Undifferentiated Intimal Sarcoma of the Inferior Vena Cava with Extension to the Right Atrium and Renal Vasculature

Abstract

Primary sarcomas of the great vessels (aorta, pulmonary artery, and inferior vena cava (IVC)) are exceedingly rare. We report a rare case of an undifferentiated intimal sarcoma of the IVC with extension to the right atrium, adrenal, and renal veins. The patient underwent extensive resection, reconstruction of the IVC, and subsequent adjuvant chemotherapy. Patient has tolerated chemotherapy and, at 17 months after resection, the patient remains free of tumor recurrence. Undifferentiated intimal sarcomas remain a rare entity with only five cases of venous undifferentiated intimal sarcomas reported in the literature, two of which occurred in the IVC. Intimal sarcomas tend to carry a poor prognosis with the limited literature available on treatment approaches. Our objective is to highlight this rare entity and possible treatment approach which we utilized. Primary sarcomas of IVC need to be included as part of a complete differential diagnosis in patients with atrial masses or recurrent pulmonary emboli.

Primary aortic intimal sarcoma masquerading as intramural hematoma

“…Fourth, the aortogram demonstrated mild stenosis in the thoracic aorta lesion without severe atherosclerotic changes in the aortic wall and obvious intimal flap. Furthermore, the following features with regard to a leak and periaortic hematoma after stent graft implantation differed from those on previous reports [ 12 , 13 ]: first, there was no open channel inside the stent graft and no obvious backflow in the “false lumen.” Second, the “false lumen” gradually became enlarged, increasing the tendency of mediastinal invasion. Third, MRI showed a heterogeneous mass with a false lumen and a new lesion in the azygos vein.…”

Intimal sarcoma of the inferior vena cava with extension to the right atrium, bilateral renal vasculature, and bilateral venae iliaca communis, accompanied by severe thrombocytopenia

“…The primary sarcomas of the major blood vessels can be divided into three categories based on their site of origin: (a) the pulmonary artery (PA), (b) the aorta and its branches, and (c) the large veins (extremely rare group) . ISs, nonmyogenic sarcomas derived from the intima, are typically seen in the arterial system, particularly the PA, and are exceedingly rare in the IVC . The signs and symptoms of these tumors result from venous system obstruction; hence, symptoms depend on the tumor sites, sizes, invasiveness, friability, and the rate of growth.…”

“…Intimal sarcoma (IS), a nonmyogenic sarcoma, is a malignant mesenchymal tumor arising in the large arterial blood vessels of the systemic and pulmonary circulation systems, commonly in the PA but rarely in the inferior vena cava (IVC). There have been less than 10 cases of venous undifferentiated IS reported in the literature, three of which occurred in the IVC …”

A Case of Undifferentiated Sarcoma in the Superior Vena Cava and Bilateral Cervical Veins

“…Over 20 cases of LMS were also reported in the pulmonary vein and over a dozen cases in the SVC and/or large cervical veins [4,20,21]. Meanwhile, sarcomas with predominantly intraluminal growth in the venous system are extremely rare, as only a few sporadic cases of the sarcomas have been reported in the English literature in systemic veins [6,7]. Two cases of IS [5,22], 5 cases of AS [7,23–26], and 2 cases of SS [7,27] have been reported in SVC and/or large cervical veins.…”