后腹腔镜手术治疗嗜铬细胞瘤46例临床观察

采用后腹腔镜手术治疗肾上腺嗜铬细胞瘤45例（双侧2例）,腹主动脉旁嗜铬细胞瘤1例。结果46例患者行后腹腔镜手术48次,47次取得成功,1例因术中出血改行开放手术。认为后腹腔镜手术切除嗜铬细胞瘤安全有效,应成为医师首选方法。     

腹腔镜手术治疗肾上腺嗜铬细胞瘤(附24例报告)

目的评价腹腔镜手术治疗肾上腺嗜铬细胞瘤的安全性及临床价值。方法对24例肾上腺嗜铬细胞瘤患者采用腹腔镜经腹腔途径手术治疗。结果23例手术取得成功，1例因粘连严重、术中出血改行开放手术。肿瘤最大径2．5～8cm，平均4．2cm；手术时间30～150min，平均70min；术中出血量20～200ml，平均50ml，均未输血；术后住院5～10d，平均6．8d；术后病理检查证实均为肾上腺嗜铬细胞瘤。全部病例获得随访3～18个．月，平均10个月，患者血压正常，B超或CT复查均未见肿瘤复发。结论对于直径〈6cm的肾上腺嗜铬细胞瘤，腹腔镜手术具有手术时间短、出血少、损伤小、恢复快、安全性高、并发症少等优点，可替代开放手术。     

嗜铬细胞瘤的诊治

一例34岁男性因波动性高血压疑为嗜铬细胞瘤而转至本科就诊。该患者临床表现典型,人院查血浆甲氧基肾上腺素(MN)及甲氧基去甲肾上腺素(NMN)均明显升高,CT和PET-CT检查均发现左侧肾上腺占位,诊断为嗜铬细胞瘤。术前予以甲磺酸多沙唑嗪控释片4 mg/d口服2周,血压平稳,在腹腔镜下行左侧肾上腺肿瘤切除术。术后病理提示嗜铬细胞瘤,MN、NMN、血压恢复正常,症状缓解。该患者术后随访3年,血压、血浆MN、NMN水平均正常,肾上腺CT未见肿瘤复发。     

肾上腺外嗜铬细胞瘤25例临床分析

目的 分析肾上腺外嗜铬细胞瘤的发病情况，病理特征及诊断方法，探讨治疗措施。方法 通过对81例经手术与病理证实的嗜铬细胞瘤进行回顾性分析。结果 81例嗜铬细胞瘤患者中，经手术与病理证实的肾上腺外嗜铬细胞瘤25例，占同期收治嗜铬细胞瘤的30．9％。病人的临床表现除特殊部位肿瘤外与肾上腺髓质嗜铬细胞瘤相似。结论 肾上腺外嗜铬细胞瘤临床表现与生化诊断与肾上腺髓质嗜铬细胞瘤相似，其恶性者较肾上腺髓质嗜铬细胞瘤明显增多，定位诊断以^131I-MIBG为特异，治疗以手术最佳。     

197例嗜铬细胞瘤临床分析

目的提高嗜铬细胞瘤的诊治水平。方法回顾性分析郑州大学第一附属医院1986～2005年所有病理诊断为嗜铬细胞瘤的197例患者的临床资料。结果197例患者均经手术治疗。良性嗜铬细胞瘤184例,恶性13例;肾上腺原发肿瘤171例,异位嗜铬细胞瘤24例,多发内分泌腺瘤（MEN）Ⅱ（嗜铬细胞瘤伴甲状腺髓样癌）2例。结论完善检查手段可提高嗜铬细胞瘤的检出率,确诊需病理检查,手术是根本的治疗方法。     

单纯家族性嗜铬细胞瘤一家系报告并文献复习

目的 探讨单纯家族性嗜铬细胞瘤的遗传和临床特征.方法 回顾分析1例单纯家族性嗜铬细胞瘤青少年患者的临床资料,并调查该家系发病情况,绘制家族发病图谱.结果 该家系三代人中有3例嗜铬细胞瘤,均在青少年期发病,发病部位均为肾上腺.均采用肾上腺肿物切除术治疗.1例术后发生对侧肿瘤复发,病理诊断均为良性嗜铬细胞瘤,随访1～29年无复发.结论 单纯家族性嗜铬细胞瘤符合常染色体显性遗传特点,多在青少年期发病.对青少年疑似患者进行候选基因突变检测有助于该病的早期诊断.     

