肺部病变所致异位促肾上腺皮质激素综合征的临床诊治经验

目的探讨肺部病变导致异位促肾上腺皮质激素(ACTH)综合征的临床诊治经验。方法回顾性分析1985年9月至2013年5月间经手术病理证实的肺部病变引起的异位ACTH综合征20例患者的资料。结果 20例患者中,男性12例,女性8例。就诊时的年龄13⁶5岁,平均年龄为37.2岁。确诊时病程为165岁,平均年龄为37.2岁。确诊时病程为1¹56个月。所有患者均存在异位ACTH综合征的临床表现,均由胸部计算机断层扫描(CT)定位肺部病变。手术治疗后血清皮质醇(P<0.001)及血ACTH浓度(P=0.001)均明显下降。无围手术期死亡病例。平均随访时间47.9个月,暂无复发及死亡病例。结论肺部病变是导致异位ACTH综合征的最常见原因,主要为肺部神经内分泌肿瘤,且以类癌为最常见,诊断及治疗均存在较大难度,CT是病变定位的最佳方法,早期发现并行手术切除病灶是治疗的关键。     

儿童胸腔内原始神经外胚层肿瘤致库欣综合征

库欣综合征在儿童及青少年很少发生，与单纯性肥胖鉴别的主要特征之一是身高生长受到抑制。一旦临床诊断库欣综合征，应重点明确病因。儿童库欣综合征分为ACTH依赖和非ACTH依赖两种类型，在不同年龄阶段，其病因谱不同。成年人异位ACTH综合征（EAS）占ACTH-依赖性库欣综合征约15％。儿童EAS的病因主要为类癌，     

支气管原发腺样囊性癌3例

原发性支气管腺样囊性癌临床较为少见,曾有文献报道其占肺癌总数的０．３％～０．５％,我院曾治疗３例患者。 例１,男性,６７岁。主因咳嗽伴发热２个月,间断性胸痛１个月于１９９３年６月就诊。经胸ＣＴ检查示左肺上叶占位病变,支气管镜检查病理为腺样囊性癌,后行左全肺切除术,术中可见肿物位于左上叶支气管开口处,且侵至主支气管,肿物约为２ｃｍ×２．５ｃｍ×３ｃｍ大小,全肺切除术后病理报告为腺样囊性癌、支气管断端阴性、支气管旁淋巴结无癌转移,术后随访７年健在。 例２,男性,３９岁。主因咳嗽伴咳痰带血２０天而就诊,…     

甲状腺髓样癌术后淋巴转移致异位ACTH综合征：一例报告并文献复习

目的：总结一例甲状腺髓样癌术后淋巴转移所致异位ACTH综合征患者的诊治过程,结合文献对本病的发病机制、临床表现及分型、诊断和治疗加以探讨。方法：对南京市鼓楼医院内分泌科2009年收治的一例甲状腺髓样癌术后淋巴转移所致异位ACTH综合征的一例病例进行总结分析,复习相关文献,阐明本病的基本特征及研究进展。结果：甲状腺髓样癌术后淋巴转移所致异位ACTH综合征临床十分少见。充分认识其临床表现、理化检查及影像学定位,给予及早诊断和综合治疗十分重要。结论：早期发现、早期诊断、早期治疗是控制ESA的关键。     

容易误诊的弥漫性肺腺癌(附14例报告)

1990年1月至1996年12月我院经纤维支气管镜（简称纤支镜）确诊的支气管肺癌共1402例。其中中心型924例（65．9％），周围型478例（34．1％）。周围型中表现为弥漫性肺病变者15例，占周围型肺癌的3．1％。本文报告了14例弥漫性肺腺癌，并就其误诊原因及诊断方法加以讨论。临床资料1．14例病人多由外院转来，均经纤支镜取材病理证实。在同期经纤支镜确诊的14OZ例肺癌，鳞癌623例（44．4％），小细胞未分化癌485例（34．6％），腺癌233例（16．6％），大细胞性未分化癌7例（0．5％），其它（恶性组织细胞癌、类癌、腺鳞癌及转移癌等）54例（3…     

