Surgical treatment of endocrine hypertension experience in India

Between 1980 and 1994, 162 cases of endocrine based hypertension were diagnosed and treated surgically. Seventy-nine cases (48.7%) of phaeochromocytoma, 63 cases (38.8%) of Cushing's syndrome, and 20 cases (12.3%) of Conn's syndrome were diagnosed. In phaeochromocytoma 75% of the tumours arose from the adrenal glands and 25% arose from the extra-adrenal sites. Cushing's syndrome was caused by adenoma (45%), diffuse bilateral adrenal hyperplasia (36%), pigmented macronodular hyperplasia (9%), and adrenal carcinoma (10%). The most common cause of Conn's syndrome was adenoma (95%) which arose mainly from the left adrenal gland (60%). In the present series the success rate of surgical treatment was 100% for phaeochromocytoma, 90% for Cushing's syndrome and 96% for Conn's syndrome. Trucut biopsy of the kidneys of these patients showed hypertensive changes, the moderate hypertension could be due to renal damage.     

Renal cell carcinoma in a von Hippel-Lindau syndrome: when should phaeochromocytoma be anticipated?

A 39-year-old man was diagnosed with von Hippel-Lindau syndrome, which was associated with retinal haemangioblastoma, cervical cord haemangioblastoma and bilateral renal cell carcinoma. He subsequently underwent an arterial embolisation and cervical laminectomy, following a spinal angiogram of the cervical lesion. He also had a right radical nephrectomy, with no perioperative complications. However, on admission for the left radical nephrectomy, he was noted to have preoperative hypertension. Further investigation revealed an enlarged left adrenal gland on abdominal computed tomography scan and raised urinary catecholamines. We discuss the risk of renal cell carcinoma and phaeochromocytoma arising concomitantly in von Hippel-Lindau syndrome, and how best to investigate and manage them.     

Multiple endocrine neoplasia type 2b: long-term follow-up of a case

Multiple endocrine neoplasia type 2b is a rare inherited syndrome which comprises the association of medullary thyroid carcinoma, phaeochromocytoma, widespread neuromatous proliferation and a characteristic body habitus. In this report we present the late clinical course and autopsy findings of the first patient with this syndrome described in Australia. At presentation she was found to have a right adrenal phaeochromocytoma and medullary thyroid carcinoma which were resected in separate operations. No clinical or biochemical evidence of residual medullary thyroid carcinoma was identified in life. However, in spite of serial vanillylmandelic acid estimations, which showed normal or only mildly elevated levels, and normal results of urinary catecholamine studies, a left adrenal phaeochromocytoma was identified in a metaiodobenzylguanidine (MIBG) study performed 14 years after presentation. Her late clinical course was dominated by progressive dysphagia, intestinal dysmotility and megacolon associated with unrelenting malnutrition. After her death due to an intracerebral haemorrhage, an autopsy confirmed the presence of a left adrenal phaeochromocytoma and revealed diffuse intestinal ganglioneuromatosis to be the cause of her intestinal dysmotility. No residual medullary thyroid carcinoma was found. This case emphasises the propensity for multiple endocrine tumours in these patients and highlights the potentially significant role of intestinal ganglioneuromatosis in the natural history of this condition.     

胎儿肾和肾上腺重的研究

研究目的提供河南地区胎儿发育的数据资料,探讨胎儿体重与肾上腺重和肾重间的关系。研究背景不同地区、不同民族胎儿发育有一定差异。现有文献中,未见河南地区胎儿此方面的资料。研究方法水囊引产胎儿娩出后速测体重、肾重、肾上腺重。测重数据用微机作统计学处理。研究结果肾重、肾上腺重与体重、胎龄有极显著的正相关关系。相关系数均在0.98以上。回归方程:(?)(肾重)=0.4958+0.0030x(x-体重)、(?)(肾上腺重)=0.3871+0.0012x(x-体重)、(?)(肾重)=0.1959e~(0.4003x)(x-胎龄)、(?)(肾上腺重)=0.1169e~(0.3658x)(x-胎龄)。肾重与肾上腺重也有极显著的正相关关系。回归方程:(?)(肾重)=-0.4104+2.4483x(x-肾上腺重)。研究结论肾重、肾上腺重与体重有很好的线性回归关系,与胎龄有很好的指数曲线回归关系;肾重与肾上腺重也有很好的线性回归关系。     

The impact of recent advances in diagnostic technology on the clinical presentation of phaeochromocytoma.

