Clinical Analysis Of 168 Cases Of Pheochromocytoma

Objectives Our investigation analyzes retrospectively the 168 cases suffered from pheochromocytoma treated by open or laparoscopic surgery in West China Hospital of Sichuan University from June 1996 to June 2006, and in combination with the follow-ups of these cases. Through studying the factor such as epidemiology, clinical manifestation, diagnosis technique, surgical technique and prognosis outcomes, to summarize the clinic characteristics of pheochromocytoma, discuss the safety and current indications of laparoscopic resection of pheocromocytoma, and improve the diagnosis and treatment ofpheochromocytoma.Patients and methods A 10-year retrospective review (1996.6-2006.6) was conducted of all cases with pheochromocytoma treated by open or laparoscopic surgery. Pheochromocytoma was revealed in all cases by histologic evaluation. The follow-up of all cases was investigated with detailed tables through letters and/or telephones.Results(1) Data delineationIn all, there were 168 cases (164 patients) treated for pheochromoctoma by open or laparoscopic surgery. There were 73 men and 95 women. The average age was 40.14±14.13 years(range, 9-69 years). Mean hospital stay was 23.98±10.26 days. In all 168 cases, there were 10 cases with recurrent pheochromocytoma. Two patients were affected by the MEA II a syndrome and one patient were affected by von Hipple-Lindau disease.Pheochromocytoma has a highly variable clinic presentation. 112 cases had hypertension (66.67%) , in these cases there were 64 cases with paroxysmal hypertension and 48 cases with sustained hypertension. There were 30 cases had hypertensive crisis. 5 cases had acute heart failure, and 15 cases had obvious orthostatic hypotension. The other symptom were headache/dizzy, palpitation/tachycardia, sweating, abdominal discomfort, hyperglycemia etc. There were also 42 asymptomatic cases.The sensitivity was 90.37% for plasma norepinephrine, 56.30% for plasma epinephrine, 94.07% for plasma catecholamine, 76.00% for urine norepinephrine, 36.00% for urine epinephrine, 92.00% for urine catecholamine, 91.67% for 24-hours urine normetanephrine, 37.50% for 24-hours urine Metanephrine, 100% for 24-hours urine Metanephrines, 55.56% for urine vanillylmandelic acid.The completely coincidence was 92.97% for ultrasonography, 93.71% for computerized tomography, 100% for magnetic resonance imagine, 75% for ¹³¹I-MIBG SPECT.Alpha-adrenergic blockade was used in 146 cases. For patients with a coexisting tachycardia, beta-blockade was added. 70 cases were infused with colloid and/or crystalloid solution for intravascular volume expansion during the 24 to 48 hours prior to surgery.In these cases, there were 136 adrenal pheochromocytoma, 29 extraadrenal pheochromocytoma, 3 adrenal pheochromocytoma coexist with extra-adrenal pheochromocytoma. 152 cases were sporadic pheochromocytoma and the else 16 pheochromocytoma were multifocal.159 surgeries were made by open technique, in these cases 91 were transperitoneal and 68 were retroperitoneal. The operative incision included: 11th-rib transcostal incision, 12th-rib transcostal incision, subcostal incision, median abdominal incision, transrectal incision, paramedian incision, thoraco-abdominal incision, abdominal transverse incision etc.The mean size of the lesions was 6.294±3.14 cm. The mean operative time was 159.35±80.37 minutes. The median estimated blood loss was 175 ml. The transfusion rate was 44.05%. Only one case required conversion to open surgery. The mean duration of drainage was 2.664±1.21 days. The mean hospital stay after surgery was 8.07±3.20 days.The total complication rate was 20.24%. The intraoperative complication rate was 11.90%. The postoperative complication rate was 8.33%.For our cohort of 164 patients, the mean followup for 102 patients was 42.64±30.97months, with a range of 7 to 144 months. 17 patients have persistent hypertension. 4 patients had hypertensive emergencies episodes. 2 patients had development of contralateral adrenal pheochromocytomas. 6 patients had recurred tumors. 7 patients had developed tumor metastasis and 3 patients were dead because of tumor metastasis.The 1 year metastasis-flee survival rate of malignant pheochromocytoma was 57.1%, and the 5 years metastasis-flee survival rate was 14.3%. The median metastasis-free survival time was 38 months.Until the end of our follow-up, in the 7 patients with malignant pheochromocytoma, 3 patients were dead because of the metastasis of the tumors. The 1 year survival rate was 85.7%, and the 5years survival rate was 57.1%. The median survival time was 60.86 months.(2) Data comparisonThe mean tumor size of laparoscopic surgery was smaller than open surgery (P＜0.05) .Laparoscopy was superior with regard to hospital stay (P＜0.05) .When comparing mean operative time, estimated blood loss, transfusion rate, transfusion volume, mean duration of drainage, mean duration of gastrointestinal tract recovery, total complication rate, intraoperative and postoperative complication rate, the difference were not statistically significant (P＞0.05)The mean tumor size of transperitoneal open surgery was larger than retroperitoneal open surgery and laparoscopic surgery (P＜0.05) .Most of the extraadrenal tumors were resected by transperitoneal open surgery. The mean operative time of retroperitoneal open surgery was shorter than transperitoneal open surgery and laparoscopic surgery (P＜0.05) .The mean duration of gastrointestinal tract recovery of transperitoneal open surgery was longer than retroperitoneal open surgery