甲状腺髓样癌术后淋巴转移致异位ACTH综合征:一例报告并文献复习

目的:总结一例甲状腺髓样癌术后淋巴转移所致异位ACTH综合征患者的诊治过程,结合文献对本病的发病机制、临床表现及分型、诊断和治疗加以探讨。方法:对南京市鼓楼医院内分泌科2009年收治的一例甲状腺髓样癌术后淋巴转移所致异位ACTH综合征的一例病例进行总结分析,复习相关文献,阐明本病的基本特征及研究进展。结果:甲状腺髓样癌术后淋巴转移所致异位ACTH综合征临床十分少见。充分认识其临床表现、理化检查及影像学定位,给予及早诊断和综合治疗十分重要。结论:早期发现、早期诊断、早期治疗是控制ESA的关键。     

经内镜激光消融联合激光光动力学治疗气管支气管肿瘤

[目的]探讨经内镜激光消融联合激光光动力学治疗气管支气管肿瘤的临床疗效。[方法]对56例气管支气管肿瘤患者先用YAG激光经内镜进行消融治疗，然后按2mg/kg静注光敏剂（photosan或癌光灵），48及72h后用630nm激光照射肿瘤部位。[结果]完全效应（CR）32例（57．1％）；明显效应（SR）21例（37．5％）；微效应（MR）3例（5．4％）。2例类癌患者现已分别存活4年和3年2个月，虽已有转移但仍生存。其余患者生存时间最短为2个月，最长为19个月，中位生存期为8．5个月。[结论]经气管镜激光消融联合光动力治疗气管支气管肿瘤起效快，能迅速改善症状，也能取得较为满意的远期疗效。     

DNA图像分析技术在肺良恶性病变诊断中的价值

目的：研究支气管肺良恶性病变中DNA倍体及DNA指数（D1）情况,评估其在支气管肺良恶性病变诊断中的价值。方法：用图像分析系统（MPIAS－500）对39例支气管粘膜慢性炎症,45例鳞状上皮化生、45例异型增生、63例癌进行DNA倍体分布及DNA指数的检测。结果：支气管粘膜慢性炎症组、鳞状上皮化生组非整倍体均＜1．3％,DI均值分别为0．92和1．03;异型增生组非整倍体为2．8％,DI均值为1．34;而癌症组非整倍体为18．93％;DI均值为1．72,明显高于良性病变组（P＜0．001）。结论：支气管肺良恶性病变细胞核DNA倍体及非整倍体所占的百分比不同。定量分析检测细胞核DNA倍体及DI均值有助于支气管肺良恶性病变的诊断及鉴别诊断,并可为临床治疗和预后判断提供有效的客观指标。     

补充性全肺切除术对残肺病变的治疗价值

目的：评估补充性全肺切除术的适应证、危险性和结果。方法：回顾性分析６８例年龄在１５￣７１岁的残肺良恶性病变的患者的补充性全肺切除术，其中第二肺癌１７例，肺复发癌３２例，良性肺及胸膜疾患１９例。再次手术间隔期为恶性者２９个月，良性为２１７个月。结果：７例患者死亡（１０．４％），２例死于术中，５例死于术后，癌性患者死亡率（１１．６％）高于良性患者（５．９％）。全部病例５年生存率为４８％，癌症患者３３％     

18F-FDG PET/CT鉴别肺占位病变性质方法的研究

目的：探讨^18F-FDG PET／CT对肺占位病变性质鉴别诊断方法的研究。方法：发现肺部占位病变患者74例，不含有支气管阻塞性肺炎，所有患者均进行3-14个月的随访。恶性患者55例（包括1例假阴性）其中23例经手术病理证实，32例临床诊断肺癌；良性患者19例其中7例经手术病理证实，12例经3～14个月随访病灶范围缩小或消失。所有患者均进行^18F-FDG PET／CT全身检查、呼吸控制的CT扫描及重建1．25mm薄层图像。^18F-DDG PET／CT诊断恶性的标准：1）肺病灶的SUVmax≥2．5；2）延迟显像SUVmax升高明显（≥20％～30％）；3）1．25mm薄层CT图像典型形态学征象；4）从病灶中心至各个胸壁变化曲线SUVmax变化曲线。结果：74例肺部痛灶患者中恶性55例，其中临床诊断肺癌32倒，腺癌13例，鳞癌5例，细支气管肺泡癌3倒，小细胞肺癌2例；良性19例，其中慢性炎症13例，肉芽肿2例，炎性假瘤2例，结核2例。PET、图像SUVmax、延迟SUVmax增高情况及薄层CT的典型征象综合最终^18F-FDG PET／CT诊断恶性病灶共57例，其中真阳性54例，假阳性3例；良性病灶共17例．其中真阴性16例，假阴性1例其敏感性、特异性、准确率、阳性预测值及阴性预测值分别为：98．2％，84．2％，94．6％，94．7％及94．1％。结论：PET／CT检查观察病灶SUVmax、延迟SUVmax结合薄层CT图像典型的形态学征象，并从每个病灶中心至各胸壁做SUVmax空间变化曲线等，明显提高了诊断的准确率。     

