Renal Lipoma-Like Angiomyolipoma of Tumour Thrombus Extending to the Confluence of Inferior Vena Cava with Right Atrium: A Rare Case Report and Literature Review

Angiomyolipoma (AML) complicated with tumour thrombus extending to the confluence of inferior vena cava (IVC) with right atrium is rarely observed. We report a female AML patient admitted to our centre on January 21, 2020, with complication of tumour thrombus extending to the confluence of IVC with right atrium and had no sign of difficult breathing. She underwent whole-abdominal enhanced CT for abdominal pain and was diagnosed with a possible renal AML with tumour thrombus. Open radical nephrectomy and thrombectomy of vena cava were performed. Intraoperative transoesophageal echocardiography indicated that the tumour thrombus has reached the confluence of IVC with right atrium. The operation took 255 min with an intraoperative haemorrhage of 800mL. The patient was discharged 7 days after surgery. Pathology revealed lipoma-like AML. Immunohistochemistry showed vimentin (+), EMA (−), HMB45 (+), S-100 (−), SMA (+), TFE-3 (−), melan A (+). After 2 years of follow-up, we found that the patient showed full recovery and had no recurrence. Therefore, lipoma-like AML should also be followed closely for recurrence and metastasis. When AML involves IVC tumour thrombus, open thrombectomy and radical nephrectomy are safe and effective methods.

[1]  S. Eggener,et al.  Clinical and pathological features of renal epithelioid angiomyolipoma (PEComa): A single institution series. , 2021, Urologic oncology.

[2]  S. Daneshmand,et al.  Vena Cava Tumor Thrombus Associated with Renal Angiomyolipoma in a Jehovah's Witness Patient. , 2021, Urology.

[3]  M. Xie,et al.  Right renal angiomyolipoma with intracardiac extension and pulmonary embolism , 2020, Journal of clinical ultrasound : JCU.

[4]  B. Sureka,et al.  Epithelioid angiomyolipoma with tumor thrombus in IVC and right atrium , 2020, Autopsy & case reports.

[5]  A. Osunkoya,et al.  A Rare Case of Vena Cava Tumor Thrombus Associated with Epithelioid Angiomyolipoma. , 2020, Urology.

[6]  M. Abdessater,et al.  Renal angiomyolipoma with IVC thrombus: A case report , 2020, International journal of surgery case reports.

[7]  S. Al-katib,et al.  Aggressive Renal Angiomyolipoma in a Patient With Tuberous Sclerosis Resulting in Pulmonary Tumor Embolus and Pulmonary Infarction. , 2018, Urology.

[8]  H. Zeng,et al.  A rare case of renal angiomyolipoma involved both inferior vena cava and pulmonary arteries. , 2018, Journal of thoracic disease.

[9]  M. McInnes,et al.  Renal angiomyolipoma without visible fat: Can we make the diagnosis using CT and MRI? , 2018, European Radiology.

[10]  Mark W. Ball,et al.  Defining the Role of Intraoperative Transesophageal Echocardiography During Radical Nephrectomy With Inferior Vena Cava Tumor Thrombectomy for Renal Cell Carcinoma. , 2017, Urology.

[11]  R. Sotelo,et al.  Robot-assisted Level II-III Inferior Vena Cava Tumor Thrombectomy: Step-by-Step Technique and 1-Year Outcomes. , 2017, European urology.

[12]  A. Evans,et al.  Natural History of Renal Angiomyolipoma (AML): Most Patients with Large AMLs >4cm Can Be Offered Active Surveillance as an Initial Management Strategy. , 2016, European urology.

[13]  Hong-zhao Li,et al.  Robot-assisted Laparoscopic Inferior Vena Cava Thrombectomy: Different Sides Require Different Techniques. , 2016, European urology.

[14]  Ximing J. Yang,et al.  Update on the Diagnosis and Management of Renal Angiomyolipoma. , 2016, The Journal of urology.

[15]  Hao Ning,et al.  Invasive Epithelioid Angiomyolipoma with Tumor Thrombus in the Inferior Vena Cava: A Case Report and Literature Review , 2015, Urologia Internationalis.

[16]  M. Janík,et al.  Renal cell carcinoma pulmonary embolism: a rare cause of sudden death , 2015, Forensic Science, Medicine, and Pathology.

[17]  C. Milani,et al.  Renal angiomyolipoma with renal vein extension , 2015 .

[18]  A. Satelli,et al.  Vimentin in cancer and its potential as a molecular target for cancer therapy , 2011, Cellular and Molecular Life Sciences.

[19]  W. Kassouf,et al.  Renal cell carcinoma with inferior vena caval extention: impact of tumour extent on surgical outcome , 2009, BJU international.

[20]  A. Khallouk,et al.  [Aggressive renal angiomyolipoma extending to the renal vein: about a case and literature review]. , 2017, The Pan African medical journal.

[21]  J. Bissler,et al.  Renal angiomyolipomata. , 2004, Kidney international.