In September 2015, a 24-year-young man was admitted to our hospital with complaints of headache, sweating, anxiety, dizziness. It was confirmed that he had a history of hypertension and type 2 diabetes from clinical syndromes and examination. He was a smoker with no significant family history. His resting pulse rate was 100 beats/min. The patient’s blood pressure was 163/100 mmHg. Physical examination revealed mild lower abdominal tenderness, both edema of lower extremity and collateral formation over abdomen were unremarkable. Hepatomegaly and abdominis hydrops were also absent in the young man.
Abdominal CT revealed a well defined, heterogenous mass lesion of size 11.1 × 10.5 × 11.1 cm at the upper pole of right kidney, which was rooted in right adrenal gland. Vena cava area exhibited filling defect, which indicated the possibility of tumor thrombosis extension from localized tumor. Magnetic resonance imaging (MRI) reveal the proximal part of inferior vena cava was widened (Figure 1A), and confirmed intraluminal thrombus extending proximally up to the confluence of hepatic veins immediately inferior to the right atrium, the distance of extended tumor thrombosis was 6.5cm approximately (Figure 1B). Echocardiography indicated this patient had atrial septal defect, the defect size was 0.2cm, and only showed mild tricuspid regurgitation with 63% EF. Surgical treatment was been suggested. However, the patient gave up the surgical treatment and left hospital.
In April 2018, this young man once again presented to the emergence department with a persistent cough and intermittent wheezing character for 5 hour. At this time, his blood pressure was 160/120 mmHg with a heart rate of 142 beats per minute and SpO2 was 95% on room air. Bilateral lower limb arterial and venous examination was normal. Contrast-enhance CT showed a 12-cm right adrenal mass with suspected tumor thrombosis extending into right atrium of the heart(Figure 2A), the distance of extended tumor thrombosis was 13.5cm approximately (Figure 2B). Echocardiography conformed that existing IVC tumor thrombus had extended into the right atrium (Figure 3), EF was only 42%. The biochemical evaluation revealed elevated of dopamine 6.03 nmol/L (normal range: 2.09-3.91 nmol/L), adrenaline 15.15 nmol/L ( normal range:1.31-2.51 nmol/L), noradrenline 33.17nmol/L (normal range:1.31-2.30 nmol/L). Preoperative electrocardiography showed supraventricular tachycardia and 24 h Holter monitoring showed no significant cardiac arrhythmia except intermittent junctional rhythm. A primary diagnosis of pheochromocytoma with IVC and right atrium tumor thrombosis was established. The patient finally agreed to receive a surgical treatment. Before receiving the surgical treatment, the patient recurrently presented heart failure in intensive care unit, a combination of alpha-adrenergic and beta-adrenergic blockade and calcium channel blockers were routinely used to maintain the blood pressure and stabilize heart function. In order to avoid the occurrence of hypotension after resecting the phaeochromocytoma, phentolamine was discontinued on the day before the operation. In addition, the beta-adrenergic blockade was continued until the day of operation.
Nearly two months later, our anesthetist team finally evaluated the cardiac system of the patient. And we considered that it was an appropriate time to perform the operation based on the relatively favorable physical condition. After being administered intravenous midazolam (1.5 mg), the patient was brought to the operating room. The initial SBP and HR were 144 mmHg and 110 beats/min, respectively, Spo2 was 98% under room air. Two peripheral venous access were established by nurse immediately. After intravenous midazolam (2 mg) and sufentanil (5ug), the left radial artery was cannulated for continuous arterial blood pressure monitoring. Meanwhile, FloTrac system was employed to monitor the cardio output (CO), stroke volume (SV), stroke volume variation (SVV), systemic vascular resistance (SVR). Midazolam (4 mg), sufentanil (70 μg), rocuronium (70 mg) and etomidate (20 mg) were injected via peripheral venous access in sequence. After 2min of mask positive pressure ventilation, reinforced tracheal tube was intubated. Intravenous infusion of propofol and remifentanil were induced to maintain the anesthesia during the surgery, which were combined with sevoflurane. Internal jugular vein puncture was performed to place the central venous catheter and monitor central venous pressure (CVP). Subsequently, transesophageal echocardiography (TEE) probe was inserted to evaluate the ventricular function. Before incising the skin, methylprednisolone (80 mg) and Ulinastatin (7×10 unit) were used to resist systematic inflammation reaction. Pantolazole (80 mg) was induced to protect stomach. Subsequently, a midline xiphoid to pubic symphysis incision was induced. Because of the tumor thrombosis in the inferior vena cava and the right atrium, the liver was enlarged and congested secondary to the Budd-Chiari syndrome. A large and well-circumscribed right adrenal phaeochromocytoma was observered clearly, which actually extended through the adrenal vein and lateral wall of inferior vena cava into the right atrium. After the carefully dissociation of tissue, the tumor was successfully dissected from the kidney, liver, and retroperitoneum until it was only attached to the lateral wall of the vena cava at the area of tumor invasion, the resected tumor was shown in Figure 4A. During the exploration of abdominal, the circulation was extremely unstable. Sodium nitroprusside (0.5-3ug/kg/min) and phentolamine (0.5-3ug/kg/min) were induced to control the SPB in 60-160mmHg and the DPB in 50-100 mmHg. Nicardipine (1mg) and phentolamine (1mg) were also discontinuously intravenous injected to control the blood press cooperatively. Esmolol (50-300ug/kg/min) was induced to revolt tachycardia, but the control of heat rate was unsatisfactory, the heat rate was maintained in 90-140 beats/min. Subsequently, the cardiac surgeon continued to perform median sternotomy. After systemic heparinisation, the right femoral vein, superior vena cava, and the aorta were cannulated to establish cardiopulmonary bypass. Before the aorta was cross-clamped, the patient was gradually cooled to 32℃. After the infusion of cold cardioplegia into aortic root, cardiac arrest was achieved. Meanwhile, cardiopulmonary bypass was induced to support the circulation system. By cutting open the right atrium, tumor thrombosis at the right atrium was then resected, the elimination of tumor thrombosis in the vena cava was performed subsequently. The removal tumor thrombosis form IVC and right atrium was shown in Figure 4B. After repairment of right atrium and vene cava, The patient was weaned off from cardiopulmonary bypass after full re-warming. During the cardiopulmonary bypass, the blood pressure remained relatively stable with any severe hemodynamic disorder. In summarize, the operating time of this patient was totally 11.5 hours, the blood loss was approximately 10000 mL. The diagnosis of phaeochromocytoma was finally confirmed by immunohistochemical method. After one month post-operation care, the patient healthily left our hospital.