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PRIMARY LEIOMYOSARCOMA
NITISH JHAWAR*, SURYAKANT CHOUDHARY*, PRAKASH PATIL**, PARESH PAI***, YG AGNIHOTRI***
*Senior Resident, Department of Oncosurgery; **Associate Cancer Surgeon; ***Associate Vascular Surgeon, Bombay Hospital Institute of Medical Sciences, Mumbai, India.
We discuss here the surgical management of primary tumour of Inferior vena cava. There was an extensive lieomyosarcoma extending from diaphragmatic level to femoral vein. Cardiac bypass provided us with the ease of careful dissection and wall preservation of inferior vena cava and iliac veins, thus abolishing the need of IVC graft.
CASE REPORT
37 year old male patient from Mumbai, noticed a mass in the left groin of 1 1/2 year duration. Six months later he noticed venous congestion and oedema of left lower limb, later on it appeared in the right lower limb too.
On clinical examination he had a soft tissue mass of 15 x 5 x 5 cm size in left groin region, extending into left iliac fossa. There was clinical evidence of inferior vena caval obstruction. CT scan of the abdomen and pelvis revealed soft tissue mass arising from left femoral vein (Fig. 1), extending to iliac vein and IVC without any plane of cleavage between tumour thrombus and IVC wall. Distal IVC (Fig. 2) and right iliac vein showed tumour thrombus (Fig. 3). Renal veins were free from tumour extension. FNAC of the groin mass revealed spindle cell sarcoma; CT scan of the chest showed three tiny pulmonary metastases in right lower lobe of the lung and two metastases in the left lower lobe of the lung.
Resection of the IVC tumour and reconstruction with venous graft and pulmonary metastasectomy was planned. Abdomen was explored through midline incision and IVC was exposed. Tumour thrombus in IVC was extending up to diaphragm level. Transverse bilateral thoracotomy through lower sternum was carried out, pericardium was opened and IVC was clamped within pericardium. Balloon extraction of the thrombus was attempted through a small opening in the right iliac vein, but thrombus was firmly adherent to wall, so IVC was split opened longitudinally. Tumour thrombus in IVC and iliac vessels was extracted. IVC and common iliacs were sutured with 6-0 prolene. Sarcoma was inseparable from the vessel wall distal to the left common iliac bifurcation till femoral vein in groin. Left common iliac vessel was ligated at bifurcation. Incision was extended across the inguinal ligament into the left groin. Left femoral artery and nerve were dissected off the tumour. Femoral vein was ligated proximal to tumour and the tumour from the femoral and iliac vessels was resected en bloc (Fig. 4). With finding of adequate venous collaterals, replacement of left iliac vein and femoral vein with graft was not carried out. Bilateral pulmonary metastasectomy was done.
Post Operative period was uneventful.
Histopathology report was primary leiomyosarcoma of IVC.
On follow up at 14 months, disease was controlled locoregionally.
IVC and right common iliac vein.
DISCUSSION
Medline search result of last 30 years was done. There are plenty of case reports of IVC tumour thrombus secondary to renal cell carcinoma,[1] Wilm's tumour,[2] adrenal neuroblastoma,[3] benign angiomyolipoma,[4] retroperitoneal tumours,[5] hepatocellular carcinoma.[6]
There were only 11 cases of the primary IVC tumour of which 10 were leiomyosarcomas and one was rhabdomyosarcoma. Complete surgical excision with IVC replacement was done with bilateral auto renal transplant in 4 cases. Patients were disease free at the end of 26 months.[6]
Of the 4 cases from China one patient had cardiac metastases which was resected and one patient had uterine and IVC lieomyosarcoma which presented with Budd-Chiari syndrome.[7]
CONCLUSION
Many reports recommend en bloc excision of IVC along with tumour and reconstruction with a graft. This saves operation time and blood loss. But we found that when surgery is done under bypass, then IVC wall preservation is possible without excessive blood loss. At the same time grafting can be avoided and so are the want of long term anticoagulant therapy and fear of graft complications.
REFERENCES
1.Nesbi HJC, Soltero ER, Dinneyop, et al. Surgical management of the renal cell carcinoma with IVC tumour thrombus. Annels of Thoracic Surgery 1997; 63 (6) : 1592-1600.
2.Dale PS, Well MW, Wilkinson AH Jr. Resection of IVC for recurrent Wilm's tumour. Journal of Paediatric Surgery 1995; 30 (1) : 121-22.
3.Custadio CM, Scmalka RC, Balci MC, Mitchell KM, Freeman Jr. Adrenal neuroblastoma in an adult with tumour thrombus in the inferior vena cava. Journal of Magnetic Resonance Therapy 1999Atml; 9 (4) : 621-3.
4.Bernstein MR, Malcowizz SB, Sergelman ES, et al. Diagnostic angiomyolipoma with inferior vena cava tumour thrombus. Urology 1997; 50 (6) : 1975-7.
5.Wray RC Jr, Dawkins H. Primary smooth muscle tumour of Inferir vena cava. Annals of Surgery 1971; 17-4 (6) : 1009-18.
6.AV Nanomi T, Nakao A, Harada A, Kanekot, Keurokava T, Takagi H. Hepatic resection for hepatocellular carcinoma with a tumour thrombus extending to IVC. 1997; 44 (15) : 798-802.
7.Kramybill VG, Callary MP, Haiken JP, Flye MW. Radical resection of tumour of the inferior vena cava with vascular reconstruction and Kidney auto transplantation. Surgery 1997; 121 (6) : 31-6.
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