Wednesday, November 23, 2011

Intravascular Leiomyosarcoma Of The Femoral Vein:Is It Rare?



Intravascular leiomyosarcomas are extremely rare.Going by the literature fewer than 40 cases have been reported so far wherein the tumour arose from the femoral vein.

Leiomyosarcomas usually arise from the muscular layers of the uterus or from the GI tract.Its an uncommon occurence for them to arise from the blood vessels.We report to you a case of leiomyosarcoma arising from the femoral vein.

A 40 years old male came to us with the complaints of swelling from the right upper thigh which he noticed some 2 weeks back.The swelling was associated with localised pain.There was no history of peripheral oedema and the initial radiological evaluation was suggestive of tumour arising in the vicinity of blood vessels.Further evaluation with MRI indicated the possibility of leiomyosarcoma.Doppler venous scan done for the status of deep veins was suggestive of chronic DVT of the popliteal and proximal superficial veins.Its surprising that the patient never had any symptoms of DVT like limb oedema etc.

At surgery the mass was found to be arising from the SFV from just where it drains into the CFV involving almost 10 cms of the venous segment.It was closely adherent to the femoral artery which was excised alongwith for wider surgical clearance.The femoral artery was reconstructed with ePTFE graft. SFV was not not reconstructed though because of chronic changes.

The histopathology specimen consisted of typical spindle cells disposed in interlacing fascicles with areas of hemorrhage.The final diagnosis was proved by IHC studies.

The postoperative course was uneventful.The patient is doing well and is referred to the medical oncologist for chemotherapy/ radiation therapy since these tumours are known to metastize to lung liver and scalp.

DISCUSSION
Clinical presentation depends on the extraluminal/intraluminal growth of the tumour mass.If extraluminal it can result in nerve compression causing pain and if intraluminal it can mimicthe symptoms of DVT.
Management of such cases are challenging because of very low incidence with most of the data available in literature being single reports.