|Year : 2013 | Volume
| Issue : 7 | Page : 183-185
Surgical resection of multiple liver metastasis of functional malignant pheochromocytoma: A case report and literature review
Shaozhong Wei1, Dongde Wu2, Junqiu Yue3
1 Department of Urology Surgery, Cancer Hospital of Wuhan University and Hubei Cancer Hospital Wuhan 430079, China
2 Department of Hepatobiliary and Pancreatic Surgery, Cancer Hospital of Wuhan University and Hubei Cancer Hospital Wuhan 430079, China
3 Department of Pathology, Cancer Hospital of Wuhan University and Hubei Cancer Hospital Wuhan 430079, China
|Date of Web Publication||30-Nov-2013|
Wu, 116, South Road, Zhuodaoquan, Wuhan 430079
Source of Support: None, Conflict of Interest: None
Functional malignant pheochromocytoma is a rare tumor. Also, liver metastatic pheochromocytoma is deemed to be incurable because of its poor prognosis. Surgical debulking and removal of primary and metastases tumors, as well as radiosurgical or surgical treatment are recommended for patients. We treated a patient with multiple liver metastasis of functional malignant pheochromocytoma 4 years after the adrenal primary lesion was resected. The liver metastases were successfully removed by surgical resection. During the operation, the blood pressure increased quickly when the tumor was held between the fingers, reached the highest (201/110 mmHg), and then quickly declined to normal when the pressure was relieved. The patient is alive for more than 5 years after the operation. Thus, we believe that functional liver metastases of malignant pheochromocytoma may be curable by surgical resection with careful monitoring of anesthesia and blood pressure. Every effort should be taken to avoid direct stimulation of the tumor in the operation.
Keywords: Functional malignant pheochromocytoma, liver metastasis, prognosis, surgical resection
|How to cite this article:|
Wei S, Wu D, Yue J. Surgical resection of multiple liver metastasis of functional malignant pheochromocytoma: A case report and literature review. J Can Res Ther 2013;9, Suppl S2:183-5
|How to cite this URL:|
Wei S, Wu D, Yue J. Surgical resection of multiple liver metastasis of functional malignant pheochromocytoma: A case report and literature review. J Can Res Ther [serial online] 2013 [cited 2019 Sep 16];9:183-5. Available from: http://www.cancerjournal.net/text.asp?2013/9/7/181/122522
| > Introduction|| |
Pheochromocytoma is a tumor derived from chromaffin cells. Every incidentally discovered adrenal mass should be investigated for pheochromocytoma since it accounts for about 20% of the adrenal mass  and approximately 10% of it is malignant.  It can metastasize to lymph nodes, bone, liver, lung, etc., Pheochromocytoma can synthesize and secrete catecholamine automatically. Its secretion is not regulated by the nervous system.
Functional malignant pheochromocytoma is rarely seen. The clinical manifestation are mainly associated with the catecholamine secreted by the tumor itself, which results in various cardiovascular and metabolic symptoms. The former is mainly manifested as continuous or intermittent high blood pressure and causes multiple organ damage. The latter is manifested as increased blood glucose or impaired glucose tolerance, increased basal metabolism, accelerated decomposition of fat, weight loss, etc.
Surgical resection can be carried out with minimal morbidity and mortality,  but resection of liver metastasis is difficult. One of challenges is that the blood pressure can dramatically change due to the increase of catecholamine while pressuring on the tumor. Sometimes, the metastatic tumor in liver parenchyma is located deep and it is hard to avoid pressing, which would make it dangerous to operate. At the same time, it is challenging to adjust the body fluid balance.
| > Case Report|| |
A 38-year-old female patient presented with the complaint of cardiopalmus for 3 months after 4 years of operation for malignant adrenal pheochromocytoma, and was therefore hospitalized.
History of her illness showed that the patient was admitted at the Department of Urinary Surgery in Tongji Hospital, Wuhan in February 2003 for paroxysmal headache. Results of her physical examination at that time showed her blood pressure to be 184/110 mmHg. Biochemical investigations showed K+ 3.2 mmol/l, plasma rennin activity (PRA) 0.87 ng/ml, angiotensin II 516 pg/ml, aldosterone (ALD) 728 pg/ml, catecholamine 530 nmol/ml, epinephrine 260 nmol/ml, norepinephrine 270 nmol/ml, and vanillylmandelic acid (VMA) 264 μmol/ml. Computed tomography (CT) image showed that a tumor was located in left kidney, measuring about 40 mm × 55 mm × 50 mm, with enhanced heterogenicity. It was suspected pheochromoctyoma after the CT scanning [Figure 1] and [Figure 2]. In December of the same year, the patient underwent left adrenal tumor resection. The pathologic diagnosis was adrenocortical carcinoma.
|Figure 1: CT image and surgical exploration showing multiple liver tumor metastases, with two tumors located in segments V and VI (Couinaud segment)|
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|Figure 2: CT image and surgical exploration showing the other two multiple liver tumor metastases located in segments IVa and VIII (Couinaud segment)|
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About 3 months before liver surgery, the patient started to experience paroxysmal chest tightness and palpitation. The symptoms are intermittent and they will relieve after a short rest. The, the attack occurred several times a day and gradually increased. The systolic blood pressure ranged from 57 mmHg to 194 mmHg and the diastolic pressure did from 88 mmHg to 112 mmHg. Primary adrenal tumor biopsy after consultation with our pathology department revealed left malignant adrenal pheochromocytoma. CT image also showed that the liver contained multiple lesions that were considered metastases. Clinical diagnosis was liver metastasis of functional malignant adrenal pheochromocytoma. We confirmed that the original adrenal focus areas had no tumor recurrence and no metastasis in other organs. Then, we removed the liver metastasis of functional malignant adrenal pheochromocytoma successfully as follows.
