双侧巨大肾上腺髓脂瘤

肾上腺髓脂瘤是较罕见的良性肿瘤，由成熟脂肪细胞和造血细胞组成。通常在尸检或影像学检查时被偶尔发现。多数病灶孤立，体积较小，虽然无激素活性，但与功能性肾上腺疾病如库欣综合征及先天性肾上腺增生症罕有关联。该类患体内高水平的促肾上腺皮质激素被认为是肿瘤的促发因素。该报道了1例有先天性肾上腺增生症的双侧巨大肾上腺髓脂瘤患的影像学资料。据了解，这是所报道的与先天性肾上腺增生症相关的最大双侧肾上腺髓脂瘤。该首次报道了经一段时间皮质激素治疗后，该患影像学表现的显变化。     

肾上腺大结节样增生一例报道

库欣综合征(CS)中15% ～20%是由肾上腺原因引起的,其中约10%的患者具有双侧肾上腺增生.肾上腺性CS中皮质醇分泌有时可受肾上腺异常膜受体(包括抑胃肽受体、精氨酸加压素受体、儿茶酚胺类受体、LH/hCG受体和5-羟色胺受体等)的调节.该文报道了1例促肾上腺皮质激素非依赖性大结节样肾上腺增生引起的CS患者,并通过体内试验证实了肾上腺异常膜受体的存在.     

肾上腺大结节样增生一例报道

库欣综合征（CS）中15%～20%是由肾上腺原因引起的,其中约10%的患者具有双侧肾上腺增生。肾上腺性CS中皮质醇分泌有时可受肾上腺异常膜受体（包括抑胃肽受体、精氨酸加压素受体、儿茶酚胺类受体、LH/hCG受体和5-羟色胺受体等）的调节。该文报道了1例促肾上腺皮质激素非依赖性大结节样肾上腺增生引起的CS患者,并通过体内试验证实了肾上腺异常膜受体的存在。     

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.     

A clinico-pathological study of Cushing's syndrome at the University Hospital of the West Indies and a review of the literature

Cushing's syndrome is an uncommon but important disease. Twenty-one confirmed cases of spontaneous Cushing's syndrome were documented at the University Hospital of the West Indies over a 21-year period. They were predominantly young females (F:M ratio of 17:4; mean age 25 years and 3 months). The commonest presenting symptoms were amenorrhoea (41%) and obesity (19%). Common clinical features were cushingnoid features (95%), hypertension (76%) and hirsutism (82%). Twenty-nine per cent had frank hyperglycaemia. Cushing's syndrome was due to Cushing's disease in 10 cases, adrenal adenoma in 3 and adrenal carcinoma in 2 cases. In 4 cases with presumed adrenal hyperplasia, the histology was either unavailable or was not consistent with the diagnosis. Two cases appear now to have had the ectopic ACTH Syndrome. Adrenalectomy was the commonest treatment offered. There were no intra-operative or post-operative deaths but recurrence was common after subtotal adrenalectomy in Cushing's disease. Twenty-seven per cent of the patients developed Nelson's syndrome, which was fatal in 50%. Long-term hormone replacement therapy was unnecessary after surgery for adrenal adenomas. Treatment of Cushing's syndrome was well tolerated by the patients.     

Adrenal histoplasmosis in Cushing's syndrome with bilateral adrenocortical nodular hyperplasia

Adrenal histoplasmosis in Cushing's syndrome of adrenal origin is rare. A patient with Cushing's disease with bilateral nodular hyperplasia and histoplasmosis of both the adrenal glands is described. The diagnosis of histoplasmosis was only made post operatively as the constitutional manifestations, besides being partially masked by hypercortisolism also resemble those of tuberculosis.     

CLINICAL USE OF 131I-19-IODOCHOLESTEROL ADRENAL SCINTIPHOTOGRAPHY

51 cases of adrenal disease were diagnosed by 131I_19_iodocholesterol adrenal scintiphoto- graphy. Of these, 40 were primary aldosteron- ism and 11 Cushing's syndrome. 32 0f the 40 cases of primary aldosteronism lvere operated ripori througli incision on the side with higher radioactivity. Adenomas were found in 26, hyperplasia in 5 and normal histology in l. Re troperitoneal oxygen insafflation performed in 21 cases derrtonstrated an enlarged adrenal gland in 3. Scintiphotography is of great clinical value in Iocalizing adrenal cortical adenoma in pri- mary aldost'eronism antl Cushing's syndrome.     

