Inferior vena cava leiomyosarcoma
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At the time the article was created Francis Deng had no recorded disclosures.View Francis Deng's current disclosures
At the time the article was last revised Michael P Hartung had the following disclosures:
- Otsuka Pharmaceutical, Consultant (ongoing)
- Innovenn, Inc, Consultant (ongoing)
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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.
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Three-quarters of cases occur in women, usually aged 40-60 years 2.
When symptomatic, patients most commonly present with abdominal pain or distension 8. A minority of patients present with lower extremity deep venous thrombosis 8.
Leiomyosarcomas of the inferior vena cava are slow-growing malignancies 1.
As with other leiomyosarcomas, the histologic origin is smooth muscle cells. These are present in the vessel wall.
The tumors may be classified by the segment of inferior vena cava they involve, which carries implications for surgical planning and prognostication 1,5:
- 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 7,8.
The tumor may be intraluminal, extraluminal, or both 3-5,8. The presence of a tumor limited to and expanding the inferior vena cava is most likely an inferior vena cava leiomyosarcoma 1.
After identifying a retroperitoneal mass that contacts the inferior vena cava, CT features may help distinguish the origin 4:
- 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 5.
For tumors with an extraluminal component, CT-guided core needle biopsy is typically employed to obtain tissue and establish the diagnosis 1.
For intraluminal tumors, venography may show obstruction of the inferior vena cava and presence of collateral veins 9. The tumor itself demonstrates neovascularity with marked parenchymal tumor blush 9.
For tumors with predominantly intraluminal growth, transvenous biopsy is a feasible alternative to percutaneous biopsy 1.
Signal characteristics depend on the degree of necrosis, which appears low intensity on T1-weighted images and high intensity on T2-weighted images 1.
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 1.
Treatment and prognosis
The treatment includes surgical resection, most commonly with inferior vena cava ligation and primary/patch repair 8. The prognosis is poor. Recurrence affects half of the patients and the 5-year survival is 33% 8.
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 5.
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:
- 1. Bednarova I, Frellesen C, Roman A, Vogl TJ. Case 257: Leiomyosarcoma of the Inferior Vena Cava. (2018) Radiology. 288 (3): 901-908. doi:10.1148/radiol.2018160821 - Pubmed
- 2. Monteagudo Cortecero J, Guirau Rubio MD, Payá Romá A. Leiomyosarcoma of the inferior vena cava: AIRP best cases in radiologic-pathologic correlation. (2015) Radiographics : a review publication of the Radiological Society of North America, Inc. 35 (2): 616-20. doi:10.1148/rg.352130105 - Pubmed
- 3. Hartman DS, Hayes WS, Choyke PL, Tibbetts GP. From the archives of the AFIP. Leiomyosarcoma of the retroperitoneum and inferior vena cava: radiologic-pathologic correlation. (1992) Radiographics : a review publication of the Radiological Society of North America, Inc. 12 (6): 1203-20. doi:10.1148/radiographics.12.6.1439022 - Pubmed
- 4. Webb EM, Wang ZJ, Westphalen AC, Nakakura EK, Coakley FV, Yeh BM. Can CT features differentiate between inferior vena cava leiomyosarcomas and primary retroperitoneal masses?. (2013) AJR. American journal of roentgenology. 200 (1): 205-9. doi:10.2214/AJR.11.7476 - Pubmed
- 5. Smillie RP, Shetty M, Boyer AC, Madrazo B, Jafri SZ. Imaging evaluation of the inferior vena cava. (2015) Radiographics : a review publication of the Radiological Society of North America, Inc. 35 (2): 578-92. doi:10.1148/rg.352140136 - Pubmed
- 6. Mu D, Wang D, Zhou K, Zhu B. Radiographic features of intraluminal leiomyosarcoma of the inferior vena cava: an atypical case report. (2011) Abdominal imaging. 36 (5): 586-9. doi:10.1007/s00261-010-9673-x - Pubmed
- 7. Ganeshalingam S, Rajeswaran G, Jones RL, Thway K, Moskovic E. Leiomyosarcomas of the inferior vena cava: diagnostic features on cross-sectional imaging. (2011) Clinical radiology. 66 (1): 50-6. doi:10.1016/j.crad.2010.08.004 - Pubmed
- 8. Hollenbeck ST, Grobmyer SR, Kent KC, Brennan MF. Surgical treatment and outcomes of patients with primary inferior vena cava leiomyosarcoma. (2003) Journal of the American College of Surgeons. 197 (4): 575-9. doi:10.1016/S1072-7515(03)00433-2 - Pubmed
- 9. Tegtmeyer CJ, Buschi A. The angiographic diagnosis of leiomyosarcoma of the inferior vena cava. (1977) Radiology. 122 (3): 683-5. doi:10.1148/122.3.683 - Pubmed
- 10. Narata M, Okuhata Y, Abe K, Takemoto A, Maebayashi T, Furuhashi S, Takahashi M. Primary leiomyosarcoma of the inferior vena cava: case report. (2010) Abdominal imaging. 35 (4): 481-4. doi:10.1007/s00261-009-9549-0 - Pubmed