Before the operation, blood pressure were well controlled and the body fluid was satisfyingly sustained. We gave the patient anti-angiotensin drug as hypotensor and injected her aseptic glucose and normal saline 2000 ml/day for 7 days.
Soft texture of the liver was without cirrhosis. Multiple liver tumor metastases were detected, two located in the segments IVa and VIII and the other two located in segments V and VI (Couinaud segment). The four tumors had similar size measuring 4 cm × 3 cm. When respectively squeezing the surface of tumors, the patient's blood pressure started increasing rapidly.
The postoperative pathological study conformed a diagnose of the metastatic pheochromocytoma in the liver [Figure 3].
|Figure 3: Hematoxylin– Eosin (HE) staining of tumor biopsy specimen of liver metastasis: The cells were polygonal and arranged as closely packed nests. Tumor cells had moderate to abundant granular eosinophilic cytoplasm with a small, round, and uniform nucleus and evenly dispersed chromatin. Cytoblastema was acidophilic, the nucleus was circle or oval, and chromatin was exquisite|
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Occlusion of the hepatic portal vessel was done twice for 15 min. The two metastasis tumors located in segments V and VI were resected in the first 15 min occlusion. The other two metastasis tumors located in segments IVa and VIII were resected in the second occlusion. The gallbladder was also resected together because the tumor in segment IVb was near the gallbladder. It is worth mentioning that we did not touch the tumors during the operation, and therefore, the blood pressure was maintained in the patient.
Postoperative pathologic diagnosis was liver metastasis of malignant pheochromocytoma [Figure 3].
The patient was followed up and monitored by close surveillance for 5 years and 8 months. No recurrence and other metastasis were found.
| > Discussion|| |
Among the cancers arising in most of the organs, malignant pheochromocytoma, especially functional malignant pheochromocytoma is rare as only less than 1% incidence has been reported.  The clinical manifestations are mainly associated with catecholamine, with cardiovascular and metabolic disorder symptoms. The former is mainly manifested as continuous or intermittent high blood pressure and causes multiple organ damage, and the latter is manifested as increased blood glucose or impaired glucose tolerance, increased basal metabolism, accelerated decomposition of fat, weight loss, etc., Generally, systemic therapies for treating patients with recurrent or metastatic disease have been ineffective. Radiation therapy is mainly applied for palliative relief of pain associated with bony metastases.  Local control of metastatic pheochromocytoma with percutaneous ablation can play an important role in disease management when the lesions are unresectable surgically and there is potential for prolongation of patient function or amelioration of metastasis-related symptoms.  Morikawaon reported one case of liver metastasis of malignant pheochromocytoma 10 years after adrenocortical pheochromocytoma, who underwent surgical treatment.  The patient lived in good health after right half hepatectomy. A literature review revealed that there are no other reports concerning this topic and no one else reported on the prognosis of the treatment of surgery, especially.
Given the limited experience in surgical operation for liver metastasis of functional malignant pheochromocytoma, preoperative stage must be carefully prepared.
Proper preoperational handling is the first step in reducing the risk of surgery. At first, we should know the pathophysiological features of low blood volume hypertension. Through proper preparation, we can minimize the risk of surgery by including diastolic blood vessels, expansion of blood volume, correcting the possible arrhythmias, maintenance of electrolyte imbalance, adjustment of blood glucose, and the psychological preparation for the operation.
Risks in the course of operation occur during the induction of anesthesia, in the course of cancer treatment, and in the process of blocking tumor blood supply. The key measurements in the monitoring of blood pressure for the sudden changes include radial artery puncture to measure the blood pressure instantaneously and effective and sufficient blood recyling. At the same time, this puncture channel can also be used to quickly administer vasoactive drugs, anti-hypertensive drugs, crystals and colloidal solutions, etc.
Hepatic metastatic tumors' surgical resection
Because the metastatic lesions presented in both the left and right hepatic lobe, we could only resect the tumor itself and the 1-2 cm normal liver tissues surrounding the tumor and could not perform standard left or right half hepatectomy. The liver tumor resection would also have a strong stimulation or oppression effect on the tumor. In the exploration and full analysis, we decided to cut off the tumors located in segments V and VI which could be easily resected at first as they were relatively fully exposed. We then dissected the secondary portal of liver and resected the tumors located in segments IV and VIII.
It is inevitable to avoid squeezing the tumor during the surgical operation. Pressing of liver metastatic malignant pheochromocytoma can easily lead to secretion of large amount of catecholamine into blood, which could result in sharp fluctuation of hemodynamics. Hence, every effort should be taken to minimize squeezing the tumors. When the blood pressure surges or becomes abnormal, the procedure should be suspended. Only when blood pressure is stable or arrhythmias are corrected, the operation should continue. In addition, liver cancer is located close to the hepatic great vessels; so, liver section is prone to massive bleeding. Injury to hepatic great vessels should be avoided in the course of the operation.
| > Conclusions|| |
may be the only means to cure functional liver metastases of malignant pheochromocytoma. Carefully controlling the blood pressure and rectifying relative body fluid insufficiency are the two important goals to be achieved in the patients before and during operation. It is particularly critical to detect the location and characteristics of each liver tumor, try the best to avoid direct stimulation of the tumor, and then minimize the time of tumor resection. If efforts in this direction are properly taken, functional liver metastases of malignant pheochromocytoma may be resected and the patient may recover well.
| > References|| |
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[Figure 1], [Figure 2], [Figure 3]