Gunasekaran Vasanthakumar, Rammohan Ashwin, Reddy Srinivas Mettu, Rela Mohamed
Institute of Liver Disease & Transplantation, Gleneagles Global Health City, Chennai, Tamil Nadu, India.
Institute of Liver Disease & Transplantation, Dr.Rela Institute & Medical Centre, Bharat Institute of Higher Education & Research, Chennai, Tamil Nadu, India.
BMJ Case Rep. 2018 Sep 28;2018:bcr-2018-225827. doi: 10.1136/bcr-2018-225827.
Leiomyosarcoma (LMS) of primary vascular origin is a rare entity with only potentially curative option being complete surgical resection; despite which the prognosis remains dismal. Tumour recurrence is very common, and the benefits of adjuvant therapy are undefined. A 39-year-old woman presented with 6 months' history of abdominal pain, abdominal distension and pedal oedema. On evaluation, she was diagnosed to have chronic Budd-Chiari syndrome (BCS) secondary to a tumour arising from the inferior vena cava (IVC) on evaluation. Her liver decompensation included jaundice, gastrointestinal bleed and ascites. Following a detailed multidisciplinary team discussion, she underwent complete excision of the tumour along with a segment of the IVC with living donor liver transplantation. She remains disease-free 24 months following surgery. This is the first reported case of liver transplantation for IVC LMS causing chronic BCS.
原发性血管源性平滑肌肉瘤(LMS)是一种罕见的疾病,唯一可能治愈的方法是完整的手术切除;尽管如此,其预后仍然很差。肿瘤复发非常常见,辅助治疗的益处尚不明确。一名39岁女性有6个月的腹痛、腹胀和足部水肿病史。经评估,她被诊断为继发于下腔静脉(IVC)肿瘤的慢性布加综合征(BCS)。她的肝脏失代偿包括黄疸、胃肠道出血和腹水。经过详细的多学科团队讨论,她接受了肿瘤连同一段IVC的完整切除以及活体供肝移植。术后24个月她仍无疾病复发。这是首例因IVC LMS导致慢性BCS而进行肝移植的报道病例。