Fact-sheet: Leiomyosarcoma of inferior veina cava
Updated on 09/17/2021 at 12:16 PM
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Definition
Primary venous tumors are Rare
Most often malignant
Leiomyosarcomas: most common histologic subtype
Benign tumors: leiomyomas, are exceedingly rare
Two-thirds of tumors involve the IVC
Hemangioendothelioma: 2nd most common venous tumor
Treatment: surgical with the widest possible excision +/- postoperative radiotherapy
Poor prognosis with high metastatic potential
Median survival 35 months
Leiomyosarcoma
Mesenchymal tumor
Arises from smooth muscle fibers
95% of primary IVC tumors
Epidemiology: female – middle-aged
Slow local progression: ¾ of cases show extravascular extension, ¼ intraluminal
Late hematogenous systemic dissemination: liver, lungs
Location of leiomyosarcomas:
Proximal IVC 20%
Middle IVC 43%: better prognosis given its accessibility to surgery
Distal IVC 37%
Clinical features
Clinical presentation: nonspecific
Abdominal pain
Palpable abdominal mass
Lower limb edema
Budd-Chiari syndrome
General malaise…