Tumors That Invade the Inferior Vena Cava: an Illustrative Review of the Main Imaging Features on Computed Tomography and Magnetic Resonance

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Rev ChilRadiol 2016; 22(1): 39-46. Tumors that invade the inferior vena cava: An illustrative review of the main imaging features on computed tomography and magnetic resonance

* Matías Molina , Giancarlo Schiappacasse y Andrés Labra

Facultad de Medicina, Universidad del Desarrollo-Clínica Alemana, Santiago, Chile

Abstract obtain healing results if other secondary locations or The objective of this article is to describe the multi- invasion of adjacent organs is ruled out(1-3). ple entities that can affect and invade the inferior vena This pictographic review aims to expose the main cava. Among these we can list not only benign entities, entities that can invade or infiltrate this structure. but with an aggressive behavior, but also malignan- Among these several of benign strain that can adopt an cies that originate in organs adjacent to this vascular aggressive behavior and invade neighboring vascular structure or therein such as leiomyosarcoma. In this structures, including the IVC, and on the other hand, review different examples on computed tomography malignant neoplastic entities that spread through it and magnetic resonance with cases of angiomyolipo- via endovascular areas or by direct local extension. ma, pheochromocytoma, adrenal carcinoma, renal cell In addition, we mention the various pseudo lesions carcinoma, hepatocellular carcinoma, retroperitoneal affecting the IVC, the majority benign and which can sarcoma and leiomyosarcoma originating in the inferior lead to errors when interpreting the images. vena cava, are presented. In addition, situations that may lead to misdiagnosis such as flow artifacts and Renal angiomyolipoma pseudolipoma are presented. This is a rare tumor of mesenchymal origin, with a Keywords: CT, Inferior vena cava, Radiology, Tumors. structure composed of vascular, smooth muscle and fatty elements. It presents sporadically or in association Introduction with tuberous sclerosis. By nature, the vast majority is Currently there is a significant increase in the acqui- benign, however it can have an aggressive evolution sition of images for diagnostic purposes. The use of invading the renal vein, IVC and even the right atrium(4,5) computed tomography (CT) and magnetic resonance (Figures 1 and 2). imaging (MRI) are becoming more frequent and thus the detection of abnormalities of the inferior vena cava (IVC) has become more common, so the radiologist should be familiarized with the various entities that may affect it. The development of new imaging modalities has made it possible to improve the processes of detection, staging and monitoring of neoplasms originating in the surrounding structures, as well as provide information to clinical and surgical oncologists regarding the local and distant extension of the various types of abdomi- nal tumors. For its part, the affection of the IVC may be due basically to 2 types. The first corresponds to a tumoral thrombus occupying the vessel lumen and the second to the invasion and infiltration as such of the vascular wall. This differentiation is essential when Figure 1. Venous phase axial and coronal CT images. making surgical decisions and defining the therapeutical Shown here is a Lipomatosa strain lesion in the upper third management. Thus, the intraluminal extension of the of the left kidney, which invades the ipsilateral renal vein tumor may be susceptible for complete resection and and inferior vena cava (arrow).

Received 25th February 2016; accepted 26th February 2016, Available on Internet 3rd April 2016 © 2016 Published by Elsevier Spain, S.L.U. on behalf of SOCHRADI. This is an Open Access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). *Autor for correspondence. email address: [email protected] (M. Molina). 39 Molina M, et al. Rev ChilRadiol 2016; 22(1): 39-46.