External irradiation of the hypophysis for Cushing''s disease

During the past 12 years 17 patients with Cushing's disease (bilateral adrenal hyperplasia secondary to excessive pituitary adrenocorticotrophic hormone) have been treated initially with external pituitary irradiation. Of the 15 patients who have had adequate follow-up, nine showed complete biochemical remission, and one showed biochemical improvement. There were no complications. It is therefore recommended that the first mode of therapy for all patients with Cushing's disease should be pituitary irradiation if the patient's clinical condition permits.     

肾上腺肿瘤及增生27例诊治经验

27例肾上腺肿瘤及增生中皮质醇症8例、嗜铬细胞瘤16例、原发性醛固酮增多症、髓质增生症及无功能性肿瘤各1例。定位诊断主要为CT检查及腹膜后注气造影。皮质醇症术前应有效地控制血糖;嗜铬细胞瘤术前使用皮质激素有助于患者顺利地度过手术关。本组患者以手术治疗为主,并阐述了手术治疗的经验。     

Adrenal insufficiency after operative removal of apparently nonfunctioning adrenal adenomas

We describe a woman who developed adrenal insufficiency after removal of an apparently nonfunctional adrenal adenoma. She displayed no stigmata of Cushing's syndrome and had normal plasma and urinary cortisol levels. A second patient without clinical findings of Cushing's syndrome also had normal basal steroid levels. This patient displayed partial suppressibility with dexamethasone, had low-normal levels of serum corticotropin, and excreted a low concentration of urinary 17-ketosteroids. She also developed mild adrenal insufficiency after the operation. We believe the adrenal adenomas in these patients secreted enough cortisol to suppress the contralateral adrenal gland but not enough hormone to elevate basal steroid levels. Therefore, we suggest that all patients with adrenal masses be studied with the overnight dexamethasone suppression test rather than basal steroid hormone measurements to detect low levels of autonomous cortisol secretion. In addition, patients with adrenal masses that are not removed surgically should have serial adrenal function tests performed.     

Adrenal autotransplantation with A-V anastomosis for treatment of Cushing''s disease. A follow-up study of 31 cases.

Thirty-one patients with Cushing's disease were treated with bilateral adrenalectomy and partial left adrenal autotransplantation with A-V anastomosis. They were followed up for 1 to 5 years. Three patients failed after adrenal autotransplantation to the abdominal muscle with the left gland's central vein anastomosed to the inferior epigastric artery. Twenty-eight patients underwent adrenal autotransplantation to the omentum with the gland's vein anastomosed to the gastroepiploic artery. Steroid could be omitted a short period after operation and life-long replacement therapy was avoidable in most patients. A few patients subsequently developed hypocorticalism. Two died and one had recurrence of Cushing's disease. Clinical and animal experimental results showed that the omentum is suitable for adrenal autotransplantation with A-V anastomosis. Transabdominal bilateral adrenalectomy and adrenal autotransplantation to the omentum with A-V anastomosis are acceptable in the treatment of Cushing's disease. The subsequent development of hypocorticalism and recurrence of Cushing's syndrome are also discussed.

     

Clinics in diagnostic imaging (39). Conn's syndrome due to adrenocortical adenoma (Conn's tumour)

A 33-year-old man presenting with muscle weakness and hypertension was found to have hypokalaemia and metabolic alkalosis. Computed tomography (CT) showed an adrenal mass. Conn's syndrome due to an adrenocortical adenoma was confirmed at surgery. Hypertension-related adrenal disorders such as Cushing's syndrome, carcinoma and phaeochromocytoma are discussed. CT is currently the single most useful imaging method for identifying adrenal masses.     

