Clinical Combined Round presentation By Dr Anil on rare Case :Primary Leiomyosarcoma of Juxtarenal IVC:

1. PRIMARY LEIOMYOSARCOMA OF IVC
A RARE ENTITY
SURGERY UNIT- II

2. 60 year old female presented with six months
history of,
 vague right loin pain
 Loss of weight and appetite-10kg in
6 months
weakness

3. No h/o hematuria
No h/o fever
No h/o altered bowel habits
No h/o walking difficulties
H/o B/L knee replacement for arthritis 2years
back.
H/o lap.cholecystectomy -one year back

4. Moderately built.
pale.
Pedal Edema +
P/A
Few dilated superficial veins over lower
abdomen and upper thighs.
no lump found.

5. Hb – 9.7g/l
Tlc -8700
Plt -124000
Urea -45
Cr -1.1mg/dl
Sr .bil-0.9mg /dl
Sgot/pt -27/23
Sr.Alp -98 iu /dl
Coagulation profile-normal

7. Usg abdomen
6*7 cm heteroechoic lobulated
retroperitoneal mass lesion adjacent to renal
part of IVC on right side with partial luminal
compression
? RP mass with IVC thrombus
? IVC tumor
Suggested Usg doppler

8. IVC doppler revealed compressed juxta renal
IVC with reduced upward flow
?thrombus
?tumor
CECT abdomen was advised.

10. Malignant spindle cell tumor
IHC-Positive for desmin and smooth muscle
actin and negative for s-100,c-kit
? Leiomyosarcoma-retroperitoneum.

11. Exploratory laparotomy and proceed

12. Intra operatively :
Hard infiltrating tumor arising from juxta
renal ivc which was extending about 5cm
above and 3cm below the Rt renal vein.
Rt renal vessels were completely encased by
the tumor.
Aorta free

15. The liver was mobilized to expose the sub hepatic
part of the IVC to achieve proximal clearance of the
tumor.
The infra-diaphragmatic aorta was slung and a clamp
was placed at the level superiorly to control inflow.
Left renal vein was ligated and transected from IVC.
The patient was heparinised and the tumor resected
en block along with a right nephrectomy after
applying proximal and distal clamps to the IVC

16. A Dacron 18mm interposition graft was used
to reconstruct the IVC.
The left renal vein was re-implanted in an
end to side manner into the Dacron graft.

22. Stable, Good urine output
NPO for 4days
On anticoagulation therapy
Discharged on day 15.

24. Doxorubicin based
chemotherapy.
Radiotherapy also
planned.
Now on 6 months
follow-up without any
complaints.

26. • Extremely rare tumor with fewer then 200
cases reported in literature since 1871 *
• First reported case : Perl in 1871
• Documented in the surgical literature mostly
as case reports rather than case series.
• Invariably malignant
• Prognosis depends on early diagnosis and
management
* 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(5B):3201-3205.

27. Origin – arises from the smooth muscle cells
of the media of the cava
Venous leiomyosarcoma occur 5 times more
often than arterial ones
Among all the veins 50% originating in the
IVC.

29. Encapsulated, consisting of lobulated whorls
Histologically demonstrates spindle shaped
bundles of cells with high mitotic activity
Positive staining for desmin,HHF35,vimentin
and smooth muscle actin

30. PRIMARY TUMOR
Arises directly from the vessel wall.
SECONDARY TUMOR
Arises from adjacent retroperitoneal
structures and invades IVC.
RCC
Pheochromocytoma
Testicular tumor etc

31. Depending on site of IVC involved:
 Supra hepatic IVC tumor (24%)
Arises from above hepatic vein origin
to right atrium.
Very difficult to operate
Poor prognosis.

32.  Retro hepatic IVC tumor (42% ) :
Arises from middle part of IVC (from
renal vein origin to hepatic vein
origin).
Most common site of primary tumor
 Lower IVC tumor (34%)
Arises from lower part of IVC
(below renal vein draining into IVC )


33. Pathologically *
(1) Primarily Exophytic tumor – 62%
(2)Primarily Intraluminal tumor-5%
(3) Combined -33%
(difficult to differentiate from secondary
tumor arising from retro peritoneum and
invading IVC)
*Hartman DS, Hayes WS, Choyke PL,Tibbets GP. From the archives of
the AFIP. Leiomyosarcoma of the retroperitoneum and inferior vena cava:
radiologic-pathologic correlation. Radio graphics 1992;12:1203–1220.

34. Usually asymptomatic in early stage
thus delaying diagnosis.
More common in females M:F=1:5
Usually presents at sixth- seventh
decade

35. Symptoms and signs are nonspecific-
• Abdominal Pain (66%)
• Abdominal mass (48%)
• Lower limb edema (39%)
• Budd – Chiari syndrome (22%)
• Others- fever, weakness , anorexia ,
nocturnal sweating and dyspnoea
Bower TC, Stanson A. Diagnosis and management of tumors of
the inferior vena cava. Vascular surgery, 5thed. Philadelphia: WB
Saunders, 2000: 2077–2091.

