bland thrombus is also seen in distal IVC.
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Primary
leiomyosarcoma of inferior vena cava. Heterogeneously enhancing retroperitoneal mass which is in the location of IVC. Note the dilated collateral veins posterior to liver.
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Primary
leiomyosarcoma of inferior vena cava. Large lobulated heterogeneously enhancing mass involving lower and mid segments of IVC extending into right renal vein. The mass stops short of the hepatic veins. Thrombus is also seen in distal IVC.
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Primary
leiomyosarcoma of inferior vena cava. Hypoechoeic mass seen medial to right renal hilum involving renal vein and IVC.
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Primary
leiomyosarcoma of inferior vena cava. Hypervascular mass anterior and to the right of abdominal aorta. The lesion is inseparable from the IVC. Note the dilated subcutaneous veins in anterior abdominal wall.
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Pleomorfic
leiomyosarcoma of the inferior vena cava. PET-CT study. A maximum SUV of 3.4 of the mass is demonstrated. Other foci of hypermetabolism are not identified.
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Inferior vena cava leiomyosarcomas are the most common type of retroperitoneal leiomyosarcoma and most common primary malignancy of the inferior vena cava.

Epidemiology

Three-quarters of cases occur in women, usually aged 40-60 years .

Clinical presentation

When symptomatic, patients most commonly present with abdominal pain or distension . A minority of patients present with lower extremity deep venous thrombosis .

Pathology

Leiomyosarcomas of the inferior vena cava are slow-growing malignancies .

Etiology

As with other leiomyosarcomas, the histologic origin is smooth muscle cells. These are present in the vessel wall.

Location

The tumors may be classified by the segment of inferior vena cava they involve, which carries implications for surgical planning and prognostication :

  • hepatic (6-24%)
  • renal and suprarenal (42-50%)
  • infrarenal (34-44%)

Metastatic disease occurs in up to half of cases at presentation, most commonly involving liver, followed by lung .

Radiographic features

The tumor may be intraluminal, extraluminal, or both . The presence of a tumor limited to and expanding the inferior vena cava is most likely an inferior vena cava leiomyosarcoma .

CT

After identifying a retroperitoneal mass that contacts the inferior vena cava, CT features may help distinguish the origin :

  • in inferior vena cava leiomyosarcomas, the inferior vena cava wall is usually imperceptible at the point of maximal contact with the mass
  • in other retroperitoneal tumors, the inferior vena cava is usually compressed at the perimeter of the mass (negative embedded organ sign)

On contrast-enhanced CT, if the tumor has an intraluminal component, it appears as a heterogeneously enhancing filling defect in the inferior vena cava .

For tumors with an extraluminal component, CT-guided core needle biopsy is typically employed to obtain tissue and establish the diagnosis .

Angiography

For intraluminal tumors, venography may show obstruction of the inferior vena cava and presence of collateral veins . The tumor itself demonstrates neovascularity with marked parenchymal tumor blush .

For tumors with predominantly intraluminal growth, transvenous biopsy is a feasible alternative to percutaneous biopsy .

MRI

Signal characteristics depend on the degree of necrosis, which appears low intensity on T1-weighted images and high intensity on T2-weighted images .

In contrast to bland thrombus, intraluminal leiomyosarcoma tumor thrombus appears iso- to hyperintense on T2-weighted images, enhances on T1-weighted postcontrast images, and expands the lumen .

Treatment and prognosis

The treatment includes surgical resection, most commonly with inferior vena cava ligation and primary/patch repair . The prognosis is poor. Recurrence affects half of the patients and the 5-year survival is 33% .

The best prognosis is associated with tumors in the renal and suprarenal inferior vena cava, while the worst prognosis is associated with tumors in the hepatic segment of the inferior vena cava .

Differential diagnosis

Intraluminal tumor should be distinguished from bland thrombus (inferior vena cava thrombosis).

The differential includes other less common retroperitoneal mesenchymal neoplasms that may involve the inferior vena cava:

Involvement of adjacent organs should be identified, raising the more likely possibility of tumor thrombus extending from those organs into the inferior vena cava:

Siehe auch:
Assoziationen und Differentialdiagnosen zu Leiomyosarkom der Vena cava inferior:
Leiomyosarkom
 
Wikipedia • CC BY-SA 3.0
Vena cava
inferior Thrombose
Wikipedia • CC BY-SA 4.0
Leiomyosarkom
von Venen
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Tumoren der
Venen
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Leiomyosarkom
des Pankreas
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