Malignant carcinoid syndrome. Failure of anti-oestrogenic therapy

A pre-menopausal patient with a six-year history of symptoms of the metastatic carcinoid syndrome leading to progressively worsening carcinoid heart disease is described. The failure of anti-oestrogenic therapy (sequential bilateral oophorectomy and Tamoxifen therapy) to halt progression of disease was documented. Death resulted from right ventricular failure associated with pulmonary and tricuspid valvular disease.     

卵巢原发性类癌二病例报告并文献复习

目的探讨卵巢原发性类癌的临床病理特征。方法报道2例卵巢原发性类癌,并结合相关文献进行临床病理分析。结果病例1为岛状型类癌,肿瘤细胞呈圆形或多角形,排列成岛状或假菊团状,行单侧附件切除术;病例2为梁状型类癌,肿瘤细胞呈柱状,小梁由单至多层瘤细胞构成,瘤细胞核长轴与小梁走向垂直,其临床上有便秘的症状,术后缓解,行全子宫加双附件切除术。术后随访45个月和63个月均未见复发证据。免疫组化肿瘤细胞均呈不同程度的Syn（＋）、CK（＋）;α-inhibin、CD99、TTF-1、ER、PR均（-）;Ki-67增殖指数〈1%。结论卵巢原发性类癌临床罕见,诊断需和颗粒细胞瘤、支持细胞瘤等鉴别,并排除转移性类癌。作为一种低度恶性肿瘤,卵巢类癌多数预后良好。     

Lung carcinoid related Cushing's syndrome: report of three cases and review of the literature

Three patients with lung carcinoid related Cushing''s syndrome (LCRCS) treated at Frenchay Hospital, Bristol between 1984 and 1994 are described. The first patient presented with hyperpigmentation 13 years after bilateral adrenalectomy. The second patient had no recurrence or metastases 14 years after removal of a typical carcinoid tumour. The last patient survived nine years after diagnosis of liver metastasis. The possibility of LCRCS should be considered in every patient proved to have Cushing''s disease and bilateral adrenal enlargement on abdominal computed tomography. Biochemical sets of investigation (for example, adrenocorticotrophic hormone (ACTH) stimulation, dexamethasone suppression, and metyrapone response) could be misleading and should not be relied upon solely. Search for an ectopic ACTH source should be called off only when ACTH has been demonstrated in the surgically removed specimen, and most importantly, when the serum ACTH concentration returns to normal after surgery. Lung carcinoid tumours are compatible with long survival, and liver metastasis could prove indolent and slowly growing.


     

Primary carcinoid tumor of medulla spinalis: case report and review of the literature

Background

Carcinoid tumors are slow growing neuroendocrine tumors which can originate from various sites within the body. A carcinoid tumor originating in the medulla spinalis has not previously been reported in the literature.

Case report

We report a case of a 33-year-old man, presenting with a five-month history of bilateral lower extremity pain, as well as paresthesia, and mild weakness in one lateral lower extremity. A lumbar laminectomy of L3 to L5 and en bloc resection of the tumor was performed. Postoperative histopathology and immunohistochemical analysis of the tumor were consistent with that of a carcinoid tumor. There were no clinical or radiological signs of tumor recurrence or metastasis at the patient’s two year postoperative follow-up.

Conclusions

During the differential diagnosis of medulla spinalis tumors, the possibility of a primary carcinoid tumor originating within the medulla spinalis should be considered. An accurate tumor classification is imperative to ensure that the most effective course of treatment is pursued.

Electronic supplementary material

The online version of this article (doi:10.1186/s40001-014-0071-7) contains supplementary material, which is available to authorized users.     

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.     

结肠类癌8例报道

本文报道8例结肠类癌,常见的发病部位在盲肠和升结肠。因结肠类癌和腺癌在临床表现和X线方面很难鉴别,所以术前确诊较为困难,给合文献复习,我们认为类癌综合征的存在和尿中5-HIAA测定在本病的术前诊断中有重要价值。其治疗方法是手术切除,预后与术时有无转移有关,本文还讨论了类癌综合征及类癌的病理。     

胃肠类癌5例临床分析

目的：分析胃肠类癌的临床特点及治疗手段。方法：回顾性分析5例胃肠类癌临床资料、治疗及预后。结果：贲门类癌1例、阑尾类癌2例、直肠类癌2例,均手术切除,3例直径肿块小于2cm的类癌患者已存活5年以上。结论：胃肠类癌以阑尾、直肠类癌为多见,没有转移及直径小于2cm的类癌治疗效果好,早期诊断、早期治疗有利于延长类癌患者的生存时间。     