36. Upper IVC involvement=Budd chiari
syndrome
Middle IVC involvement=Nephrotic syndrome
Infrarenal involvement=Lower extremity
edema

37. Mainly haematogenous
To liver,lung and brain
In advanced stage may spread through
lymphatics

38. ULTRASONOGRAPHY
Initial screening test
Doppler USG helps to assess vascularity of
tumor
It also helps to differentiate IVC tumor
from Intraluminal thrombus.

39. Fig 1- USG showing Intraluminal IVC tumor
Fig-2 colour doppler usg showing slow
resistance flow in intraluminal tumor
 Fig 1- USG showing Intraluminal IVC
tumor
Fig-2 colour doppler usg
showing slow resistance flow in
intraluminal tumor

41. It is useful screening test to diagnose IVC
tumor.
CT guided biopsy can be taken from
exophytic component.
However sometimes it becomes difficult on
CT to differentiate between primary and
secondary IVC tumor.

42. Fig- Contrast CT scan showing heterogenously
enhanced mass in the IVC.

44. Advantage includes-
• Allows multi-planer imaging
• This gives highly accurate assessment of
relationship of tumor with adjacent
structures.
• Vessel patency can be assessed using flow
void or flow enhancement properties.

46. The cephalo caudal
extent of tumor within
the cava, and thus
potential resectability can
also be determined.
Fat suppression and
Gadolinium enhancement
futher increases quality
of MRI imaging.

47. Invasive technique
Intraluminal tumor – on Cavography, the
IVC is dilated and the tumor is detected as a
mass dilating and filling the lumen.
Exophytic tumor is detected as a mass
surrounding the IVC that may protrude into
the lumen.

48. Primary leiomyosarcoma of IVC are slow
growing tumor and are invariably malignant.
Usually diagnosed late due to nonspecific
symptoms.
Surgical resection is treatment of choice
whenever tumor is resectable.
Even if unresectable , surgery gives best
palliative treatment.
Even on recurrence sx is the only option
Chemotherapy and radiotherapy- role is
controversial .

49. Limited studies about surgical management
and limited data on long term survival after
surgery.
Complete surgical resection with a one cm
of tumor free margin is considered
treatment of choice
Hemant D, Krantikumar R, Amita J, Chawla A, Ranjeet N: Primary
Leiomyosarcoma of inferior vena cava, a rare entity: Imaging features. Austral
as Radiol 2001, 45(4):448-451

50. Ligation of IVC associated with severe limb
edema due to disruption of collaterals.
Current recommendation is to use prosthetic
graft for reconstruction of the IVC .
Ring enforced PTFE or Dacron graft are
commonly used Prosthesis for IVC
reconstruction.
Sarkar R, Eilber FR, Gelabert HA, Quinones-Baldrich WJ. Prosthetic
replacement of the inferior vena cava for malignancy. J Vasc Surg 1998; 28:
75–83.

51. International registry of IVC leiomyosarcoma
analyzed 218 patients.
A radical tumor resection was undertaken in 134
(61.5%) patients, 26 (11.9%) had a palliative
resection, and 58 (26.6%) were inoperable.
Radical tumor resection was associated with
better 5- and 10-year survival rates (49.4% and
29.5%) when compared to patients undergoing
palliative resection or those who were inoperable
Mingoli A , Cavallaro A, Sapienza P, Di Marzo L, Feldhaus RJ, Cavallari
N . International registry of inferior vena cava Leiomyosarcoma. Analysis of
World Series of 218 patients. Anticancer Res 1996;16: 3201-3206

52. Hollenbeck et al reported 25 patients of
primary IVC tumor treated between 1982
and 2002.
Study showed that patients undergoing
complete resection had 3- and 5-year
survival rates of 76% and 33% respectively.
Hollenbeck ST, Grobmeyer SR, Kent KC, Brennan MF: Surgical
treatment and outcomes of patients with primary inferior vena cava
Leiomyosarcoma. J Am Coll Surg 2003, 197(4):575-579.

53. Role still controversial
Adriamycin/ Ifosfamide based regimen are
commonly used.
However no case series study to suggest its
exact

54. Less information about its role
Some study suggest radiotherapy reduces the
recurrence rate.

55. Poor Prognostic Factors:
• Upper IVC involvement
• Lower limb edema
• Budd- chiari syndrome
• IVC occlusion
• Distant metastasis

56. Primary leiomyosarcoma a rare tumor which
are invariably malignant.
Slow growing tumor with delayed nonspecific
presentation.
MRI is investigation of choice
Sometime difficult to differentiate between
primary and secondary IVC tumor
Surgery is treatment of choice , even for
palliation .
Role of chemotherapy and radiotherapy
controversial.

57. THANK YOU !