Leiomyosarcoma of the Inferior Vena Cava: Report of a Case

Abstract

We report a case of leiomyosarcoma of the inferior vena cava, which was successfully treated by surgical en bloc resection and reconstruction of the inferior vena cava, followed by adjuvant radiation therapy. A 39-year-old man presented with nausea, vomiting, epigastric pain, and weight loss. Radiologic examinations showed a mass originating from the inferior vena cava and surgical resection was performed. Histopathological examination of the specimen revealed a moderately differentiated (grade II) leiomyosarcoma arising from the inferior vena cava. We believe that radical resection with clear surgical margins followed by adjuvant radiation therapy is a good curative strategy for achieving any chance of long-term survival.

Prosthetic Replacement of the Infrahepatic Inferior Vena Cava for Leiomyosarcoma

“…12 Histopathological grading of the tumor has not been correlated with survival. 8,21,22 The role of neoadjuvant or adjuvant therapy is still unclear, 4 and statistical evidence of any benefit from radiation therapy and chemotherapy in reducing recurrences is still lacking. 6,23,24 Several malignancies of different origin may extend to the infrahepatic IVC, including lymphoma, renal cell carcinoma, Wilms tumor, adrenal tumors, hepatic carcinoma, retroperitoneal metastatic lymph nodes, and different retroperitoneal sarcomas.…”

“…1,6,7 Standard surgical resection of leiomyosarcoma en bloc with the affected portion of the IVC should attain the goals of complete excision of the tumor, prevention of recurrence, and preservation of venous return. 6,8 Preservation of venous return is increasingly being obtained through expanded polytetrafluoroethylene (PTFE) graft replacement of the IVC. 9,10 Tumor involvement of the IVC can be classified as infrahepatic, ie, below the caudate lobe, or retrohepatic, ie, above the caudate lobe.…”

Leiomyosarcoma of the retrohepatic vena cava: Report of a case treated with resection and reconstruction with polytetrafluoroethylene vascular graft

“…Mingoli et al (7) reported an international registry of 218 cases in 1996, and Laskin et al (3) reported the largest single center experience of 40 cases in 2010. Several centers have reported their experience, commonly in case reports, with a smaller number of patients (2,5,8,9).…”

“…There is limited experience with neoadjuvant and adjuvant therapy for IVC leiomyosarcoma. Some studies have reported that adjuvant radiation therapy may be of benefit in local tumor control after incomplete resection or in high grade tumors (1,(7)(8)(9). In the few studies of the management of recurrent IVC leiomyosarcoma, surgery appears to be the only option if the risk of morbidity is low for local recurrence and isolated metastasis.…”

“…In the few studies of the management of recurrent IVC leiomyosarcoma, surgery appears to be the only option if the risk of morbidity is low for local recurrence and isolated metastasis. Adjuvant chemotherapy and radiation threapy has not been proven but may be an option instead of surgery or following surgery (8,9). In this case, we performed a total resection of the retrohepatic vena cava (Level 2 IVC) with negative surgical margins and reconstructed the vena cava with a polytetrafluoroethylene vascular graft (Figure 2b).…”