胃肠道类癌8例报告及国内文献复习

<正> 胃肠道类瘤是比较罕见的疾病,它是由Merling于1838年首先介绍阑尾肿瘤时提到的。在1907年Oberndorfer首先介绍了“类癌”(carcinoid)这一名词以与腺癌相区别。由于部分类癌可分泌五羟色胺并引起阵红等症状即所谓类癌综合症,(Carcinoid syndrome),因此近年来许多学者把类癌归并于所谓胺前体摄取及脱羧细胞瘤(Apudoma)一类,属胃肠道内分泌肿瘤。     

卵巢原发性胰腺类癌

背景：卵巢原发性胰腺类癌是一种罕见的肿瘤，在全身类癌发病总数中的比例〈1％。除1例病例报道外，先前所有报道的功能性卵巢类癌直径均〉10cm。因此，肿瘤的直径与类癌综合征具强相关性。病例报道：本文描述1例卵巢原发性功能性胰岛类癌，其最大直径仅为6cm。结论：虽然卵巢原发性类癌很罕见，但在临床推断中还是应当给予足够重视，尤其是患者表现出类癌综合征而附件肿物很小时。     

胃肠道类癌中MMP-2和p21WAF1/CIP1蛋白表达的意义

目的 探讨MMP-2和p21WAF1/CIP1蛋白阳性表达与胃肠道类癌组织分化、浸润和转移的关系.方法 采用免疫组化S-P法对36例胃肠道类癌组织MMP-2和p21WAF1/CIP1蛋白的表达进行检测.结果 36例类癌组织中,MMP-2蛋白在低分化类癌组高表达,MMP-2和p21WAF1/CIP1蛋白在高分化类癌组高表达(P＜0.05),随着肿瘤的浸润和淋巴结转移MMP-2阳性表达率显著增加(P＜0.01),MMP-2和p21WAF1/CIP1阳性表达降低(P＜0.05).结论 MMP-2和MMP-2和p21WAF1/CIP1在类癌组织中的表达与组织分化、浸润和淋巴结转移关系密切,可用于临床对病人进行预后判断.     

Carcinoid tumour of the cervix.

A case of non-metastatic carcinoid tumour of the cervix is described from a patient without the carcinoid syndrome. The rarity of carcinoid tumour involving gynaecological structures other than the ovaries is discussed and a bried review of carcinoid disease presented.     

Carcinoid tumour of gall bladder--a case report

Gall bladder carcinoid tumours are rare, constituting less than 1% of all carcinoid tumours arising from different parts of the body. They usually lack specific symptoms as they typically are unassociated with the carcinoid syndrome, despite frequent hepatic spread and mostly detected after cholecystectomy. A case of gall bladder carcinoid is reported in a 35-year-old woman who underwent laparoscopic cholecystectomy for clinical features of cholelithiasis. Several comparative evaluation was attempted mainly between the carcinoid and variant groups (endocrinomas) and occasionally between the typical and atypical carcinoid series to solve the existing serious problem in categorisation of this group of tumours. While the usual criteria for judging malignancy, such as anaplasia and mitotic figures, are unreliable in these sites of carcinoid tumour, malignancy is well determined from evidence of tumour invasion into adjacent structures. This rarity and the ambiguity around it stresses for its documentation and warrants for international agreements and standards on basic criteria for such classification of these endocrine carcinomas.     

消化道类癌43例Ki-67表达与肿瘤分期相关性研究

目的:分析肿瘤增殖抗原Ki-67在消化道类癌细胞中的表达及其与侵袭、转移关系。方法 :消化道类癌病理组织切片43例,运用免疫组织化学SP法检测Ki-67在类癌细胞核中的阳性表达率。结果:(1)病变部位:胃、十二指肠11例,小肠及结肠6例,阑尾5例,乙状结肠及直肠21例。(2)恶性程度:早期癌仅局限于粘膜及粘膜下27例;进展期癌浸润至肌层以下16例,淋巴结转移18例。(3)Ki-67在早期类癌27例中阳性表达8例(29.63%),在进展期类癌16例中阳性表达12例(75.00%),两者比较差异有统计学意义(P<0.05)。而Ki-67在无淋巴结转移类癌25例中阳性表达8例(32.00%),在有淋巴结转移类癌18例中阳性表达14例(77.78%),两者比较差异也有统计学意义(P<0.05)。结论:Ki-67的表达与消化道类癌的恶性程度存在相关性,可作为消化道类癌恶性程度及预后的检测指标。     

