Metastatic Leiomyosarcoma of the Inferior Vena Cava presenting with a hepatic mass

Posted By Renan Ibrahem
Metastatic Leiomyosarcoma of the Inferior Vena Cava presenting with a hepatic mass

Summary: A case report of a 72-year-old female patient presenting with right-sided abdominal pain and back pain, on whom initial abdominal ultrasound revealed a hepatic mass. With the aid of subsequent CT imaging and biopsy, a leiomyosarcoma of the inferior vena cava was found. Palliative chemo-radiotherapy was offered to the patient. The literature on leiomyosarcoma, its presentation in the inferior vena cava and the use of diagnostic imaging has been discussed. Case report: A 72-year-old lady with a background of diet-controlled diabetes and hypertension, presented with 4 months history of non-specific right-sided abdominal and back pain, which had not resolved with simple analgesia. On examination, she was found to have marked lower limb oedema extending up to the middle of the torso. Abdominal examination revealed old mid line laparotomy scar with palpable hepatomegaly, 1cm below the sub-costal margin. There was no lymphadenopathy. Cardiovascular examination was normal and the jugular venous pressure (JVP) was not elevated. An initial ultrasound scan revealed a mass in the right lobe of the liver (Figure 1). A CT scan revealed a heterogeneous soft tissue tumour originating from the inferior vena cava (IVC). The mass was centered on the intra-hepatic IVC measuring 46 x 61 x 122 mm extending down to the infra-hepatic IVC (Figure 2a). The IVC lumen was nearly fully occluded by the tumour. The radiological appearances were typical of an IVC leiomyosarcoma. The middle and right hepatic veins were occluded with radiological features in the right lobe of the liver similar to appearances of Budd Chiari syndrome (Figure 2b). CT imaging also demonstrated a nodule in segment 7 of the liver suspicious of a metastatic deposit alongside bilateral pulmonary metastasis and small volume ascites. The liver function tests and tumor markers including alpha-fetoprotein, carcinoembryonic antigen and CA19-9 were normal. A transhepatic ultrasound-guided biopsy was performed. Histology showed a moderately pleomorphic spindle cell neoplasm with typical features of smooth muscle histogenesis3. Positive immunohistochemical staining for smooth muscle actin and desmin confirmed a diagnosis of a leiomyosarcoma. Following discussion at the regional sarcoma MDT meeting a decision to treat the patient with palliative chemo-radiotherapy was made. Discussion: Leiomyosarcoma (LMS) is a type of soft tissue sarcoma that originates from smooth muscle cells and considered the malignant counterpart of a leiomyoma. The most common specific subtypes of soft tissue sarcoma registered in the UK between 2008-2010 were leiomyosarcoma (18%), fibroblastic sarcoma (14%) and liposarcoma (13%). According to data published by Cancer Research UK1, 20% of sarcomas were recorded as “sarcoma NOS” a group which includes tumours diagnosed as undifferentiated sarcoma and those without a specific subtype named. Two thirds of the cases of leiomyosarcoma were foun

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  • Roland Talanow 2016-02-28 18:04:34

    Thank you very much for this case. In case you would like to submit it to the JRCR:

    1. Please do not copy and paste your entire manuscript - just [provide the key points and findings and please explain what makes it so special and provides merit for publication? Thank you.

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