Some authors have reported that the invasion of the un- derlying venous structures can occur between 4-10%(8,9). This information, at the moment of issuing the report, is of fundamental importance because some groups have reported that it affects patient survival and also determines the surgical technique (Figures 5 and 6). In the particular case of renal cell carcinoma, invasion of the vena cava by tumor thrombus does not contrain- Figure 2. Selective aortogram and arteriography of left renal dicate surgical resection, with the possibility existing of artery is performed via the right femoral artery, with stenosis (3) approximately 1 cm distal to the ostium being visible. The resecting the tumoral thrombus . On the other hand, stenosis causes a reduction of 50% in the diameter of the it is important to confirm the extension of the thrombus, artery and 75% of the area thereof. either infra or supradiaphragmatic, the latter being the worst prognosis, and also if the vascular wall is infiltrated, Pheochromocytoma which involves resecting the vascular segment block, Another tumor that can invade the IVC, both in its with the respective implications both in the immediate malignant and benign versions is the pheochromocyto- postsurgical outcome as well as long term(3,9). ma, which corresponds to a rare tumor composed of chromaffin cells originating from neuroectodermal tissue of the adrenal medulla and which can be a producer of catecholamines. Traditionally these have been referred to as “the 10 percent” tumor, because this percentage is applicable to various features. Thus, 10% are extra-adrenal, 10% malignant and 10% are associated with particular syndromes such as multiple endocrine neoplasia IIA and III, Von Hippel Lindau syndrome and neurofibromatosis(6). Pheochromocytomas may present a locally aggressive Figure 4. MR imaging sequences T2 Fat-Sat and T1 respectively. A solid heterogeneous and hyperintense mass on T2-weighted behaviour, invading structures such as the renal capsule sequence and isointense on T1 was identified, adjacent to or the IVC (Figures 3 and 4). the vascular hilum of the right kidney (arrows), which invades the IVC lumen and which corresponds to an extra-adrenal pheochromocytoma.

Figure 5. Venous phase CT coronal reconstructions. It features a large mass in the upper pole of the right kidney with invasion of the IVC extending to the right atrium compatible with renal cell carcinoma.

Figure 3. Axial and coronal CT images pre and post-contrast (venous phase). Shown is a right adrenal solid tumor of heterogeneous enhancement (arrows) and its invasion of the IVC determining also thrombosis of same toward flow (curved arrows). Histopathological result shows a pheochromocytoma.

Renal cell carcinoma This corresponds to the most common kidney cancer Figure 6. MR images in post-Gd T1, T2 FSE axial and coronal SSFP sequences. A heterogeneous mass that affects the upper subtype and represents about 2% of malignant cancers half of the right kidney and invades the renal vein and the IVC, (7) affecting adults . Usually renal cell carcinoma (RCC) is shown. In the post-contrast sequence enhancement of the has an expansive growth and can invade or displace endoluminal mass is highlighted, which allows differentiation the capsule. If its extension is deep, it can invade the from a non tumoral thrombus. Alterations correspond to a renal pyelocaliceal system or the vessels of the renal hilum. cell carcinoma. 40 Rev ChilRadiol 2016; 22(1): 39-46.

Figure 9. Axial and coronal CT images in venous phase, showing a liver mass affecting segments VII and VIII, of heterogenous appearance and that invades the hepatic vein and the IVC, which corresponds to a hepatocellular carcinoma (arrows).