直肠类癌诊治分析（附27例病例报告）

目的分析直肠类癌的临床特征。方法回顾性分析2001年1月～2010年12月收治的27例直肠类癌患者的诊断方法、手术方式及生物学特性。结果本组直肠类癌患者直肠指检＋肠镜活检正确诊断率可达92.6%。本组27例中17例行肛肿块局部切除术,2例行Dixon术,1例行Mile’s术,1例行TEM术,其余6例行ESD术。术后并发症发生率7.4%。27例患者其中25例得到随访,随访率92.6%,5年生存率85.7%。结论直肠类癌症状不典型,临床诊断率低,易误诊;直肠指检＋肠镜病理为诊断直肠类癌首选方法,手术切除是治疗结直肠类癌的首选方法。     

Primary carcinoid tumor of the testis: case report

Carcinoid tumor of the testis is exceedingly rare. Most carcinoid tumors occur in the appendix or ileocecal region (85%), while others are found in the lung, liver, and genitourinary tract (15%). A primary carcinoid testis tumor may originate from argentaffin or Kulchitsky's cells, which are located in the Lieberkuhn crypt. Preoperative ultrasound may show a solid, hypoechoic, well-defined margin mass combined with calcification or a cyst. Differential diagnosis of the ultrasound appearance is testicular tumor (teratoma/embryonal cell carcinoma), epidermoid tumor, tuberculous epididymo-orchitis, and the result of trauma. Radical orchiectomy remains the main treatment for a carcinoid testis tumor. Grossly, surgical removal of the tumor presents with a solid mass, tan to white in color. Immunohistochemical study shows that tumor cells are diffusely reactive to antibodies to keratins AE1 and AE3, chromogranin-A, neuron-specific enolase (NSE), and synaptophysin. A pure primary testicular carcinoid tumor has been treated as a benign lesion, while metastatic carcinoid tumor has a poor prognosis regardless of the primary site. To rule out the possibility of metastasis resulting from an extra-testicular primary carcinoid, careful and thorough postoperative whole body surveys are important. Chest X-ray, chest computed tomogram (CT), abdominal and pelvic CT, and octreotide scintigraphy are indicated. We herein describe a case of primary carcinoid tumor of the testis and review the literature.     

Synchronous carcinoid tumour of the small intestine and appendix in the same patient

Carcinoid tumours have been reported in a wide range of organs but most frequently involve the gastrointestinal tract. Many of these carcinoid tumours are associated with metachronous and synchronous lesions of another histological type. Primary carcinoid tumours of the different organ in the same patient is rare. In this paper, the authors present a case with synchronous carcinoid tumour of the small intestine and appendix in the same patient.     

消化道类癌的内镜诊断及治疗

目的:探讨内镜在消化道类癌的诊断及治疗中的价值.方法:分析54例消化道类癌的形态学特点、治疗方法及预后.结果:本组类癌内镜确诊率达97.8%, 直肠类癌最多见(6 1.1%).16例最大径不超过1 cm消化道类癌行内镜下治疗,未见并发症发生,随访3个月～1 1年 ,未见复发.结论:内镜检查为诊断消化道类癌的有效方法,对最大径<1 cm的类癌采用内镜下治疗是一种简单、安全、有效的治疗方法.     

Carcinoid syndrome without liver metastasis.

The carcinoid syndrome generally occurs due to metastasis of a carcinoid tumour in the liver. A patient is presented who had carcinoid syndrome due to abdominal disease without hepatic metastasis.     

Carcinoid tumour arising in a Meckel's diverticulum

A patient with a carcinoid tumour arising in a Meckel's diverticulum and presenting with acute small intestinal obstruction is described. Review of previously reported cases indicates that the initial clinical presentation of carcinoid tumours occurring in a Meckel's diverticulum is usually similar to that of appendiceal carcinoids. While most carcinoids in Meckel's diverticula present as incidental findings at post-mortem examination or laparotomy, their metastatic potential and subsequent behaviour resembles that of other small intestinal carcinoid tumours.     

胃肠类癌17例临床分析及文献复习

目的:探讨胃肠道类癌的临床特点、诊断和治疗方法。方法:回顾性分析由病理切片确诊的17例胃肠道类癌病例的临床资料。结果:在17例类癌病例中,以胃和直肠较多见,除1例结肠类癌外,其余均直径大于3cm,且有76.5%浸及肌层,65%有区域淋巴结转移,但无1例有类癌综合征。结论:病理学诊断是胃肠类癌确诊的主要方法,早期发现、早期确诊及早期治疗是预后的关键。