22例肾上腺疾病CT诊断体会

目的探讨CT对肾上腺疾病的诊断价值。方法对22例肾上腺疾病的CT资料进行回顾性分析,在CT图像上观察病变的位置、形态、大小、密度及与周围结构的关系。结果5例肾上腺腺瘤,5例嗜铬细胞瘤,3例肾上腺皮质增生症,1例髓样脂肪瘤,5例肾上腺转移,3例肾上腺结核。结论CT对肾上腺疾病诊断具有重要价值。     

ADRENAL INCIDENTALOMAS:ANALYSIS OF 126 CASES

Adrenalmassesdiscoveredbyimagingtechniquesforreasonsunrelatedtoadrenaldiseasesarecalleda-drenalincidentalomas(1-3).Incidentalomaisadefi-nition,notadiagnosis.Thedetectionofmoreinci-dentalomasisexpectedastheuseofabdominalimag-ingincreases,suchasultrasound,computedtomog-raphy(CT)andmagneticresonanceimaging(MRI).Thecurrentprevalenceofunsuspectedadrenalmassesisapproximately1%to5%inabdominalCTscanseries(4,5).Infact,theincidenceofadrenalnod-ulesatautopsyisashighas32%amongpatientswithoutpremortemsu…     

Myelolipoma within a non-functional adrenal cortical adenoma

Myelolipoma within an adrenal cortical adenoma is a very rare cause of adrenal incidentaloma, and only nine cases have been reported in the English and Japanese literature. We report a 66-year-old Chinese man, with a history of hypertension and hyperlipidaemia, who presented with lower limb oedema and had a computed tomography (CT ) of the abdomen done to exclude intra-abdominal mass. His lower limb symptoms resolved after switching his antihypertensive medication. CT of the abdomen showed a large heterogeneously-enhancing mass in the left suprarenal region, measuring 72 mm by 55 mm. Clinical history, physical examination and laboratory results did not show any evidence to suggest metabolic disorder such as Cushing's syndrome, hyperaldosteronism or catecholamine hypersecretion. The patient underwent a left adrenalectomy, and a histopathological study confirmed the mass to be a non-functional adrenal cortical adenoma containing myelolipoma. The patient was well postoperatively and was discharged uneventfully. To the best of our knowledge, this is the first non-functional adrenal cortical adenoma reported; in the nine cases of myelolipoma within an adrenal cortical adenoma reported previously, all the patients had Cushing's syndrome. The literature on synchronous myelolipoma with adrenal adenoma, and myelolipoma within functional adrenal adenoma, is reviewed.     

Use of adrenal arterial embolization in severe ACTH-dependent Cushing's syndrome

The management of a patient with severe Cushing's syndrome due to ectopic ACTH produced by a medullary carcinoma of the thyroid is described. Initial treatment with maximal adrenolytic medical therapy and two attempts at bilateral adrenal venous infarction had failed to control the disease, and she was at that time unfit for surgery. Subsequent use of bilateral adrenal arterial embolization enabled medical therapy to produce sufficient control of the Cushing's syndrome to allow bilateral adrenalectomy to be performed successfully, with resultant cure of the excessive cortisol production.     