Carcinoid Heart Disease in Patients With Bronchopulmonary Carcinoid

Introduction

The prevalence of carcinoid heart disease (CaHD) in bronchopulmonary carcinoid and its relationship with left-sided valvular disease are unknown.

Ectopic Cushing syndrome associated with thymic carcinoid tumor as the first presentation of MEN1 syndrome-report of a family with MEN1 gene mutation

Multiple endocrine neoplasia type 1(MEN1) is an autosomal dominant syndrome. Although thymic carcinoid tumor is recognized as a part of MEN1 syndrome but functioning thymic carcinoid tumor as the first presentation of the MEN1 seems to be very rare. In this report, we present a 29-year-old male who developed ectopic Cushing syndrome secondary to thymic carcinoid tumor and was diagnosed as MEN1 syndrome 2 years later. Further evaluation revealed the presence of carcinoid tumor and other MEN 1 manifestations in several other member of family. Genetic evaluation showed presence of a previously reported mutation in exon 10(R527X) of MEN1 gene in these patients. This presentation showed that thymic neuroendocrine tumor could be the first manifestation of the MEN1 syndrome and it might be diagnosed as a dominant manifestation of this syndrome in a family. We suggest biochemical or genetic screening for MEN-1 syndrome for patients with thymic carcinoid.     

Bronchopulmonary carcinoid in multiple endocrine neoplasia type 1

BACKGROUND: Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal-dominant syndrome associated with neoplasia of pituitary, pancreas, parathyroid, and foregut lineage neuroendocrine tissue. Although enteropancreatic carcinoid has been well described in patients with MEN 1, it was believed that bronchopulmonary carcinoid was relatively uncommon, occurring in approximately 5% of patients. It is unclear whether the increased screening of asymptomatic patients with MEN 1 will facilitate early diagnosis of this tumor and improve patient prognosis. METHODS: The authors reviewed the patient records and, when available, thoracic computed tomographic (CT) images of 129 MEN 1-affected adult members of a single family to determine the prevalence and prognosis of bronchopulmonary nodules and carcinoid. RESULTS: Among 129 patients, a diagnosis of bronchopulmonary carcinoid was noted in the records for 6 individuals (1 male and 5 females; 5%). Thoracic CT scans also were available for review from 32 of those patients. Twelve patients (38%) had pulmonary nodules evident on CT scans. Only hypergastrinemia was significantly more common in patients with pulmonary nodules; otherwise, the spectrum of neoplasia was similar between individuals with and without pulmonary lesions. Histologic diagnoses were available in four patients (three female) with abnormal CT images, and carcinoid was confirmed in each patient. No deaths or distant metastases occurred among the patients despite long-term follow-up (mean, 127 months). CONCLUSIONS: The findings suggested that bronchopulmonary carcinoid is more prevalent in patients with MEN 1 than was recognized previously. Furthermore, the diagnosis did not appear to portend a poor prognosis in the majority of affected patients.     

Difficulties in Diagnosis and Management of Ectopic Cushing Syndrome

A 18-year-old man presented to a local hospital with muscle weakness, facial edema, and a 6 kg weight gain over 2 months. After a complete work-up, he was diagnosed with pituitary Cushing syndrome and treated with a bilateral adrenalectomy for Cushing syndrome and pituitary radiotherapy for Nelson syndrome. Twenty-five years later, his ectopic source of adrenocorticotropic hormone was revealed as a pulmonary neuroendocrine tumor, and a pulmonary resection was performed. Subsequently, a biochemical and clinical remission including hyperpigmentation was achieved.     