血管介入结合手术切除巨大无症状嗜铬细胞瘤1例

无症状嗜铬细胞瘤约占肾上腺嗜铬细胞瘤的10%-17%,由于缺乏特殊的临床症状和体征,其定性及确定组织来源均比较困难,加之其往往有丰富的血供和供血来源极不规则,因此手术切除难度大.我院1例患者因反复右上腹隐痛伴腹胀2年,加重7d于2011-10-10入院.术前诊断为右上腹巨大肿瘤,于2011-10-18行血管介入结合手术切除,术后病理诊断为:嗜铬细胞瘤.     

以少见表现为首发症状的9例嗜铬细胞瘤临床分析

目的：探讨以少见表现为首发症状的的嗜铬细胞瘤的临床特点.方法：回顾性分析经手术病理证实为嗜铬细胞瘤并具有特殊临床表现的9例患者的临床资料结果：2例患者术前诊断为急性冠脉综合征;2例患者术前诊断休克;1例患者术前诊断为糖尿病酮症酸中毒;4例患者术前诊断为视神经炎.所有患者均有高血压.影像学检查均发现肾上腺占位病变,均经手术病理证实为肾上腺嗜铬细胞瘤,术后症状好转.结论：嗜铬细胞瘤表现多样化,高血压是重要的诊断线索,实验室及影像学检查对明确诊断有重要意义.     

31例嗜铬细胞瘤手术的麻醉及围手术期管理

目的探讨嗜铬细胞瘤围手术期治疗安全性。方法对31例嗜铬细胞瘤的围手术期治疗结果进行总结,分析其术前、术中及术后情况。结果28例嗜铬细胞瘤术前准备充分,3例术中发现为嗜铬细胞瘤,全部病例手术经过顺利,术后恢复良好。结论切除肿瘤是治疗嗜铬细胞瘤的根本方法。①充分的术前准备,包括控制血压、扩容、纠正心律失常,改善一般情况。②合理麻醉方式;严密的术中监测及管理是手术治疗成功的重点。③术后密切监测。并提出术前术中处理较术后处理更重要。     

嗜铬细胞瘤20例临床分析

目的：探讨嗜铬细胞瘤临床特点，提高诊治水平。方法：对20例嗜铬细胞瘤的临床资料总结分析。结果：20例均经手术治疗，单侧17例，家族性双侧3例，恶性嗜铬细胞瘤4例，肾上腺外嗜铬细胞瘤1例，无症状嗜铬细胞瘤4例。结论：嗜铬细胞瘤典型表现是高血压、头痛、心悸和出汗，而无症状性、家族性和其他非典型的特殊表现需引起注意。儿茶酚胺及影像学检查是主要的诊断手段。     