and laparoscopic surgery (P＜0.05) . The estimated blood loss, transfusion rate and transfusion volume of retroperitoneal open surgery were smaller than transperitoneal open surgery (P＜0.05) . When comparing mean duration of drainage, total complication rate, intraoperative and postoperative complication rate, the difference among the 3 groups were not statistically significant (P＞0.05) .There was no difference of usage of intraoperative vasoactive agents between laparoscopic surgery and open surgery. Mean heart rate was significantly higher in open surgery group at tumor dissection, but was no difference during preoperation, anesthetic intubation, tumor excision, postoperation. The arterial systolic and diastolic blood pressures were no difference during preoperation, anesthetic intubation, tumor dissection, tumor excision, and postoperation. The incidence of intraoperative hemodynamic instability was also no difference between the two groups.The mean tumor size of transperitoneal open adrenal surgery was larger than retroperitoneal open adrenal surgery and laparoscopic surgery (P＜0.05).The mean operative time of retroperitoneal open adrenal surgery was shorter than transperitoneal open adrenal surgery and laparoscopic surgery (P＜0.05).The estimated blood loss, transfusion volume and mean duration of drainage of retroperitoneal open adrenal surgery was superior to transperitoneal open adrenal surgery (P＜0.05) , but there was no statistically significant between the other groups (P＞0.05) . The mean duration of gastrointestinal tract recovery of transperitoneal open adrenal surgery was longer than the other groups (P＜0.05) . The post surgery hospital stay of transperitoneal open adrenal surgery was longer than laparoscopic surgery (P＜0.05) , but there was no statistically significant between the other groups (P＞0.05) .Larger tumors required significantly longer operative time, more estimated blood loss, higher transfusion rate and transfusion volume P＜0.05). When comparing duration of drainage, duration of gastrointestinal tract recovery, post surgery hospital stay, total complication rate, intraoperative and postoperative complication rate, the difference were not statistically significant (P＞0.05) .There was no difference of usage of intraoperative vasoactive agents between the two groups of different tumor size. The hemodynamic parameters were also no difference at the time of pre-operation, anesthetic intubation, tumor dissection, tumor excision and post-operation. The incidence of tachycardia was higher in larger tumor size group, but the incidence of hypertensive emergencies and hypotension were no difference in the two groups.Extraadrenal pheochromocytoma had higher possibility of development of malignant tumors than adrenal pheochromocytoma. The incidence of local pain is higher in malignant tumors. The other clinic manifestation is no difference between the malignant and benign tumors. There was also no difference in the results of various kinds of biochemical detections, but the only exception was the concentration of plasmas norepinephrine which was higher in malignant tumor groups.Conclusion1. Pheochromocytomas are rare neuroendocrine tumors. Parts of the pheochromocytomas have been linked to the hereditary neoplastic syndrome, so if possible, all the patients with pheochromocytoma should undergo routine genetic mutation screening.2. Clinical presentation of pheochromocytoma can vary greatly, with similar signs and symptoms produced by many other clinical conditions. Most but not all the clinical signs and symptoms of pheochromocytoma are due to the direct actions of secreted catecholamines. There also many cases are lack of the symptoms caused by secreted catecholamines. Therefore, the diagnosis of pheochromocytoma can not be made only according to the clinical symptoms and signs.3. Most of pheochromocytomas are located in unilateral adrenal gland. The incidence of malignant pheochromocytoma is higher in extraadrenal lesions.4. Establishing the diagnosis of a pheochromocytoma requires biochemical evidence of catecholamine overproduction. In the various biochemical analysis methods adopted in our hospital: the 24-hours urinary outputs of metanephrines are the most sensitive method, the sensitivity of the urine and plasma catecholamines are similar, the urine VMA analysis is the least sensitive method.5. The tumor localization by imaging procedures has an important value for the choice of surgical approach. USG and CT scanning are the most preferred methods. For extraadrenal lesions, MRI scanning and ¹³¹I-MIBG SPECT have an advantage of USG and CT scanning. ¹³¹I-MIBG SPECT has important value in screening the ectopic pheochromocytomas, multiple lesions and in follow-ups.6. There is no significant difference in patient demographics, clinical symptoms and signs, biochemical analysis outcomes and histopathologic evaluation outcomes between malignant and benign pheochromocytomas, therefore to establish a diagnosis of malignant pheochromocytoma relied on long-term follow-ups.7. Most of the small unilateral adrenal pheochromocytomas are resected by retroperitoneal approach. And most of the large and/or extraadrenal pheochromocytomas are resected by transperitoneal approach. 8. In proper selected patients, LA is another choice other than open surgery, but its long-term oncological outcomes still need further evaluation.9. It is very important to think highly of the health instructions and follow-ups of the patients with pheochromocytoma, in order to promote the patients' health awareness of themselves.