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|Year : 2011 | Volume
| Issue : 4 | Page : 332-334
Leiomyosarcoma of inferior vena cava
SA Jadhav, VS Atluri, R Prajapati, RR Satoskar
Department of General Surgery, K.E.M Hospital, Mumbai, Maharashtra, India
|Date of Submission||05-May-2011|
|Date of Decision||13-Jun-2011|
|Date of Acceptance||04-Aug-2011|
|Date of Web Publication||22-Nov-2011|
S A Jadhav
Department of General Surgery, K.E.M Hospital, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
Malignancy arising from the blood vessels is a very rare finding in daily clinical practice. In addition, the finding can often be misdiagnosed and ill-treated. These tumors usually go unnoticed unless it has metastasized to distant tissues. Among these rare tumors, leiomyosarcoma is the most common. It mostly arises in the inferior vena cava (IVC). Clinical signs and symptoms are very vague. Usually it is often misdiagnosed as an abscess cavity in the liver or primary hepatic malignancy (when present at level II) or as a thrombus in the IVC. Radiological investigations are the key to proper diagnosis. Depending upon the exact location, further treatment options vary. Generally, it is believed that level II and level III tumors are amenable to surgery followed by chemo or radiotherapy. We present a rare case of leiomyosarcoma of IVC at level II being diagnosed with proper radiological investigations and its management with further stress on offering chemo-radiotherapy after its surgical removal as compared to only surgery performed earlier. This case report will throw some light on the proper management of such rare tumors in terms of their exact diagnosis and treatment in order to prolong patient survival.
Keywords: Inferior vena cava, leiomyosarcoma, chemoradiotherapy
|How to cite this article:|
Jadhav S A, Atluri V S, Prajapati R, Satoskar R R. Leiomyosarcoma of inferior vena cava. J Postgrad Med 2011;57:332-4
| :: Introduction|| |
Vascular tree as such is a very uncommon site for any tumor formation. However, among the blood vessels, inferior vena cava (IVC) has more predominance for soft tissue tumors arising from its media. We report one of such case of IVC soft tissue tumor extending from the confluence of hepatic veins till the confluence of renal veins. Since these tumors are rare findings in daily surgical practice the diagnosis and management needs to be well understood in order to clinically manage the patient, as most of these tumors till date are still misdiagnosed and incorrectly treated or diagnosed correctly but managed only with surgical excision.
| :: Case Report|| |
A 65-year-old female presented in the emergency with on and off chronic pain in right flank since last 1 year. Patient had low-grade fever since last 1 month. She was investigated and her ultrasonography abdomen showed suspected caudate lobe abscess. She was referred to our tertiary center for further management. Her repeat USG abdomen was reported as suspected caudate lobe mass stuck to IVC. In view of above findings, a computerized tomographic (CT) scan of the abdomen was done. CT abdomen was suggestive of mass arising from the retro hepatic part of the IVC [Figure 1]. To support the diagnosis further, an MRI scan of the abdomen was carried out which also showed the mass to be arising from IVC extending till the point of emptying of renal veins [Figure 2]. The hepatic veins and the renal veins were free from the tumor. An interventional radiological procedure was done on the patient and transfemoral biopsy of the IVC mass was taken. The cavogram showed complete lumen occluding growth present in the IVC [Figure 3]. The histopathological report came as soft tissue tumor arising from smooth muscle cells of the IVC. The tumor was a low-grade spindle cell sarcoma. On immunohistochemistry (IHC) the spindle cells expressed desmin, while they were negative for S-100 and SMA was equivocal. In view of mass arising from the IVC and being free from any metastases, resection of the tumor and prosthetic reconstruction was contemplated. But patient and her relatives were not willing for the proposed surgery and thus the patient was discharged without any operative intervention. The patient is on regular follow-up since last 6 months with no evidence of any metastasis.
