直肠神经内分泌肿瘤1例并文献复习

类癌(carcinoid)是来源于神经内分泌细胞的肿瘤,其好发于胃肠道,因其缺乏特异性的临床症状,且临床上较为少见,容易漏诊、误诊,临床医师应当高度重视。本文结合直肠神经内分泌肿瘤1例,对其来源、临床特征、病理特征、诊断、治疗及预后进行回顾及文献复习。     

内镜黏膜下剥离术治疗上消化道神经内分泌肿瘤的临床价值

目的 探讨内镜黏膜下剥离术(ESD)治疗上消化道神经内分泌肿瘤的安全性及其疗效.方法 采用ESD方法对19例上消化道神经内分泌肿瘤进行治疗,术后标本送病理检查,记录不良反应发生情况及疗效,随访肿瘤复发及转移情况.结果 肿瘤直径0.4 ～1.5 cm,平均0.9 cm,均一次性完整剥离切除,ESD手术时间(自黏膜下注射至完整剥离病变)15～ 50 min,平均20 min,无严重出血及穿孔发生.术后18例病理诊断为神经内分泌瘤,其中G1级16例、G2级2例,基底和切缘均未见病变累及;另1例病理显示有浸润性生长倾向,诊断神经内分泌癌(G3级),行外科扩大切除,术后病理未见肿瘤组织残留,无淋巴结转移.平均随访28个月,无一例出现肿瘤复发和转移.结论 ESD治疗上消化道神经内分泌肿瘤具有较好的安全性和疗效,值得在临床上推广应用.     

食管胃交界神经内分泌肿瘤的病理形态观察

目的观察食管胃交界神经内分泌肿瘤的病理形态。方法对216例食管胃交界肿瘤分别用Synapto-physin、Chromogranin、CD56和Neuron-specific enolase（NSE）四种抗体进行免疫组化检测,筛选出51例神经内分泌肿瘤,进行病理形态学观察。结果 51例神经内分泌肿瘤中包括单纯性神经内分泌癌9例,混合性外分泌—内分泌癌42例。Synaptophysin、Chromogranin、CD56和NSE四种抗体在单纯性神经内分泌癌中呈弥漫强阳性表达,而在混合性外分泌—内分泌癌中的神经内分泌癌区域呈不同强度表达。51例神经内分泌肿瘤中溃疡型39例、局部浸润型6例、弥漫浸润型4、黏膜局部隆起型2例,侵及网膜37例、浆膜层7例、肌层5例、黏膜下层2例,肿瘤长径2.0～7.0 cm。结论食管胃交界神经内分泌肿瘤病理形态比较复杂,需要依据免疫组化检查与其他肿瘤进行鉴别诊断。     

胰腺异位副脾误诊为神经内分泌肿瘤：1例报道并文献复习

胰腺异位副脾临床少见，多误诊为神经内分泌肿瘤而进行不必要的治疗，本文报道1例胰腺异位副脾误诊为神经内分泌肿瘤经病理活检最终确诊，并回顾国内外最新文献，以期为临床诊疗提供借鉴。     

胰腺神经内分泌癌合并皮肌炎一例

神经内分泌肿瘤(neuroendocrine neoplasms,NENs)是来源于具有神经内分泌标志物的细胞且可以产生生物活性物质和/或多肽类激素的一类肿瘤,其中神经内分泌癌(neuroendocrine carcinoma,NEC)是病理分级为高级别的一种NENs。有报道称,皮肌炎(dermaomyositis,DM)与恶性肿瘤的发生具有相关性,但合并胰腺癌较为少见,合并胰腺NEC的更是鲜有报道,我科收治了一例胰腺NEC合并皮肌炎的患者,明确诊断后给予抗肿瘤治疗,二者均好转,本文旨在讨论胰腺NEC的诊治经过以及皮肌炎与恶性肿瘤发生的内在联系,为临床提供借鉴。     

男性乳腺神经内分泌癌1例

<正>乳腺神经内分泌癌非常罕见,发病率约为2%⁵%,在所有乳腺肿瘤中比例低于0.1%,而在各种类型的神经内分泌癌中,其所占比例低于1%〔1〕。关于乳腺神经内分泌癌的生物学行为、临床治疗方案以及预后尚缺乏足够的认识,已成为该领域亟待解决的难题。本文报道1例男性乳腺神经内分泌癌患者的病理特征、临床表现及治疗方案,并结合相关文献以探讨该病的特征。1病例报告1.1病史男性,66岁,2009年发现右乳房肿块在当地医院     