Treatment of Metastatic Carcinoid Tumors and the Carcinoid Syndrome with Recombinant interferon Alpha

Fourteen patients with metastatic carcinoid tumors were treated with recombinant interferon alpha-2b at a dosage of 3-4 × 10⁶IU s.c. daily or every second day. No objective tumor regression was observed. Six out of 8 patients with carcinoids of the ileum and the caecum showed stable disease lasting for a median of 25 months (range 4-57). in 3 out of 6 patients with carcinoids of rectum, lung and of unknown primary site, stable disease was observed lasting for 2-7 months. the remaining patients had progressive disease. Six out of 9 evaluable patients had a more than 50% reduction of urinary 24 h 5-hydroxyin-doleacetic acid excretion lasting for a median of 4 months (range 2-11). Decrease of flushing was noticed in 3 out of 6 evaluable patients and decrease of diarrhea in 5 out of 9 evaluable patients. in 4 patients dose reduction was necessary due to confusion and fatigue.     

Treatment of metastasized carcinoid tumor of the ileum and cecum with recombinant alpha-2b interferon

Seven patients with progressive ileal or caecal carcinoid tumors and liver metastases were treated with human recombinant alpha-interferon (IFN alfa-2b) at a dosage of 2-4 x 10(6) U daily or every other day subcutaneously. Six patients had symptoms of the carcinoid syndrome. No change of tumor size lasting 4 to 40+ months (median, 18 months) was noted in 6 patients, and 1 patient had hepatic tumor progression. A decrease in urinary excretion of 5-hydroxyindoleacetic acid by more than 50% lasting 2-11 months (median, 4) was observed in 5 patients. Four patients were completely or partially relieved of flushing, diarrhea, obstruction or abdominal pain. The side-effects were negligible with the exception of mild fever, headache and confusion only during the first days of therapy. Treatment with IFN alfa-2b offers good palliation to patients with disseminated ileal or caecal carcinoid tumor and carcinoid syndrome.     

Carcinoid Tumors

Carcinoid tumors are rare, slow‐growing neuroendocrine tumors arising from the enterochromaffin cells disseminated throughout the gastrointestinal and bronchopulmonary systems. Though they have been traditionally classified based on embryologic site of origin, morphologic pattern, and silver affinity, newer classification systems have been developed to emphasize the considerable clinical and histopathologic variability of carcinoid tumors found within each embryologic site of origin. These neoplasms pose a diagnostic challenge because they are often innocuous at the time of presentation, emphasizing the need for a multidisciplinary diagnostic approach using biochemical analysis, standard cross‐sectional imaging, and newer advances in nuclear medicine. Similarly, treatment of both primary and disseminated carcinoid disease reflects the need for a multidisciplinary approach, with surgery remaining the only curative modality. The prognosis for patients with these tumors is generally favorable; however, it can be quite variable and is related to the location of the primary tumor, extent of metastatic disease at initial presentation, and time of diagnosis.     

Primary Pleuropulmonary Neoplasms in Childhood: Fourteen Cases from a Single Center

Background: We aimed to review clinical characteristics, treatment results and outcome of pediatric patientswith primary pleuropulmonary neoplasms. Methods: Medical records of 14 cases diagnosed between 1972-2009were reviewed retrospectively. Results: The male/female ratio was 5/9 and the mean age at diagnosis was 9.1years (2-16). All but one were symptomatic, presenting with fever, coughing, dyspnea, or weight loss. One patientpresented with hemoptysis, and another with digital clubbing. One mesothelioma was diagnosed incidentally.Some 8/14 patients were initially diagnosed as having pneumonia (median delay in diagnosis of 2.5 months).Diagnoses included pleuropulmonary blastoma (PPB, n=5), inflammatory pseudotumor (n=3), mesothelioma(n=2), mucoepidermoid carcinoma (MEC, n=2), and carcinoid tumor (n=2). Patients with PPB underwent surgeryand received chemotherapy ± radiotherapy. Two carcinoid tumor cases underwent surgery, one further receivedchemotherapy. Patients with mesothelioma were treated with chemotherapy. Inflammatory pseudotumors wereall resected. Two cases with MEC received chemotherapy, one after surgery. 2/5 PPB patients survived withoutrecurrence, 3 died; all carcinoid tumors and inflammatory pseudotumors were alive; 1/2 MEC patients wasalive after 252 months, the other one was lost without disease; 1/2 mesothelioma patients was alive withoutdisease, the other was died. For all cases, median follow-up was 30.5 months (0.6-252). Conclusions: Primarypleuropulmonary tumors are rare but clinical presentation can be varied and delay in diagnosis is common.Children with persistent coughs, recurrent pneumonia or hemoptysis should be considered as indicators forearly diagnosis, very important because the prognosis of these tumors varies with histology and stage.