Leiomyosarcoma of inferior vena cava: A case series of four cases

Abstract

Vascular leiomyosarcomas are rare tumors, arising most frequently from inferior vena cava (IVC). They are mostly seen in sixth decade, with a female predominance. Their diagnosis is often challenging, as patients may present with non-specific complaints such as dyspnea, malaise, weight loss, abdominal pain, or back pain, preceding the diagnosis by several years. Leiomyosarcoma of the IVC most frequently occurs in the middle segment. The final diagnosis can be made by an ultrasound or computed tomography guided biopsy. Because of limited experience with this disease, optimal management of IVC leiomyosarcoma is unknown. Curative surgical resection remains the current treatment of choice for primary leiomyosarcoma of IVC. Neoadjuvant therapy may be given to downsize the tumor and increase resectability rates. Nonetheless, when complete resection is not possible, debulking combined with radiation therapy still provides good palliation. We, hereby, report four cases of this rare entity with emphasis on management.

Banked Vena Caval Homograft Replacement of the Inferior Vena Cava for Primary Leiomyocsarcoma

“…Therefore, nephrectomy, kidney auto-transplantation, or re-attachment of the tumor-free renal vein stump to the re- been established yet because of the insufficient respondence [1,5]. Therefore, there have been several previous reports that the adjuvant chemo-radiotherapy might not have any benefits in patients with complete tumor resection [2,4,6].…”

“…The clinical diagnosis is usually delayed because the malignancy is often asympto- Survival of the patients with IVC leiomyosarcoma depends on curative tumor resection, its anatomic site, histological grade, invasion of the adjacent structures, and histologic subtype [3]. It has been observed that en bloc surgical resection with negative margins of more than 1 cm is the only treatment that improves survival [3,5]. However, surgical procedures can be complex due to the tumor involvement in the renal vein confluence and at the retrohepatic location.…”

Leiomyosarcoma of inferior vena cava complicated by Budd-Chiari syndrome and disseminated intravascular coagulation — case report

“…Histopathology of leiomyosarcoma reveals spindle tumor cells, which are positive for markers of smooth muscle activity including vimentin, muscle actin, alpha-smooth muscle actin and desmin [5]. Material could be obtained by USG or CT guided biopsy or intraoperatively [16]. However, because of nonspecific symptoms, delay in diagnosis is common -33% leiomoysarcomas of IVC is diagnosed at autopsy [17].…”

Primary leiomyosarcoma in the inferior vena cava- a case report

“…Tumor in the upper segment give rise to varying degree of Budd Chiari syndrome due to hepatic vein thrombosis. 3,5 With the development of imaging modalities like USG, CT and MRI, preoperative diagnosis of Leiomyosarcoma is possible. CT scan clearly delineates the intra luminal vascular tumor, which is usually large, lobulated and sometimes heterogeneous owing to haemorrhage and necrosis.…”

“…The final diagnosis can be made by ultrasound or CT guided biopsy. 5,6 Histopathological examination reveals spindle cells arranged in fascicles with variable degree of nuclear atypia and mitotic activity.…”