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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 26  |  Issue : 2  |  Page : 219-221

Leiomyosarcoma of inferior vena cava


1 Department of General Surgery, Kerala Institute of Medical Sciences, Thiruvananthapuram, Kerala, India
2 Department of Surgical Oncology, Kerala Institute of Medical Sciences, Thiruvananthapuram, Kerala, India
3 Department of Cardiovascular and Thoracic Surgery, Kerala Institute of Medical Sciences, Thiruvananthapuram, Kerala, India

Date of Submission03-Aug-2020
Date of Decision13-Aug-2020
Date of Acceptance14-Oct-2020
Date of Web Publication07-Nov-2020

Correspondence Address:
Dr. Mohamad Safwan
ARRA, 13, Avittom Road, Kmarapuram, Thiruvananthapuram - 695 011, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ksj.ksj_15_20

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  Abstract 


A 57-year-old female presented with intermittent abdominal pain and bilateral pedal oedema. She was diagnosed as leiomyosarcoma from inferior vena cava (IVC). Imaging suggested encasement and occlusion of infra-renal IVC. She had received palliative chemotherapy from elsewhere, with no regression. We excised the tumour. At 14 months after the surgery, she is disease-free. Surgery is the mainstay in the treatment of retroperitoneal sarcomas. Even locally advanced retroperitoneal sarcomas may be successfully managed with complete surgical resection. Reconstruction of resected IVC segment may be avoided in selected cases.

Keywords: Inferior vena cava tumour, leiomyosarcoma, retroperitoneal mass, vena cava resection


How to cite this article:
Safwan M, Babu S, Palangadan S, Thivya A. Leiomyosarcoma of inferior vena cava. Kerala Surg J 2020;26:219-21

How to cite this URL:
Safwan M, Babu S, Palangadan S, Thivya A. Leiomyosarcoma of inferior vena cava. Kerala Surg J [serial online] 2020 [cited 2020 Dec 5];26:219-21. Available from: http://www.keralasurgj.com/text.asp?2020/26/2/219/300224




  Introduction Top


Retroperitoneal sarcomas are rare tumours, comprising of <15% of all soft-tissue sarcomas. Eighty per cent of retroperitoneal soft-tissue swellings are malignant. Liposarcomas and leiomyosarcomas (LMS) are the most common retroperitoneal sarcomas. Of these, inferior vena cava (IVC) is a rare site for retroperitoneal LMS. Amongst retroperitoneal vascular LMS, IVC is the most common site. Furthermore, LMS is the most common primary malignancy of IVC. We present a case of large and locally advanced LMS of the infra-renal IVC, to emphasise that complete resection is associated with good functional and tumour-related short-term outcomes.


  Case Report Top


A 57-year-old Somalian female presented with lower abdominal pain and bilateral pedal oedema. She had a soft-tissue mass infiltrating infra-renal IVC. Core cut biopsy had identified it as LMS. She had received 3 cycles of epirubicin + docetaxel chemotherapy. The symptoms persisted, and so she reported to our centre. On examination, she had a 10 cm × 8 cm, firm mass with well-defined borders and restricted mobility, occupying the right lumbar, umbilical and right iliac fossa. There was no ascites or hepatosplenomegaly. Pelvic examination was normal. Bilateral pitting pedal oedema and skin changes of chronic venous insufficiency were noted. Biopsy confirmed LMS. Immunohistochemistry demonstrated smooth muscle actin, desmin, myogenin and Ki 67 of 75%. Magnetic resonance imaging (MRI) scan demonstrated a large, well defined, lobulated mass in the right lumbar and iliac fossa regions [Figure 1], [Figure 2], [Figure 3]. Distension of IVC lumen and total luminal occlusion was noted [Figure 4]. The iliac veins, the renal veins and the suprarenal IVC segment were patent. There was no infiltration of surrounding structures, indicating that this might be a primary malignancy of IVC. Numerous collaterals were seen draining into the vertebral plexus and left iliac veins. Positron emission tomography (PET) computed tomography (CT) scan showed increased fluorodeoxyglucose uptake (standardized uptake value max: 14.33) in a heterogeneously enhancing retroperitoneal soft-tissue lesion with cystic areas in the right lumbar region measuring 8.2 cm × 7.4 cm × 6.3 cm. There was no evidence of metabolically active disease elsewhere in the body.
Figure 1: Magnetic resonance imaging scan showing the lesion at the right side of the vertebral body at the level of the kidneys

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Figure 2: Magnetic resonance imaging scan showing the lesion at the right side of the vertebral body, at the lower part

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Figure 3: Magnetic resonance imaging scan, sagittal view showing the lesion becoming larger, at the lower part

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Figure 4: Magnetic resonance imaging scan showing the involvement of the inferior vena cava