OBJECTIVE: To examine the impact of recent advances in diagnostic technology on the spectrum of clinical and biochemical features of patients presenting with a new diagnosis of phaeochromocytoma. DESIGN: A retrospective review of the clinical and biochemical features of patients diagnosed by our laboratory as having phaeochromocytoma within a 27-month period up to December, 1990. Noradrenaline, adrenaline and dihydroxyphenylglycol were assayed in 24-hour urine specimens (19 patients) or plasma (1 anuric patient) by gas chromatography/mass spectrometry. SETTING: A tertiary level chemical pathology department. PATIENTS: Twenty patients with a new diagnosis of phaeochromocytoma. RESULTS: The classic, episodic adrenergic symptoms traditionally associated with phaeochromocytoma were absent in 9 of the 20 patients (45%). "Atypical" phaeochromocytoma presented as a mass on computed tomography imaging (6 patients, 30%), "phaeochromocytoma crisis" (4 patients, 20%) or family screening (1 patient, 5%). Excessive adrenaline production was found in 11 patients (55%) and six (30%) had predominantly adrenaline-secreting tumours. The urinary noradrenaline:dihydroxyphenylglycol ratio was raised in all nine patients with predominantly noradrenaline-secreting tumours but was not raised in nine out of ten patients with adrenaline-secreting phaeochromocytoma. Adrenaline excretion was significantly correlated with tumour size (r = 0.8; P less than 0.05). CONCLUSIONS: Advances in diagnostic technology, particularly specific adrenaline assays and computed tomography, have made possible the early diagnosis of patients with phaeochromocytoma presenting in ways previously thought to be uncommon. All patients with adrenal masses noted incidentally on CT scan should be investigated for phaeochromocytoma. Adrenaline-secreting tumours are common and both noradrenaline and adrenaline should be assayed in all patients investigated for phaeochromocytoma.     

肾上腺畸胎瘤五例临床分析

目的 探讨肾上腺畸胎瘤的特点、诊治方法及预后.方法 回顾性分析2004年至2013年我院收治5例肾上腺畸胎瘤患者的临床资料.其中男2例,女3例.年龄35 ～64岁,中位年龄57岁.左侧3例、右侧2例.肿瘤最大径5.0～8.5 cm.常规查体发现2例,因乏力就诊发现2例,因腰疼就诊发现1例.5例患者内分泌检查均正常.超声检查示肾上腺区囊实性肿块,肿物为混杂回声.CT检查示肾上腺区不规则软组织密度肿物,其内密度欠均匀,可见钙化斑.MRI检查肾上腺不规则软组织信号肿块,其内可见多发长T1长T2信号.考虑肾上腺肿块伴囊变,神经节细胞瘤可能性大.结果 5例均行后腹腔镜下肾上腺切除术.肿块不规则,切面为灰褐色或灰白色,囊实性,囊液为黄色皮脂样物质或胶冻样物质,3例可见骨骼样物质.病理诊断为肾上腺成熟性畸胎瘤.随访3个月～9年,无复发及转移.结论 肾上腺畸胎瘤临床罕见,临床表现缺乏特异性,腹腔镜手术治疗效果良好.     

Adrenal sparing surgery for renal cell carcinoma

OBJECTIVE: To investigate the feasibility of adrenal sparing surgery for selected renal cell carcinoma (RCC), since the necessity of routine adrenalectomy during radical nephrectomy for RCC has been challenged in recent years. METHODS: 178 patients underwent perifascial nephrectomy in a 16-year period. Of these patients, 96 had ipsilateral adrenal gland preserved during nephrectomy and 82 underwent concomitant adrenalectomy. 75 excised adrenal specimens were examined pathologically. Disease specific survival rates were assessed according to the pathological stage of the tumors. RESULTS: Of the 75 patients, 53 presented a macroscopically normal adrenal gland without pathohistological changes. The other 22 patients were suspected to have adrenal metastasis intraoperatively, while only 5 of them were confirmed to have adrenal involvement by histopathology. Two patients in the adrenal gland preservation group developed ipsilateral adrenal recurrence and synchronous or metachronous contralateral adrenal metastasis during follow-up, although both were documented to have a normal-appearing adrenal gland intraoperatively. Five patients with adrenal metastasis and 2 patients with adrenal recurrence had large renal tumors. The survival difference among subgroups of patients undergoing adrenalectomy or with adrenal gland left in situ was not statistically significant. CONCLUSION: Adrenal sparing surgery could be done for patients with small renal tumors along with macroscopically normal ipsilateral adrenal glands.     