|Figure 3: Ascending cavogram showing complete luminal obstruction with formation of collaterals|
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| :: Discussion|| |
Leiomyosarcoma of the vascular tree are rare tumors. The incidence of leiomyosarcomas has nowhere been described in the literature but has been only referred to be a rare entity arising in the vessels. They originate from the smooth muscle cells of the media and predominantly arise within the IVC.  These tumors usually grow extraluminally with very few ones having predominant intra-luminal growth.  The origin of the tumor is described in relation to the hepatic and renal veins. For this purpose, the IVC is divided into three levels: Level 1 extends from the entry of the hepatic veins up to the right atrium; level 2 comprises the area between the confluences of the renal and hepatic veins whereas level 3 includes the area below the renal veins.  The IVC can also be divided as three segments where level I is segment 3, level II is the same and level III is segment 1. Actually, level 2 or segment 2 of the IVC is most frequently affected. ,,
These patients usually present with very vague symptoms in the form of pain in right flank, upper abdominal pain, pedal edema, etc. and the presentation also is very late when the disease has metastasized to other adjacent organs. The patient may also present with Budd Chiari syndrome when the tumor is involving the supra hepatic IVC.  Our patient did not have venous stasis or pedal edema due to formation of collaterals around the IVC. The diagnosis can only be confirmed on the basis of investigations. Out of the array of radiological investigations available, ultrasonography (US) of the abdomen is the basic study that is usually indicative of the mass to be present in the retroperitoneum. This further can be confirmed by CT and an MRI scan of the abdomen. Heterogeneous contrast enhancement is a characteristic feature in CT and is important in differentiating the tumor mass from a blood thrombus. Tumor vascularity can be similarly demonstrated with Doppler US and contrast-enhanced MRI. Preoperative histopathology can be done by intra-luminal biopsy either through the transfemoral or the transjugular route.
The differential diagnosis that can be considered includes tumor arising from segment I of the liver, IVC thrombus or very rarely a caudate lobe abscess.
Due to poor long-term prognosis of the tumor and the involved high surgical risk because of the location of these tumors, surgical excision has been thought to have a limited role in the management. However, with the improvements in the surgical techniques and improved perioperative care, today surgical treatment is the mode of therapy to prolong survival if not being complete curative.  The patients are then given chemotherapy (CT) (adjuvant chemotherapy) in the form of doxorubicin-based regimens (doxorubicin/ifosfamide) or gemcitabine-based regimens (gemcitabine/Taxotere), radiotherapy (RT) (adjuvant radiotherapy -mean dose 50 Gy) or combined CRT.
The surgical management of partial resections of the IVC is a matter of current debate and includes ligation, primary repair/cavoplasty, or replacement with a graft. Reconstruction of the IVC is not always required, because gradual occlusion of the IVC allows the development of venous collaterals.  Several reports advice construction of an arterio-venous-fistula (AV fistula) for maintenance of IVC patency after graft replacement.  However, several authors believe that AV fistulas are not required if the suprarenal IVC is reconstructed because of the high-volume blood flow at this level. ,
Hence the stress should be on the finding that the outcome of patients with leiomyosarcoma of IVC can be improved by early diagnosis and administering chemo or radiotherapy  in addition to surgery. The prognosis depends on few variables like the grade of the tumor (low-grade better prognosis), segment of the IVC involved (level 2 or segment 2 - better prognosis), size of the tumor (size <9 cm better prognosis) as well as postoperative adjuvant therapy (post-operative CRT better prognosis). However, as stated above, overall leiomyosarcoma runs a poor prognosis, over half of the patients with surgical excision develop recurrence. The 5-year survival rate for these patients ranges between 31 and 62%. 
So to conclude we here suggest that surgical treatment along with CT, RT, or CRT, is the treatment of choice in patients with IVC leiomyosarcoma and is the only hope of prolonging survival.
| :: Acknowledgment|| |
We would like to thank our Director, Dr. Sanjay N. Oak for allowing us to publish the hospital data.
Also, we would like to thank Dr. Krantikumar Rathod, Department of Vascular and Interventional Radiology for helping us in performing vascular intervention on the patient and to take intra-luminal biopsy.
| :: References|| |
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[Figure 1], [Figure 2], [Figure 3]
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|[Pubmed] | [DOI]|