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Journal of Peking University (Health Sciences) ›› 2024, Vol. 56 ›› Issue (4): 617-623. doi: 10.19723/j.issn.1671-167X.2024.04.012

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Clinical diagnosis and treatment of renal angiomyolipoma with inferior vena cava tumor thrombus

Kewei CHEN,Zhuo LIU,Shaohui DENG,Fan ZHANG,Jianfei YE,Guoliang WANG,Shudong ZHANG*()   

  1. Department of Urology, Peking University Third Hospital, Beijing 100191, China
  • Received:2024-03-15 Online:2024-08-18 Published:2024-07-23
  • Contact: Shudong ZHANG E-mail:zhangshudong@bjmu.edu.cn
  • Supported by:
    the National Natural Science Foundation of China(82273389);the Beijing Natural Science Foundation(7232212)

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Abstract:

Objective: To summarize the clinical characteristics of patients with renal angiomyolipoma (RAML) combined with inferior vena cava (IVC) tumor thrombus, and to explore the feasibility of partial nephrectomy and thrombectomy in this series of patients. Methods: The clinical data of patients diagnosed with RAML combined with IVC tumor thrombus in the Department of Urology of the Peking University Third Hospital from April 2014 to March 2023 were retrospectively analyzed, and demographic and perioperative data of RAML patients with IVC tumor thrombus were recorded and collected from Electronic Medical Record System, including age, gender, surgical methods, and follow-up time, etc. The clinical characteristics between classic angiomyolipoma (CAML) patients with IVC tumor thrombus and epithelioid angiomyolipoma (EAML) patients with IVC tumor thrombus were compared to determine the clinical characteristics of these patients. Results: A total of 11 patients were included in this study, including 7 patients with CAML with IVC tumor thrombus and 4 patients with EAML with IVC tumor thrombus. There were 9 females (9/11, 81.8%) and 2 males (2/11, 18.2%), with an average age of (44.0±17.1) years. 9 patients (9/11, 81.8%) experienced clinical symptoms, including local symptoms including abdominal pain, hematuria, abdominal masses, and systemic symptoms including weight loss and fever; 2 patients (2/11, 18.2%) with RAML and IVC tumor thrombus did not show clinical symptoms, which were discovered by physical examination. Among the 11 patients, 10 underwent radical nephrectomy with thrombectomy, of whom, 3 underwent open surgery (3/10, 30.0%), 2 underwent laparoscopic surgery (2/10, 20.0%), and 5 underwent robot-assisted laparoscopic surgery (5/10, 50.0%). In addition, 1 patient underwent open partial nephrectomy and thrombectomy. The patients with EAML combined with IVC tumor thrombus had a higher proportion of systemic clinical symptoms (100% vs. 0%, P=0.003), more intraoperative bleeding [400 (240, 3 050) mL vs. 50 (50, 300) mL, P =0.036], and a higher proportion of tumor necrosis (75% vs. 0%, P=0.024) compared to the patients with CAML combined with IVC tumor thrombus. However, there was no statistically significant difference in operation time [(415.8±201.2) min vs. (226.0±87.3) min, P=0.053] between the two groups. Conclusion: Compared with the patients with CAML and IVC tumor thrombus, the patients with EAML and IVC tumor thrombus had a higher rate of systemic symptoms and tumor necrosis. In addition, in the selected patients with CAML with IVC tumor thrombus, partial nephrectomy and tumor thrombectomy could be performed to better preserve renal function.

Key words: Renal angiomyolipoma, Tumor thrombus, Partial nephrectomy, Thrombectomy

CLC Number: 

  • R737.1

Figure 1

Specimens of the patients receiving partial nephrectomy and thrombectomy"

Figure 2

Robot-assisted laparoscopic radical nephrectomy and thrombectomy A, block the distal inferior vena cava; B, free the inferior vena cava at the lesion site for opening inferior vena cava and removing the tumor thrombus; C, no significant bleeding in the surgical field after removing the tumor thrombus; D, suture the inferior vena cava."

Figure 3

Coronal CT image of the patient with renal angiomyolipoma and tumor thrombus A, CT shows right renal angiomyolipoma; B, CT shows tumor thrombus invading the inferior vena cava."

Table 1

Patients' demographic data and perioperative data"

Items Total (n=11) CAML (n=7) EAML (n=4) P value
Age/years, $\bar x \pm s$ 44.0±17.1 45.7±19.8 41.0±13.4 0.684
Gender, n(%) 0.109
  Male 2 (18.2) 0 (0) 2 (50.0)
  Female 9 (81.8) 7 (100.0) 2 (50.0)
BMI/(kg/m2), $\bar x \pm s$ 23.3±2.6 24.9±2.6 24.0±2.9 0.556
Local symptom, n(%) 9 (81.8) 5 (71.4) 4 (100) 0.491
Systemic symptom, n(%) 4 (36.4) 0 (0) 4 (100) 0.003*
Comorbidity, n(%) 3 (27.3) 2 (28.6) 1 (25.0) 0.999
Preoperative Scr /(μmoI/L), M(Min, Max) 65 (51, 87) 62 (51, 87) 80 (59, 83) 0.215
Hemoglobin/(g/L), $\bar x \pm s$ 122.4±20.6 116.9±17.1 132.0±25.2 0.262
Platelet/(×109/L)，$\bar x \pm s$ 278.6±124.7 260.9±114.4 309.8±153.8 0.560
Albumin/ (g/L), M(Min, Max) 41.3 (24.8, 47.1) 41.2 (33.0, 47.1) 41.9 (24.8, 44.6) 0.927
Blood urea nitrogen/ (mmoI/L)，$\bar x \pm s$ 3.8±1.1 3.4±1.2 4.5±0.58 0.154
Postoperative Scr /(μmoI/L), M(Min, Max) 67 (51, 100) 67 (59, 99) 73 (51, 100) 0.679
Side, n(%) 0.999
  Left 2 (18.2) 1 (14.3) 1 (25.0)
  Right 9 (81.8) 6 (85.7) 3 (75.0)
Tumor diameter/cm, $\bar x \pm s$ 7.0±4.2 5.8±4.4 9.1±3.5 0.232
Mayo grade, n 0.062
  0 1 1 0
  1 1 1 0
  2 7 5 2
  3 2 0 2
Operation approach 0.474
  Open, n(%) 4 (36.4) 2 (28.6) 2 (50.0)
  Laparoscopy, n(%) 2 (18.2) 2 (28.6) 0 (0)
  Robot-assisted laparoscopy, n(%) 5 (45.5) 3 (42.9) 2 (50.0)
IVC resection, n(%) 1 (9.1) 0 (0) 1 (25.0) 0.364
Operation time/min, $\bar x \pm s$ 295.0±160.9 226.0±87.3 415.8±201.2 0.053
Estimated blood loss/mL, M(Min, Max) 220 (50, 300) 50 (50, 300) 400 (240, 3 050) 0.036*
Blood transfusion, n(%) 4 (36.4) 2 (28.6) 2 (50.0) 0.576
Venous wall involvement, n(%) 2 (18.2) 0 (0) 2 (50.0) 0.109
Lymphadenectomy, n(%) 3 (27.3) 0 (0) 3 (75.0) 0.024*
Adhesion, n(%) 2 (18.2) 0 (0) 2 (50.0) 0.109
Necrosis, n(%) 3 (27.3) 0 (0) 3 (75.0) 0.024*
Complications, n(%) 3 (27.3) 2 (28.6) 1 (25.0) 0.999
Postoperative hospital stay/d, $\bar x \pm s$ 8.1±5.4 8.9±6.7 6.8±1.7 0.563
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