手术治疗的肾上腺疾病临床特征回顾分析

目的：分析总结手术治疗的肾上腺疾病的临床特点。方法：查阅四川大学华西医院2000-2007年入院行肾上腺手术患者的病案,对肾上腺疾病的临床特点进行回顾分析。结果：352例患者,最大径≤3 cm的肿瘤中良性肿瘤占99.2%。嗜铬细胞瘤患者患有高血压的比例为69.6%,具有特异性症状的比例是73.9%。原醛、库欣综合征以及嗜铬细胞瘤患者相关血生化检查阳性率为97.8%、97.3%、94.4%。结论：肾上腺疾病中醛固酮腺瘤的直径最小,不典型嗜铬细胞瘤是肾上腺偶发瘤鉴别诊断的重点     

肾上腺形态及其毗邻关系的薄层断层与CT断层解剖学研究

目的 阐明肾上腺在薄层断面和CT断面的周围结构毗邻关系，为肾上腺细小病变提供影像学诊断依据。方法 对中国首例可视化人体肾上腺部分的数据集进行总结和分析，观察肾上腺的形态及其周围毗邻结构。选择50例经螺旋CT扫描无肾上腺病变的断面图像与薄层断面对照分析。结果 首例中国可视化人体肾上腺部分从共计48层，每层厚度为1．0mm．每层断面所显示的肾上腺及其周围结构清晰，CT清晰显示肾上腺大小、形态及周围结构。结论 本文报道的首例中国可视化人体肾上腺部分为连续、完整的薄层断面标本，无节段性数据缺损，层厚1．0mm，厚度均匀，连续的薄层断面解剖学基础，肾上腺形态变化较大，肾上腺于前方结构毗邻变化较大．决定肾上腺病变向前生长时有不同的优势途径。     

肾上腺损伤的CT诊断

目的:探讨肾上腺损伤的CT表现和鉴别诊断。方法:CT检查肾上腺损伤22例,其中平扫22例,增强扫描12例,随访CT 17例;MRI检查1例,B超检查12例。CT检查外伤后最短时间2 h,最长达7 d,平均19.8 h。回顾性分析其CT表现及影像特点。结果:22例肾上腺损伤,单侧21例(右侧15例,左侧6例),双侧1例。CT表现为平扫肾上腺增大6例,边缘凸起,密度不均;肾上腺肿块16例,呈圆形或卵圆形均匀增高或混杂密度。肾上腺周围脂肪囊内见密度增高线条状影及高密度模糊影15例。同侧膈脚增厚11例。增强扫描12例中,呈不均匀强化4例,环状强化8例。随访CT显示肾上腺形态基本正常,其中3例肾上腺区肿块吸收、消失。结论:CT检查对肾上腺损伤诊断及鉴别诊断具有重要意义。     

大鼠含肾上腺种植体肾移植模型的建立及其对植肾保护作用的初步研究

目的建立含肾上腺种植体肾移植模型,探讨肾上腺种植体对植肾的保护作用。方法在成功制备含肾上腺种植体供肾的基础上,再行同种异体肾移植,实验动物分为Ⅰ组(含肾上腺种植体肾移植组)和Ⅱ组(单纯肾移植组),均未用任何免疫抑制剂,术后7d切除受体右肾(左侧为移植肾);观察右肾切除术后受体存活时间,检测肾上腺种植术和右肾切除术前、后不同时间点肾上腺种植体及植肾的形态学变化和血清皮质酮、肌酐和尿素氮浓度。结果肾上腺种植术后7周,肾上腺种植体成活良好并恢复内分泌功能;右肾切除术后7d,肾上腺种植体逐渐出现萎缩、变性和点状坏死,但Ⅰ组大鼠生存期10.2d±2.2d较Ⅱ组7.3d±1.2d明显延长,且前者植肾形态学观察和功能状态均明显优于后者。结论肾上腺种植体在移植术后早期对植肾具有保护作用。     

