The role of imaging in the management of IVCLMS concerns diagnosis, preoperative planning, and detection of recurrences and metastases. IVCLMS is often an unexpected finding on imaging due to the tumor rarity and the absence of symptoms and signs of IVC compression. However, at presentation nearly half of the patients shows distant metastases to the liver and lungs.

A recent study by Ganeshalingam et al. suggests that contrast-enhanced CT scan (CECTS) is a sensitive tool in the diagnosis and follow-up of IVCLMS, delineating the intravascular component of the tumor, which is usually large, irregular, lobulate, and heterogeneous owing to hemorrhage and necrosis with peripheral enhancement [20].

Conversely, MRI accurately depicts the extent of IVCLMS and is more precise than CECTS in the determination of the tumor origin due to its superior soft tissue resolution. The signal characteristics on MRI depend on the degree of cystic necrosis within the tumor. Typically, T1-weighted images show a homogeneous low-signal-intensity mass (73 %) corresponding to the regions of liquefaction, and all T2-weighted images demonstrate areas of high-signal intensity due to the cystic components of these lesions.

The authors conclude that the optimum imaging technique for initial assessment of IVCLMS is a CECTS with images obtained during the portal venous phase of contrast medium administration, while MRI is valuable in the assessment of tumors in patients suitable for surgery [20].

Differential diagnosis includes angiosarcoma, renal cell carcinoma with extension in to the IVC, primary lymphoma, liposarcoma, and leiomyomatosis. Imaging differentiation of an IVCLMS from primary retroperitoneal sarcoma or similar-appearing mass is a difficult, diagnostic task. In a recent report, Webb et al. [21] found that the most useful sign is the identification of an imperceptible caval lumen. This was seen in 75 % of IVCLMS but not in other lesions (p < 0.01). This sign had both high positive (100 %) and negative predictive values (92 %). Like previous authors, Webb found that the IVC compression by an extrinsic mass, with a crescentic configuration (negative embedded IVC sign), suggests a not caval origin of the lesion. This sign was never seen in IVCLMS cases, but was present in 79 % of other lesions (p = 0.01, PPV = 92 %) [21].

Cardiac ultrasound is an important diagnostic tool for IVCLMC localized in the upper segment, to detect eventual tumor extension to the atrium with or without thrombosis.

When metastatic disease is suspected, PET scan is the elective exam to accurately identify secondary systemic extensions of the leiomyosarcoma. In this case, percutaneous preoperative biopsy is mandatory in order to get a histopathological determination, required for the choice of the chemotherapy protocol.

The update of the International Registry of IVCLMS documented that a CT scan was performed in 65 % of patients, as the main diagnostic investigation, and a preoperative biopsy was performed on 63 % of cases (Table 3.1). The comparison between the 1996 registry and the recent update demonstrated that in recent times percutaneous biopsy is often and significantly more commonly performed in order to better establish a therapeutic algorithm and eventually indicate a neoadjuvant/palliative treatment.

Since the lack of efficient complementary treatments, complete R0 surgical resection, including IVC (with or without vascular reconstruction) and surrounding organs, is the mainstay of treatment [6–9, 17, 18].

Involvement of major blood vessels in the tumor growth had long been considered a limiting factor for curative surgery, due to high surgical risks and poor long-term prognosis. Concerns on IVC resection focused on early major morbidities such as cardiopulmonary events, hepatic and/or renal failure, lower limb edema, graft occlusion, and/or infection [5, 22]. However, technical advances have allowed wider surgical extension beyond major vascular resection, with relatively low rate of postoperative complications [23–26].

The extent of IVC resection is related to the site of the tumor and should be properly planned before surgery on the basis of preoperative imaging (CT and MRI).

There is no consensus on the optimal management of the IVC at the time of surgery. Options after resection include IVC ligation, primary or patch repair, or replacement with graft. Different materials for reconstruction are available, including autologous materials such as saphenous vein, allograft such as aortic homograft, xenograft such as bovine pericardium, and synthetic materials as Dacron and PTFE.

Three major factors influence the need and the type of vascular replacement: (1) the site of the lesion and the involvement of renal veins, (2) the extent of IVC resection (partial or circumferential), and (3) the presence of well-established collateral venous system [26, 27].