Background
Leiomyosarcoma of the inferior vena cava (IVC) is an exceedingly rare smooth muscle
sarcoma. Approximately 300 cases have been described in the literature, and further
research is needed to understand the disease and guide its management. Surgery remains
the only potential curative measure.
Methods
A retrospective chart review of patients who underwent surgical resection of IVC leiomyosarcoma
at our institution over the past 3 years was performed. The patients were identified
using a prospectively maintained database.
Results
Three patients with leiomyosarcoma of the infrahepatic IVC underwent radical resection
carried out by a team of surgical oncologists and vascular surgeons. There were 2
males (66.7%) and 1 female (33.3%). Mean age at diagnosis was 60.3 years (range 43–78).
Mean tumor size was 12.2 cm (range 5.6–22). The mean operative time was 320 min (range
180–421), mean estimated blood loss was 1,300 mL (100–2,000) mL, and average length
of stay 8.67 days (6–12). All patients achieved grossly negative margins (R1 or R0
resections) and are alive with a mean overall survival of 21 months (range 12–30).
Patient 1 was a 60-year-old man who presented with metachronous skin leiomyosarcomas
at 2 different sites. He underwent PET/CT scan that revealed an IVC mass. Resection
of the middle segment of the IVC and right kidney was performed with reconstruction
with polytetrafluorethylene (PTFE) graft. Patient 2 was a 78-year-old man with an
incidentally found a 9-cm IVC tumor. Resection of the tumor was performed, and no
reconstruction was needed since the tumor had a completely extraluminal growth pattern.
Patient 3 was a 43-year-old woman who presented with abdominal pain. Her work-up showed
a 15-cm IVC mass. She underwent resection of the middle segment of the IVC, right
nephrectomy, and cholecystectomy with reconstruction of the IVC with PTFE graft.
Conclusions
Surgical resection is the mainstay of treatment in patients with leiomyosarcoma of
the IVC. A collaborative approach involving surgical oncologists and vascular surgeons
ensures adequate resection with functional reconstruction to achieve the best patient
outcomes.
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References
- Primary venous leiomyosarcoma: a rare but lethal disease.J Vasc Surg. 1992; 15: 595-603
- International registry of inferior vena cava leiomyosarcoma: analysis of a world series on 218 patients.Anticancer Res. 1996; 16: 3201-3205
- Ein Fall von Sarkom der Vena cava inferior.Archiv für pathologische Anatomie und Physiologie und für klinische Medicin. 1871; 53: 378-383
- Sarkom der Vena cava inferior.Deutsche Zeitschrift für Chirurgie. 1928; 213: 135-140
- Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system.Int J Cancer. 1984; 33: 37-42
- AJCC Cancer Staging Manual. 7th ed. Springer-Verlag, New York2010
- Leiomyosarcoma of the inferior vena cava.Diagn Interv Radiol. 2007; 13: 140-143
- Surgical treatment and outcomes of patients with primary inferior vena cava leiomyosarcoma.J Am Coll Surg. 2003; 197: 575-579
- Great saphenous vein and leiomyosarcoma.EJVES Extra. 2013; 26: e15-e16
- Non-vena cava venous leiomyosarcomas: a review of the literature.Ann Surg Oncol. 2012; 19: 3368-3374
- From the archives of the AFIP. Leiomyosarcoma of the retroperitoneum and inferior vena cava: radiologic-pathologic correlation.Radiographics. 1992; 12: 1203-1220
- Leiomyosarcoma of the inferior vena cava: a clinicopathologic review and report of three cases.J Surg Oncol. 1997; 65: 205-217
- Leiomyosarcoma of the inferior vena cava: analysis and search of world literature on 141 patients and report of three new cases.J Vasc Surg. 1991; 14: 688-699
- Prognostic factors associated with long-term survival for retroperitoneal sarcoma: implications for management.J Clin Oncol. 1997; 15: 2832-2839
- Outcome and prognosis in retroperitoneal soft tissue sarcoma.Int J Radiat Oncol Biol Phys. 1994; 29: 1005-1010
- Initial results of a trial of preoperative external-beam radiation therapy and postoperative brachytherapy for retroperitoneal sarcoma.Ann Surg Oncol. 2002; 9: 346-354
- Outcomes after resection of leiomyosarcomas of the inferior vena cava: a pooled data analysis of 377 cases.Surg Oncol. 2015; 24: 21-27
- Synchronous small bowel and atypical primary leiomyosarcoma of inferior vena cava in a patient with RB1 mutation.Abdom Imaging. 2014; 39: 33-39
- Resection of malignant tumors invading the vena cava: perioperative complications and long-term follow-up.J Vasc Surg. 2007; 46: 533-540
- Prosthetic replacement of the infrahepatic inferior vena cava for leiomyosarcoma.Arch Surg. 2006; 141 (discussion 924): 919-924
- Pancreatic and multiorgan resection with inferior vena cava reconstruction for retroperitoneal leiomyosarcoma.World J Surg Oncol. 2009; 7: 3
- Unilateral nephrectomy and cisplatin as risk factors of ifosfamide-induced nephrotoxicity: analysis of 120 patients.J Clin Oncol. 1994; 12: 159-165
- Long-term nephrotoxicity in adult survivors of childhood cancer.Clin J Am Soc Nephrol. 2013; 8: 922-929
- Leiomyosarcoma of the inferior vena cava: experience in 22 cases.Ann Surg. 2006; 244: 289-295
- Leiomyosarcoma of the inferior vena cava: case report and treatment of recurrence with repeat surgery.Ann Vasc Surg. 2010; 24 (417.e5–9)
- Successful surgical management of a recurrent leiomyosarcoma of the inferior vena cava.Vasa. 2011; 40: 69-72
- Pulmonary metastases from soft tissue sarcoma: analysis of patterns of diseases and postmetastasis survival.Ann Surg. 1999; 229 (discussion 610–2): 602-610
- Leiomyosarcoma of the retrohepatic vena cava treated by excision and reconstruction with an aortic homograft: a case report and review of literature.Surg Innov. 2007; 14: 287-291
- Primary leiomyosarcoma of the inferior vena cava: a 2-institution analysis of outcomes.Surgery. 2012; 151: 261-267
- Leiomyosarcoma of the inferior vena cava: prognosis and comparison with leiomyosarcoma of other anatomic sites.Cancer. 1999; 85: 1077-1083
- Personalizing the approach to retroperitoneal soft tissue sarcoma: histology-specific patterns of failure and postrelapse outcome after primary extended resection.Ann Surg Oncol. 2015; 22: 1447-1454
- Surgery in retroperitoneal soft tissue sarcoma: a call for a consensus between Europe and North America.Ann Surg Oncol. 2011; 18: 2107-2110
Article info
Publication history
Published online: January 20, 2016
Accepted:
October 15,
2015
Received:
August 5,
2015
Identification
Copyright
© 2016 Elsevier Inc. All rights reserved.