Transduodenal EUS-guided FNA of the right adrenal gland to diagnose lung cancer where percutaneous approach was not possible

Although endoscopic ultrasound-guided fine needle aspiration (EUS-guided FNA) of the left adrenal gland is safe and accurate compared to the percutaneous approach, there are no reports to our knowledge about EUS-guided FNA of the right adrenal gland performed in Lebanon and the Middle East. We report the case of a 64-year-old male who presented with a swollen right calf and right flank pain and was diagnosed with deep vein thrombosis with a right calf deep venous thrombosis. A computerized tomography of the chest and abdomen revealed a round solid mass of the right adrenal gland, a right upper lobe mass and centrilobular emphysema of both lungs. Percutaneous biopsy of the right adrenal gland was declined as the patient was quoted a high risk of bleeding. EUS-guided FNA of the right adrenal was performed via the transduodenal approach confirming the final diagnosis of metastatic lung cancer. This case shows that the right adrenal gland can be sampled with EUS-FNA via the duodenal approach to diagnose metastatic lung cancer, especially when the percutaneous approach is not feasible.     

胎儿静脉动脉化肾上腺异体移植的应用解剖

对40具7个月以上新鲜胎儿的左肾上腺静脉和左肾静脉的起始、走行、外径、长度及汇入部位进行了观测,以提供胎儿静脉动脉化肾上腺异体移植的有关形态学资料。基于大网膜呼吸能力较强和有适合吻合的动脉,认为其是理想的移植部位。     

Hypoglycaemia following removal of phaeochromocytoma: case report and review of the literature

Hypoglycaemia may complicate removal of a phaeochromocytoma, but its pathogenesis is not well understood. The sixth such case in the world literature is reported together with details of glucose tolerance testing and hormone levels before and after extirpation of an adrenal phaeochromocytoma. The mechanism of hypoglycaemia probably relates to a sudden decrease in circulating catecholamines which allows a rebound increase in insulin release and in peripheral glucose uptake. It is suggested that blood sugar levels should be monitored routinely after surgery for phaeochromocytoma.     

Recurrence of Phaeochromocytoma and Abdominal Paraganglioma After Initial Surgical Intervention

BackgroundClinical and biochemical follow up after surgery for phaeochromocytoma is essential with long term studies demonstrating recurrence frequencies between 6% and 23%.AimTo examine the characteristics and frequency of tumour recurrence in a regional endocrine referral centre, in patients with surgical resection of phaeochromocytoma (P) and abdominal paraganglioma (AP).MethodsWe identified a cohort of 52 consecutive patients who attended our Regional Endocrinology & Diabetes Centre and retrospectively reviewed their clinical, biochemical and radiological data (between 2002 and 2013). After confirmation of early post-operative remission by negative biochemical testing, tumour recurrence was defined by demonstration of catecholamine excess with confirmatory imaging.ResultsPhaeochromocytoma was confirmed histologically in all cases (43:P, 9:AP, mean-age:53years). Open adrenalectomy was performed in 20 cases and laparoscopically in 32. Hereditary phaeochromocytoma was confirmed by genetic analysis in 12 (23%) patients. Median follow up time from initial surgery was 47 months, (range: 12 - 296 months), 49 patients had no evidence of tumour recurrence at latest follow-up. Three patients (6%) demonstrated tumour development, one in a patient with VHL which occurred in a contralateral adrenal gland, one sporadic case had local recurrence, and an adrenal tumour occurred in a patient with a SDHB gene mutation who had a previous bladder tumour. After initial surgery, the tumours occurred at 8.6, 12.0 and 17.7 years respectively.ConclusionIn this study tumour development occurred in 6% of patients. Although tumour rates were low, careful and sustained clinical and biochemical follow up is advocated, as new tumour development or recurrence may occur long after the initial surgery is performed.     