亚临床及肾上腺腺瘤型库欣患者的糖代谢紊乱

目的评价亚临床和肾上腺腺瘤型库欣患者糖代谢紊乱状况。方法对32例亚临床库欣(组1)、33例典型的肾上腺腺瘤型库欣(组2)及40例无功能瘤患者(组3)分别行口服糖耐量试验(OGTT),计算胰岛素敏感指数(ISI)、HOMA指数(HOMA-IR)、葡萄糖及胰岛素曲线下面积(AUCg,AUCi);同时检测各组患者血和尿皮质醇、血促肾上腺皮质激素(ACTH)及血钾等指标。以58名健康体检者作为对照组(组4)。结果组1、组2、组3和组4糖代谢紊乱的患病率分别为46.9%、48.5%、27.5%和22.4%;组2患者糖代谢相关指标,如空腹及糖负荷2 h后血糖(FBG及2 h PBG)、空腹及糖负荷2 h后血胰岛素(FIN及2 hPIN)及根据OGTT结果计算的AUCg、AUCi及HOMA-IR均高于其他组,而ISI则低于其他组别;剔除性别、年龄、腰围等相关因素的影响,多元回归分析结果显示,FBG、2 h PBG、AUCi与血、尿皮质醇水平呈正相关。组1患者皮质醇分泌仅为轻度升高,尚不足以引起库欣综合征典型的临床表现,但糖代谢紊乱和糖尿病的患病率亦升高(15.6%),其FBG、2 h PBG及AUCi等与血、尿皮质醇水平亦相关。结论亚临床及肾上腺腺瘤型库欣患者糖代谢紊乱的患病率明显升高,可能与皮质醇的高分泌状态有关。目的评价亚临床和肾上腺腺瘤型库欣患者糖代谢紊乱状况。方法对32例亚临床库欣(组1)、33例典型的肾上腺腺瘤型库欣(组2)及40例无功能瘤患者(组3)分别行口服糖耐量试验(OGTT),计算胰岛素敏感指数(ISI)、HOMA指数(HOMA-IR)、葡萄糖及胰岛素曲线下面积(AUCg,AUCi);同时检测各组患者血和尿皮质醇、血促肾上腺皮质激素(ACTH)及血钾等指标。以58名健康体检者作为对照组(组4)。结果组1、组2、组3和组4糖代谢紊乱的患病率分别为46.9%、48.5%、27.5%和22.4%;组2患者糖代谢相关指标,如空腹及糖负荷2 h后血糖(FBG及2 h PBG)、空腹及糖负荷2 h后血胰岛素(FIN及2 hPIN)及根据OGTT结果计算的AUCg、AUCi及HOMA-IR均高于其他组,而ISI则低于其他组别;剔除性别、年龄、腰围等相关因素的影响,多元回归分析结果显示,FBG、2 h PBG、AUCi与血、尿皮质醇水平呈正相关。组1患者皮质醇分泌仅为轻度升高,尚不足以引起库欣综合征典型的临床表现,但糖代谢紊乱和糖尿病的患病率亦升高(15.6%),其FBG、2 h PBG及AUCi等与血、尿皮质醇水平亦相关。结论亚临床及肾上腺腺瘤型库欣患者糖代谢紊乱的患病率明显升高,可能与皮质醇的高分泌状态有关。     

Surgical adrenal diseases

There are four adrenal disorders in which surgery is the treatment of choice: pheochromocytoma, Cushing's syndrome due to ACTH-independent adrenal disease (adenoma, carcinoma or autonomous hyperplasia), primary aldosteronism due to an adrenal adenoma and some adrenal non-functional "incidentilomas". In all four categories, the optimal result of surgical intervention depends on the following factors: 1. Definitive diagnosis by accurate biochemical assessment of the functional status of the adrenal lesion. 2. Precise localization of the lesion using simple routine as well as advanced imaging techniques. 3. Adequate preoperative preparation and intraoperative monitoring. 4. Close immediate and long-term postoperative follow-up. In this review, the above factors are briefly summarized for each of the four disorders. A small proportion of adrenal lesions requiring surgery are bilateral and in such situations, special intra- and postoperative care should take into consideration the need for appropriate gluco-corticoid and mineralo-corticoid replacement. The advent of laparoscopic surgery has served to reduce the postoperative morbidity of adrenal surgery and is becoming increasingly the procedure of choice except for large lesions (> 8 cm in diameter).     

Value of computed tomography of the abdomen and chest in investigation of Cushing's syndrome

Computed tomography (CT) scans were performed on 37 patients with biochemically proved Cushing's syndrome to evaluate the role of CT in the investigation of this condition. CT rapidly and correctly identified all 15 adrenocortical tumours, distinguishing five carcinomas from the 10 adenomas. In ACTH-dependent Cushing's syndrome appreciable bilateral adrenal enlargement was common in patients with an ectopic source (6 of 10 cases), while those with a pituitary source usually had normal sized adrenals (9 of 10). Two patients with a history of over seven years had bilateral adrenal nodules. CT was more accurate in locating a primary ectopic source of ACTH (5 of 12 cases) than any other technique and was particularly valuable in detecting small (less than 1.5 cm) peripheral lung carcinoid tumours which may be undetectable by conventional x-ray techniques. Its speed, accuracy, and simplicity make CT the technique of choice both to show the adrenal anatomy and to locate a suspected ectopic ACTH-secreting tumour in patients with proved Cushing's syndrome.     

1例肾上腺骨髓脂肪瘤报告及文献复习

肾上腺骨髓脂肪瘤是一种罕见的、无功能的良性肿瘤，它由成熟的脂肪组织和骨髓成份所组成。本文报道一例因病人偶感不适，常规体检时发现在左肾上腺骨髓脂肪瘤的病例。