内镜黏膜下剥离术治疗食管混合性神经内分泌-非神经内分泌肿瘤1例

<正>混合性神经内分泌-非神经内分泌肿瘤(mixedneuroendocrine-non-neuroendocrine neoplasm,MiNEN)是临床上较为罕见的神经内分泌成分和非神经内分泌成分分别至少占病变的30%的肿瘤^[1-2]。其发病率低,多见于中老年男性,临床表现缺乏特异性,确诊需依靠术后病理组织活检联合特异性的免疫组织化学检测^[3]。由于其罕见性,治疗方式的选择上并无明确的指南和规范,对于局限期的肿瘤,主要采取根治性外科手术切除。而进展期肿瘤,主要予以放化疗和其它分子靶向药物治疗^[4]。有文献综述认为,随着内镜技术的发展,神经内分泌肿瘤可在综合评估的基础上,适当的进行内镜下黏膜切除术(EMR)或内镜粘膜下剥离术(ESD)治疗,术后根据病理情况考虑是否追加外科手术及辅助放化疗^[5]。本文报道了1例ESD治疗食管MiNEN病例,为该疾病诊断及治疗提供临床参考。     

胃神经内分泌癌1例报告并文献复习

目的探讨胃神经内分泌癌的临床特点,加深对该病的认识。方法对1例胃神经内分泌癌患者的临床表现及诊疗经过行回顾性分析。结果患者以腹痛、腹胀伴恶心、呕吐1年入院,经检查诊断为胃幽门管溃疡A1期、慢性胃炎,给予抑酸、护胃、促胃肠动力及对症支持治疗,患者症状无改善。行上腹部强化CT示胃幽门区胃癌并肝脏、腹腔淋巴结多发转移,复查胃镜示胃窦占位、糜烂性胃炎,病理结果示胃窦神经内分泌癌,确诊为胃神经内分泌癌并肝脏、腹腔淋巴结转移。患者治疗效果不佳,病程进展迅速,最终死亡。结论胃神经内分泌癌临床表现缺乏特异性,确诊依赖于病理检查,预后较差。应提高对本病的认识。     

疑似胃神经内分泌肿瘤的胃底腺型胃癌1例

本文报道1例疑似胃神经内分泌肿瘤的胃底腺型胃癌, 回顾分析其临床、内镜、病理特征, 旨在提高胃底腺型胃癌的诊断水平以及减少误诊、漏诊。     

神经肽受体与肺神经内分泌瘤

肺神经内分泌瘤是一类特殊的肿瘤,具有神经内分泌的功能表现,可以合成、贮存和分泌各种神经胺类及肽类化学递质,并引起相应的临床综合征.近年来研究表明肺神经内分泌瘤表面存在多种神经肽受体,它们在肺神经内分泌瘤的白分泌、旁分泌及内分泌调节中具有重要作用.本文综述了多种神经肽受体在肺神经内分泌瘤表面的分布及其生物学功能,并探讨了其在肺神经内分泌瘤临床诊治中的应用前景.     

Effects of tumor origins and therapeutic options on the prognosis of hepatic neuroendocrine tumors: A retrospective study

Hepatic neuroendocrine tumors (HNETs) are uncommon neoplasms that can be subdivided into 2 types: primary and metastatic HNETs. Due to its rarity, heterogeneity and complexity, the diagnosis, treatment modalities and prognosis are still controversial.This retrospective study reviewed the effects of tumor origins and therapeutic options on the prognosis of gastroenteropancreatic neuroendocrine tumors with liver metastasis (GEP-NETLM) and primary hepatic neuroendocrine tumors (PHNETs), providing additional evidence for clinicians evaluating patients.HNETs consisted of PHNETs and GEP-NETLM. GEP-NETLM (76.2%, 112/147) was more common, which was mainly manifested as multiple lesions in both lobes of the liver. PHNETs were relatively rare (23.8%, 35/147) and were mainly single lesion located in the right lobe of the liver. In patients with GEP-NETLM, primary tumor resection could prolong survival (P = .044). As the most widely used treatment method, systematic therapy alone could not achieve a satisfactory survival. However, the combination with hepatectomy or liver-directed therapy improved the prognosis (P = .023). As the main treatment, patients with PHNETs treated with local therapy could achieve a better prognosis (P = .049). Compared with PHNETs patients, GEP-NETLM patients with higher ki-67 index showed higher mortality and poorer prognosis (P = .006).Therefore, patients with PHNETs can be distinguished from GEP-NETLM by comprehensive imaging examinations and long-term follow-ups. The choice of appropriate treatment strategies can improve the prognosis of HNETs patients.     