Laparoscopic adrenalectomy in pheochromocytomas

BACKGROUND: The aim of this study was to evaluate 17 patients undergoing laparoscopic adrenalectomy for the treatment of pheochromocytoma by transperitoneal anterior approach. METHODS: Seventeen patients underwent laparoscopic adrenalectomy for pheochromocytoma between January 1994 and May 2002. Ten females (58.8%) and 7 males (41.2%) were operated on; 14 patients (82.3%) had sporadic pheochromocytoma and 3 (17.7%) were familiar cases. Mean age was 42 yr (range 25-72 yr). All patients were treated pre-operatively with alpha-blockers. Seven patients (41.2%) underwent right adrenalectomy; 9 (52.9%) underwent left adrenalectomy and 1 (5.9%) bilateral adrenalectomy. RESULTS: No conversion to open surgery occurred and no mortality was observed. The right-side adrenalectomy required a mean operative time of 86 min (range 45-120), the left-side procedure a mean operative time of 116 min (range 80-140) and the bilateral one 219 min. In two patients (11.8%), a laparoscopic cholecystectomy and ovariectomy, respectively, were performed without changing the position of the patient on the operating table. Only 1 patient (5.9%) presented significant intraoperative hypertension, and arrhythmia resolved by medical therapy. No other intraoperative and post-operative complications were reported. Mean hospital stay was 3 days (range 2-8 days). At mean follow-up of 48 months (range 6-96 months), regression of symptoms and control of blood pressure were obtained without additional treatment in all patients. No recurrences were reported. CONCLUSION: In our experience, adrenal pheochromocytoma can be treated safely and effectively by laparoscopic transperitoneal anterior approach.     

Factors associated with perioperative morbidity and mortality in patients with pheochromocytoma: analysis of 165 operations at a single center

To identify preoperative factors associated with 30-day morbidity and mortality after pheochromocytoma surgery, we carried out an external review of the records of all patients undergoing pheochromocytoma surgery from 1975 to 1997 at a single center. One hundred and forty-seven patients, including 23 with malignant tumors at the time of the first operation, underwent 165 operations. Death, resection of a neighboring organ, further surgery, secondary transfer to an intensive care unit, and any events associated with a surgical stay exceeding 10 days were defined as complications. Mortality and morbidity were 4 of 165 (2.4%) and 38 of 161 (23.6%), respectively. Morbidity included 13 spleen resections and hematomas. Spleen complications were not related to tumor location, but were probably due to the operative strategy used, a transperitoneal complete abdominal exploration including both adrenal glands. Complications were independently associated with preoperative systolic blood pressure [odds ratio (OR), 1.14/cm Hg], urinary metanephrine excretion (OR, 1.18/10 micromol x day), and with the number of operations (repeat vs. first operation OR, 5.36). In conclusion, pheochromocytoma resection consistently involves a risk of complications. Spleen damage should be prevented by complete preoperative localization studies and an elective or laparoscopic surgical approach. Careful blood pressure control should help prevent complications. Patients with high secretion tumors and those undergoing repeat intervention are at high risk of complications and should be referred to centers familiar with pheochromocytoma management.     

Chronological variation of adrenal surgery for the past 28 years--experience with 140 adrenal operations

The author experienced 140 adrenal operations from April, 1958 to March, 1986. Surgery was performed in 54 cases of primary aldosteronism (adenoma - 52, hyperplasia - 2), 46 cases of Cushing's syndrome (carcinoma - 3, adenoma - 22, primary nodular hyperplasia - 1, hyperplasia - 20), 26 cases of pheochromocytoma (tumor - 25, hyperplasia - 1) and 11 other adrenal tumors. During the study period of 28 years, remarkable strides have been made in the image diagnostic procedures such as RI scinti-scan, ultrasonography and CT and the hormonal assay. The effect of this progress appeared mainly in the cases after 1972. Therefore, the 140 adrenal operations were studied by dividing the period into the early 14 and the late 14 years. During the period of the early 14 years, 77 operations were carried out, while 63 were performed in the late period. The decreased number of operations during the late 14 years was due to the fact that Cushing's disease came to be treated mostly by transsphenoidal hypophysectomy. Surgical approaches to the adrenal gland are usually classified as anterior transperitoneal and extraperitoneal including translumbar and posterior incisions. In the former period, the transperitoneal approach was indicated in 40 operations, while the extraperitoneal approach was indicated in 37. On the other hand, 53 operations were performed by the extraperitoneal procedure with only 10 transperitoneal surgical interventions in the latter period. The decreased frequency of the transperitoneal surgery in the latter period was considered due to improved preoperative techniques to localize the adrenal lesion. Operative and postoperative complications such as spleen injury, delayed wound healing and intestinal paralysis were compared with regards to the transperitoneal and extraperitoneal approaches. More complications were noted in the transperitoneal approach.(ABSTRACT TRUNCATED AT 250 WORDS)     