肾上腺超声诊断中的重要三维断层解剖层面

目的：为肾上腺超声诊断提供三维断层解剖学依据.方法：18具肾上腺无器质性病变的尸体制作成横断层、矢状断层、冠状断层,观察左、右肾上腺首次出现和消失的断面、最佳显示断面及其毗邻关系;结合52例正常超声检查影像,总结临床肾上腺超声探查的最佳三维解剖断层.结果：解剖断面及断层连续超声显示,肾上腺横断面4个重要价值层面为第一肝门断层、经第一肝门下方断层、经左肾上极断层及经右肾上极断层;肾上腺矢状面4个重要价值层面为经左肾上极断层、经食管腹段断层、经下腔静脉断层及经右肾上极断层;肾上腺冠状面3个重要价值层面为经左右肾前份断层、经左右肾窦后份和脾门断层、经脊位圆椎和马尾断层.结论：肾上腺三维解剖断层及超声断面确定对临床肾上腺疾病诊断具有重要价值.     

Establishment of rat model of combined kidney-adrenal gland allotransplantation

Objective： To establish a rat model of combined kidney-adrenal gland and allotransplantation, and to explore the immunoprotecive effect of the transplanted adrenal gland on the transplanted kidney in the combined transplantation. Methods. SD rats 160 served as donors and recipients. The combined kidney-adrenal gland allotransplantation was performed. Infusion was conducted and prepared at prime position ,and the kidney and adrenal gland were at the left side. Direct vascular anastomosis and operation of connecting ureter attached part of bladder with the bladder were conducted. The kidney pedicle of the right side was ligated. Results： A stable and mature rat model of combined transplantation was established. The warm ischemia time was 30 seconds, and the cold ischemia time was 90-120min.The average time was 100 min. The operation time was 150 min. The survival time of the recipients was 21 days. The successful rate of the operation was 75%. Conclusion： The model of the combined kidney-adrenal gland allotransplantation can be established with higher successful rate. The model can be used to explore that transplanted adrenal gland may have immunoprotecive effect on the transplanted kidney in the combined transplantation.     

Bilateral adrenal histoplasmosis: endoscopic ultrasound-guided fine needle aspiration as a method of diagnosis and assessment

We report a case of a healthy 78-year-old indonesian man who presented with chronic weight loss, poor appetite and lethargy. CT abdomen showed bilateral adrenal masses. EUS-guided FNA was performed on the left adrenal gland. Histopathology report was Histoplasma Capsulatum. He recovered well with antifungal treatment without any complication. In this case, we found that the role of EUS -guided FNA was not only limited to diagnosis but also helped in the prognosis of the disease since the method was able to assess the general anatomy of the adrenal gland better than other imaging modalities due to its close proximity and direct visualization.     

正常肾上腺横断面MSCT影像研究

目的：利用多排螺旋CT (MSCT)观察测量正常肾上腺,为临床提供影像解剖数据。方法回顾性分析重医附一院2011年7月至2013年1月上腹部CT平扫加增强患者220例的影像资料,观察正常肾上腺形态、位置毗邻,测量肾上腺及膈肌脚厚度和CT值。结果①均以Ⅰ型为主,右侧65.5%(144/220)、左侧76.8%(169/220)。②肾上腺体部、内侧肢、“/”线形厚度均是左侧>右侧(P<0.01),外侧肢、“△”形厚度左右侧差异无统计学意义(P>0.05);右侧肾上腺内、外侧肢厚度差异无统计学意义(P>0.05),左侧肾上腺内侧肢厚度>外侧肢(P<0.01);膈肌脚厚度右侧>左侧(P<0.01)。以体部、侧肢大于同侧膈肌脚厚度假定异常,存在不同程度假阳性率。③正常厚度95%置信区间：左侧体部(3.0 mm,8.4 mm)、内侧肢(1.8 mm,4.5 mm)、外侧肢(1.3 mm,4.2 mm);右侧体部(2.3 mm,7.2 mm)、侧肢(1.5 mm,4.0 mm)。④双侧肾上腺平扫、动脉期、静脉期CT差异均无统计学意义(P>0.05);双侧肾上腺强化均匀。动静脉期强化程度CT值差值的散点图发现部分动脉期强化高于静脉期、部分低于静脉期,差值集中在-50~50 HU。结论 MSCT能评估正常肾上腺厚度,以其厚度大于同侧膈肌脚为异常的判断标准不可靠。肾上腺强化不具规律性,因此观察其强化是否均匀更具临床价值。