A 60-year-old man presented with a 2-month history of persistent right epigastric pain and a weight loss of 9 kg, without fever, headaches, palpitations, and sweating attacks. His two brothers had previously died of esophageal cancer and gastric cancer. At a local hospital, a computed tomography (CT) scan without using contrast medium revealed a large retroperitoneal mass that was characterized by a non-homogeneous appearance and slightly low density. Magnetic resonance imaging showed both hypo- and hyper-signal intensities on T1-weighted images, whereas only hyper-signal intensity was observed on T2-weighted images. Compression of the hepatic segment of the IVC was also observed. Due to the complex anatomy of the tumor region and the need for resection, the patient was referred to a tertiary unit for further oncological and surgical assessment.
Upon admission, the patient denied any history of hypertension or any other cardiovascular disease. A general physical examination findings were normal. The patient’s blood pressure varied at normal level (110-130/69-86 mmHg). Blood and urine analyses, liver and kidney function, and clotting time, were also normal.
The patient’s fasting blood glucose level was 7.34 mmol/L (reference range: 3.9–6.11 mmol/L) and glycosylated hemoglobin level was 7.2 mg/ dL. Serum levels of tumor markers, including carcinoembryonic antigen, alpha-fe-toprotein, and carbohydrate, were normal. Preoperative levels of adrenocorticotropic hormone were assayed at various time points: 15.98 pg/mL at 0:00, 20.25 pg/mL at 8:00, and 14.40 pg/mL at 16:00. Plasma renin activity was 1.30 ng/mL (0.05–2.86 in the lying position), angiotensin II was 31.50 pg/mL (16.2–64.2, lying down), aldosterone was 91.00 pg/mL (59–174, lying down), and serum levels of cortisol were 7.95 μg/dL at 0:00, 14.53 μg/dL at 8:00, and 6.44 μg/dL at 16:00. Levels of vanillylmandelic acid and urine volume after 24 h were 31.41 mg (1.4–6.5 mg) and 2.05 L, respectively.
Ultrasonography revealed a 7.4 cm × 4.5 cm well-demarcated solid mass with mixed echoes (a mosaic of medium and low signals) located between the upper abdominal IVC and the aortic artery (Figure 1A). The hepatic segment of the IVC was also compressed and shifted forward. A contrast-enhanced CT with 3D reconstruction scan showed a 6.5 cm × 4.8 cm mass with non-homogeneous density located behind the retrohepatic segment of the IVC (Figure 1B&C). The mass was well-delineated, exhibited non-homogeneous enhancement with multiple necrotic cysts, and the adjacent lymph nodes were not swollen. Compression of the right adrenal gland, caudate lobe, and IVC, and invasion of the right side of the diaphragm horn were also observed. The retrohepatic segment of the IVC was mostly compressed, with the left vessel wall attached to the mass. There was no clear boundary between the left vessel wall and the mass. In addition, there were no filling defects visualized in the vessel lumen, and no distant metastasis was detected. Thus, a preoperative diagnosis of primary retroperitoneal tumor (except for normotensive ectopic pheochromocytoma) was made.
Prior to undergoing a laparotomy, the patient completed 3-day volume expansion therapy with artificial colloid solution. During surgical exploration, a generous right subcostal incision was made. The mass of interest was found behind the retrohepatic portion of the IVC. The adjacent lymph nodes were not swollen. Compression of the IVC was observed, with the hepatic hilum lifted in an anterior direction (Figure 2A). The tumor and retrohepatic portion of the IVC were fully exposed by resection of the hepatic left lateral lobe. The tumor was identified from the renal level to the second hepatic hilum, and a portion of the caudate lobe was found to be affected. Consequently, the affected portion of the caudate lobe were resected, and extensive dissection of the IVC followed. Briefly, the IVC was carefully mobilized clockwise in a cephalad and dorsal direction up to the level of the second hepatic hilum via a left lateral side approach. Finally, the prevertebral space was divided carefully behind the tumor, and a portion of the affected diaphragm tissue on the right side, as well as the surrounding diaphragmatic muscle, were removed (Figure 2B&C). Thus, the tumor was successfully removed along with the left lateral lobe of the liver, a portion of the caudate lobe of the liver, and the gallbladder.
Intraoperatively, there was two sharp changes in hemodynamic status of the patients observed during manipulation of the tumour (blood pressure rose to 150-160/90-100 mmHg), which was treated with nicardipine hydrochloride and sodium nitroprusside. Esmolol hydrochloride was used to decrease the heart rate. After the tumor was resected, the blood pressure dropped to 70/50 mmHg and norepinephrine was pumped continuously one half hour. Blood pressure rose and steadied at 110/85 mmHg in the end.
The resected tumor (8 cm × 6 cm × 4 cm) was solid, nodular, and encapsulated. On the tumor surface, many tortuous vessels and some localized necrotic tissue were observed (Figure 2D). Macroscopically, tumor invasion into the liver and the adrenal gland was not apparent. However, microscopically, the tumor was found to have invaded the caudate lobe. Histological analysis identified alveolar-like structures with many vascular septa within the tumor (Figure 3A). In addition, the tumor cells contained fine eosinophilic granules, while cell mitosis and nuclear atypia were infrequent. Immunohistochemical analyses demonstrated that the tumor cells were negative for α-smooth muscle actin (SMA), and positive for chromogranin A (CgA), synaptophysin (Syn), S-100, cytokeratin (CK), and CD34 (Figure 3B&C).
By 3 days after surgery, the patient undergone closed thoracic drainage for the right side of massive pleural effusion and aerothorax with the obviously compression of the right lung. The closed thoracic drainage tube was removed on postoperative day 18 and the patient was discharged on postoperative day 21. Postoperative adjuvant chemotherapy CVD-protocol (cyclophosphamide 750 mg/m2, vincristine 1.4 mg/m2, and dacarbazine 600 mg/m2 on Day 1 and dacarbaine 600 mg/m2 on Day 2; every 21 to 28 days) was recommended; however, the patient refused. During the follow-up period, the patient’s initial symptoms of paraganglioma rapidly disappeared. However, 3 months later, ultrasonography revealed hepatic metastasis (Figure 1D). The patient refused any other treatment and died 3 months later.
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