Childhood familial pheochromocytoma. Conflicting results of localization techniques

Childhood familial pheochromocytoma was investigated in four patients by abdominal computed tomographic scan, [131I]metaiodobenzylguanidine scan, and vena caval catecholamine sampling. Results conflicted with surgical findings. Computed tomographic scan identified all four adrenal tumors but missed two midline tumors in one patient. [131I]metaiodobenzylguanidine scan identified two of three adrenal tumors but also suggested extra-adrenal tumors not confirmed at operation in two of three patients. Vena caval sampling for catecholamines confirmed all adrenal tumors but suggested additional tumors not verified at operation in two of three patients. All patients are asymptomatic and have normal urinary catecholamines 15 to 51 months after operation. Because of the frequency of multiple tumors in familial pheochromocytoma, different diagnostic techniques were employed. False-positive results were more frequent with [131I]metaiodobenzylguanidine and vena caval sampling. Reinterpretation of the [131I]metaiodobenzylguanidine scans at a later date led to less false-positive interpretation, although the false-negative rate remained unchanged. More pediatric experience with [131I]metaiodobenzylguanidine scans and vena caval sampling in familial pheochromocytoma is needed. Confirmation of tumor and its localization rest with meticulous surgical exploration.     

Retroperitoneal laparoscopic adrenalectomy in a pregnant woman presenting MEN2a with a pheochromocytoma: case report and review of the literature

The diagnosis of pheochromocytoma during pregnancy is uncommon and is at high risk for both mother and baby. We report the case of a 22-year-old woman with MEN2a (mutation C634Y in exon 11 of RET) who had undergone surgery for medullary carcinoma of the thyroid and hyperparathyroidism when she was 18. She was asymptomatic when she was seen at 22 weeks of gestation because of increased urinary metanephrine levels. A 24-h blood pressure monitoring was normal. Abdominal magnetic resonance imaging (MRI) revealed a right-sided, 34x31x28mm, well-limited, adrenal mass with high signal intensity on T2-weighted images; the contralateral adrenal was normal. At 26 weeks of gestation and after an adequate labetalol preparation, a retroperitoneal laparoscopic right-sided adrenalectomy was performed without maternal or foetal complications. Pathohistological examination confirmed the presence of a 3cm pheochromocytoma in the right adrenal gland, with no sign of malignancy. The levels of urinary methoxylated metabolites were normal two months after surgery. The pregnancy progressed normally and the patient delivered a healthy child without complications. In conclusion, firstly, all MEN2a women should be screened for a pheochromocytoma with a 24-h urinary metanephrine and normetanephrine evaluation before or early during pregnancy, even with normal blood pressure; secondly, pheochromocytoma diagnosed during pregnancy should be operated on during pregnancy because of the risks for both mother and baby; thirdly, after medical therapy, retroperitoneal laparoscopic adrenalectomy can be performed during the second trimester of pregnancy.     