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A midline laparotomy was performed. A firm, smooth surfaced 15 cm × 10 cm mass was found below the third part of the duodenum. The tumour was excised along with the involved segment of IVC. The kidney, renal vessels, ureters and numerous collateral veins in this region were safeguarded. The mass appeared to arise from the IVC. It appeared encapsulated and was dissected from nearby structures. We cross clamped the IVC below the renal veins. Resection of the tumour [Figure 5] en bloc with the IVC below the renal veins, up to the iliac confluence, was completed. There were no major haemodynamic disturbances ensuing from the cross clamping of the infra-renal IVC. Hence, IVC reconstruction was not done. Post-operative renal derangement settled by 3rd day. Histopathologic evaluation [Figure 6] and [Figure 7] confirmed the diagnosis. She completed three more cycles of adjuvant chemotherapy thereafter.
Figure 5: Resected specimen showing en bloc removal of the entire of the entire mass (roughly 12 cm × 8 cm) with surrounding capsule. At the upper end of the specimen, part of the tumours is seen bulging out through the lumen of the resected inferior vena cava

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Figure 6: Histopathological examination low power view showing the spindle cells indicating leiomyosarcomas

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Figure 7: Histopathological examination high power view showing the malignant cells clearly

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  Discussion Top


LMSs of the IVC are extremely rare, constituting 0.5% of sarcomas, and 1 in 100,000 of all adult cancers.[1],[2] They are mostly seen in the sixth decade with a female predominance.[3] They are slow growing, and associated with poor prognosis, and they tend to metastasis to lungs, liver, and bone.[1] They originate from the smooth muscle cells of tunica media. Initially, they are seen as mural growths. They gradually expand to push or infiltrate surrounding structures or form a thrombus within the lumen of the vessel.[4] IVC. LMS was first described in 1871[5] and the first resection was done in 1928.[6] Results of surgery have improved considerably. Mortality of LMS after resection is 1.9% and long-term morbidity is 24.7%. However, the 5-year disease-free survival is dismal and is 6%.[7] Recurrence rate is around 57%. Imaging with Doppler ultrasound, contrast-enhanced CT, PET-CT and MRI is very useful to determine the details of the tumour and metastasis.[8] Cavography evaluates IVC status.[3] Multidisciplinary collaboration is critical for good clinical outcomes.

In our case, the patient's IVC was totally occluded by the tumour, and she had abundant collaterals connecting the infra-renal veins to the right atrium. Hence, we decided not to reconstruct the resected IVC segment. Apart from a transient derangement of renal function, and worsening of pedal oedema, she did not have any significant ill effects of IVC ligation. The patient went back to her country for further treatment and follow-up. Fourteen months after resection, she reported to us in a telephonic interview and was doing fine except for mild oedema of her legs.


  Conclusion Top


Even in cases of locally advanced LMS, surgical resection is possible, with a very good short-term outcome. A meticulous preoperative planning by an experienced team and experience of the surgeon in handling such cases is a prerequisite in its management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hollenbeck ST, Grobmyer SR, Kent KC, Brennan MF. Surgical treatment and outcomes of patients with primary inferior vena cava leiomyosarcoma. J Am Coll Surg 2003;197:575-9.  Back to cited text no. 1
    
2.
Mingoli A, Cavallaro A, Sapienza P, Di Marzo L, Feldhaus RJ, Cavallari N. International registry of inferior vena cava leiomyosarcoma: Analysis of a world series on 218 patients. Anticancer Res 1996;16:3201-5.  Back to cited text no. 2
    
3.
Hemant D, Krantikumar R, Amita J, Chawla A, Ranjeet N. Primary leiomyosarcoma of inferior vena cava, a rare entity: Imaging features. Australas Radiol 2001;45:448-51.  Back to cited text no. 3
    
4.
Dew J, Hansen K, Hammon J, McCoy T, Levine EA, Shen P. Leiomyosarcoma of the inferior vena cava: Surgical management and clinical results. Am Surg 2005;71:497-501.  Back to cited text no. 4
    
5.
Perl L, Virchow R. Ein fall von sarkom der vena cava inferior. Archiv f pathol Anat 1871;53:378-83.  Back to cited text no. 5
    
6.
Melchior E. Sarkom der vena cava inferior. Deutsche Zeitschrift f. Chirurgie 1928;213:135-40.  Back to cited text no. 6
    
7.
Wachtel H, Gupta M, Bartlett EK, Jackson BM, Kelz RR, Karakousis GC, et al. Outcomes after resection of leiomyosarcomas of the inferior vena cava: A pooled data analysis of 377 cases. Surg Oncol 2015;24:21-7.  Back to cited text no. 7
    
8.
Bednarova I, Frellesen C, Roman A, Vogl TJ. Case 257: Leiomyosarcoma of the inferior vena cava. Radiology 2018;288:901-8.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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