Leiomyosarcoma of the inferior vena cava Leiomyosarcoma is the primary malignant tumor that most often affects the IVC, and it originates from the parietal muscle cells of the vessel. Approximately Figure 7. Phases of CT images without contrast, arterial 74% of leiomyosarcomas of the IVC affect women (15) and portal, where a mass composed of multiple solid between 40 and 60 years of age . This neoplasm nodules at the level of the right adrenal gland, that presents may depend on the lower middle or upper third of the hypervascular behavior and invades the inferior vena cava IVC and have an intra or extraluminal component, (arrow), is observed. featuring in two thirds of cases a predominantly extraluminal growth, hindering the diagnosis of this Adrenal carcinoma entity because it simulates a mass with its isocenter This is a malignant neoplasm with a reported located in the retroperitoneum and which seems to prevalence of 0.5 - 2 cases per 1,000,000 patients. invade the IVC, when in fact it originates therein. 62% of patients have functional tumors that could This aspect is very important, since leiomyosarco- cause Cushing syndrome, virilization or feminization10 mas arising in the IVC have a better prognosis with (Figures 7 and 8). surgery and complete resection of the vein, unlike what might happen with other tumors, in which only Hepatocellular carcinoma radio or chemotherapy could be offered(1). On CT it Hepatocellular carcinoma usually invades the portal appears as a solid mass with calcifications and may venous system, but the invasion of the inferior vena have haemorrhagic or necrotic areas when it beco- cava and the suprahepatics may occur in up to 4% (11) mes bigger (Figure 10). On MR it may have a low of patients . The characteristic finding is the dilation sign in T1-weighted sequences and intermediate or of the veins involved and the presence of intraluminal high sign in T2-weighted sequences, which depend thrombus that enhances after contrast administration. mainly on the size of the necrotic component. To The presence of this type of condition predisposes determine whether the lesion emerges from the IVC, to the development of distant metastases and wor- the most frequent sign is the imperceptibility of the sens the prognosis, decreasing survival to only 1 to venous structure as such, followed by the presence 4 months12 (Figure 9). More so, for some authors, of intraluminal tumor(16). In addition to having its origin the invasion of the IVC is considered an exclusion (13,14) in the IVC, leiomyosarcoma can be of retroperitoneal criterion for hepatocarcinoma surgical resection . origin and secondarily invade the vena cava, as set forth in Figure 11(2). Occasionally, other malignant entities such as pancreatic adenocarcinoma, Wilms tumor and me- tastasis in retroperitoneal lymph nodes may extend to the IVC. For example, in the case of the metastatic lymph nodes, some studies have shown that between 3-11% of those derived from a non-seminoma type testicular carcinoma could invade the IVC(17). Finally, it is important to note certain entities that Figure 8. Macroscopy of the sample that shows pearly can simulate lesions of the IVC and lead to errors, whitish coloured solid masses found in relation to the adrenal especially when associated with neoplasias, causing gland whose histopathology was consistent with a cortical the radiologist to overestimate a false intravascular carcinoma with IVC invasion. affection. 41 Molina M, et al. Rev ChilRadiol 2016; 22(1): 39-46.

Flow artifacts Flow artifacts are the most frequent entities that can simulate an IVC lesion, caused by the blending of the contrasted venous blood from the renal veins when mixed with that which is not contrasted that comes from the lower extremities. It can also be seen in situations where low amounts of contrast are used, in cases of right heart failure and in patients where the acquisition sweep is performed at an early portal phase (Figure 13).

Figure 10. Leiomyosarcoma of the IVC. Axial contrast- enhanced CT image in venous phase. A large solid retroperitoneal predominantly hypodense mass, of hetero- erogeneous enhancement and with calcifications (arrow) is identified. It presents mainly extraluminal growth, with a lesser endoluminal component (curved arrow). Figure 13. Flow Artifact. Axial images with contrast in venous phase, where a hypodense endoluminal filling defect in the IVC (open arrow) is observed, which disappears after the influx from the renal veins on homogenizing the dye (curved arrow).

Venous thrombosis The most common filling defect in the vena cava is thrombosis, which occurs most frequently in users of oral contraceptives, patients with antiphospholipid Figure 11. CT and PET/CT axial slices (hybrid positron emission syndrome, vasculitis and various coagulopathies. The tomography and computed tomography image) and CT coronal use of filters also determines the presence of endo- reconstruction, where a poorly defined solid lesion located in luminal thrombus18. It is important to mention that the retroperitoneal region situated in the interaortocaval space, paraneoplastic hypercoagulable states can influence which contacts and invades the IVC (straight arrow), is shown. The tumoral mass has important tracer uptake including the development of thrombi, which are difficult to the intraluminal component (curved arrow). In the coronal differentiate from tumor thrombus. One way to diffe- reconstruction it is possible to better visualize the invasion of the rentiate them is by identifying the intraluminal mass IVC. Biopsy gives proof of a high-grade retroperitoneal sarcoma. enhancement after contrast administration.

Pseudolipoma Conclusion This is a rare pseudo-lesion in which the periarterial There are multiple entities that can affect the IVC adipose tissue located on the caudate lobe appears and they represent a challenge for the current radio- to extend into the IVC. The best way to determine if logist. This article describes a variety of diseases that it actually is real lesion or not, is by observing the can affect it. In all of them, the images allow adequate coronal or sagittal plane reconstructions (Figure 12). evaluation, thus contributing to decision-making.

Conflict of interests The authors declare no conflict of interest.

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