Adrenal incidentaloma: surgical update

Nowadays, the role of surgery in the treatment of adrenal incidentalomas (AI), considering their biologic behavior, is still debated. Surgery is mandatory in cases of hyperfunctioning adrenal masses, in the presence of suspect radiological malignancy, in cases of discordant computed tomography (CT) and scintigraphy findings and when the maximum diameter is 4 cm or more. On the other hand, studies have suggested relative inaccuracy of conventional CT in evaluating the size. The aim of this paper was to evaluate the safety and effectiveness of laparoscopic adrenalectomy (LA) in the treatment of AI by reviewing our experience. Over the period from 1995 to 2005 we laparoscopically managed 78 AI by anterior transperitoneal approach. Two LA (2.6%) were converted to open surgery. Neither intra- nor post-operative major complications were observed. The mean size of lesions was 5.5 cm (range 3-9). Twenty-one large adrenal lesions (exceeding 6 cm) were removed (27%). Definitive histology resulted as follows: adrenocortical adenoma (63), pheochromocytoma (5), nodular hyperplasia (4), myelolipoma (3), cysts (2), and adrenocortical carcinoma (1, with a size of 3 cm). The patients were followed-up by hormonal and radiological evaluation every 12 months (6 for malignancy); their follow-up (median 60.4 months, range 6-123) was uneventful. Also larger AI were treated safely. Laparoscopy has been safe and effective in the treatment of AI in our experience, according to specific literature.     

Partial adrenalectomy for pheochromocytoma with maintenance of adrenocortical function

A 30-yr-old woman with malignant hypertension was found to have biochemical evidence of pheochromocytoma. Bilateral adrenal tumors were demonstrated on computerized tomographic scanning and confirmed at the time of surgery. Complete removal of one adrenal gland with partial removal of the other adrenal gland resulted in normalization of her blood pressure and biochemical parameters (norepinephrine and epinephrine) with preservation of adrenocortical function.     

Periodic fluctuation of blood pressure and its management in a patient with pheochromocytoma. Case report and review of the literature

The case of a 69 year old man with a right adrenal pheochromocytoma who manifested cyclic fluctuations of blood pressure with a cycle length of 9 to 13 min is reported. We collected and reviewed 14 similar cases previously reported in the literature. In these cases, right adrenal pheochromocytoma was most common, while 1 case involved the left adrenal and 2 cases were of extra-adrenal origin. The incidence in males was twice that in females and the median age was 45.3 years. Although a good correlation between the blood pressure and plasma norepinephrine concentrations was observed in our patient, the exact mechanism for the cyclic fluctuations of blood pressure is not known. In our patient, both YM-09538 and bunazosin were effective in controlling severe hypertension preoperatively. YM-09538 induced significant increases in urinary norepinephrine concentrations (1327 +/- 238 micrograms/day), while bunazosin induced a significant decrement in urinary norepinephrine concentrations (475 +/- 188 micrograms/day) compared with pretreatment levels (900 +/- 42 micrograms/day) (p less than 0.01). These observations indicated that bunazosin as a postsynaptic alpha-adrenergic receptor blocker interfered with release of norepinephrine from the tumor and thus might be beneficial in the management of elevated blood pressure in patients with pheochromocytoma.     

Bilateral adrenalectomy for Cushing's syndrome: a comparison between laparoscopy and open surgery

We report our experience with bilateral adrenalectomy for treatment of Cushing's syndrome and we compare the outcome of laparoscopy with open surgery in terms of effectiveness and safety. A series of 23 patients underwent bilateral adrenalectomy for treatment of Cushing's syndrome [Cushing's disease in 16, ectopic ACTH syndrome in 2, and ACTH-independent macronodular adrenal hyperplasia (AIMAH) in 5 cases]. From 1993 to 1996, all patients were treated using an open approach (Group A), while from 1997 all patients were treated using a transperitoneal laparoscopic approach (Group B). The comparison between the 2 groups was performed considering patients characteristics, operative times, blood losses, intraoperative and post-operative complications, analgesic consumption, post-operative hospital stay and recovery. Open surgery was performed in 10 patients and laparoscopy in 13 patients. No significant difference was recorded between the two groups as to patients' characteristics and complications. Mean operative time was significantly increased in Group B, while post-operative hospital stay was significantly longer in Group A. Laparoscopic bilateral adrenalectomy can be safely and effectively employed to treat Cushing's syndrome. However, long operatives times may represent a limitation especially in high risk patients.