Clinical features and outcomes of primary hepatic neuroendocrine carcinomas

Background and Aim: Primary hepatic neuroendocrine carcinomas (PHNECs) are extremely rare, with only about 150 cases having been reported in the English‐language literature. Because of the rarity of PHNECs, its clinical features and treatment outcomes are not well understood. Here, we report our experiences with PHNECs. Methods: We identified patients diagnosed with PHNEC and analyzed their demographics, baseline laboratory data, tumor characteristics, treatment modalities and outcomes. Results: A total of 218 consecutive patients were identified with pathologically confirmed neuroendocrine carcinoma. Of these, 12 patients were diagnosed with PHNECs; the median age was 66.5 years (range, 37 to 80 years), and seven patients (58.3%) were male. Two patients who each had a single hepatic mass underwent curative surgical resection. One patient who was of inoperable status at the initial diagnosis because of multiple intrahepatic metastases showed a partial response after the ninth round of systemic chemotherapy and then underwent surgical resection. The median overall survival in the 12 patients was 16.5 months (range, 0.7 to 41.7 months). Three patients who underwent surgical treatment are alive without recurrence for 15.2 months, 18.0 months, and 36.9 months, respectively. Conclusions: Primary hepatic neuroendocrine carcinoma should be considered as a possible differential diagnosis in the management of hepatic tumors. The liver can be the primary origin of neuroendocrine tumors, and if the tumors are diagnosed as primary hepatic neuroendocrine tumors, surgical resection must be considered for curative treatment.     

Recurrent gastric neuroendocrine tumors treated with total gastrectomy

Gastric neuroendocrine tumors are rare; however,the incidence has recently increased due to the increasing use of upper endoscopy. Neuroendocrine tumors arise from the excess proliferation of enterochromaffinlike(ECL) cells. The proliferative changes of enterochromaffin cells evolve through a hyperplasiadysplasia-neoplasia sequence that is believed to underlie the pathogenesis of gastric neuroendocrine tumors. Endoscopic resection is recommended as the initial treatment if the tumor is not in an advanced stage. However,there is no definite guideline for the treatment of recurrent gastric neuroendocrine tumors following endoscopic resection. Here,we report a rare case of gastric neuroendocrine tumors in a 56-yearold male who experienced two recurrences within 11 years after endoscopic resection. The patient finally underwent a total gastrectomy. The pathological features of the resected stomach exhibited the full hyperplasia-dysplasia-neoplasia sequence of the ECL cells in a single specimen.     

Pathology diagnosis of pancreatic neuroendocrine tumors

Histopathology of pancreatic neuroendocrine tumors (PNETs) typically displays characteristic features. However, pathologists may encounter histological variants that may resemble other pancreatic tumors. Immunohistochemistry is a powerful tool in confirming neuroendocrine differentiation and differentiating PNETs with other pancreatic neoplasms. Histopathological features could be associated with inherited syndromes. Once the pathology diagnosis of neuroendocrine tumor was made, an accurate grading based on World Health Organization (WHO) classification is required. This review will focus on histology variants, immunohistochemistry and WHO classification of PNET.     

肝脏神经内分泌肿瘤报道1例并文献回顾

背景肝脏可疑恶性肿瘤行手术切除后病理诊断肝脏神经内分泌肿瘤(hepatic neuroendocrin neoplasmas,HNENs)病例,此病例易与肝癌混淆,了解转移性和原发性HNENs的特性,对临床治疗手段有很好的指导意义.病例简介一名56岁男性患者体检发现肝脏占位性病变复查腹部增强CT提示肝脏转移性恶性肿瘤可能;胃部包块考虑良性.手术切除胃部包块并行术中冰冻病理检查提示梭形细胞肿瘤;完整切除肝脏包块术后石蜡病理检查结果提示NENs(G2),患者术后恢复佳.结论本文报道了一例HNENs,考虑转移性.术前诊断不能排除肝脏转移性恶性肿瘤可能.通过对患者病情的详细分析,最终行手术切除治疗并行病理检查确诊.同时我们对HNENs相关文献进行了分析,进一步了解原发和转移性HNENs的临床特征、治疗方案以及预后,以期对此类疾病有更深入的了解,对临床治疗方案决策做证据支撑作用.     

Nonfunctioning neuroendocrine pancreatic tumors: our experience and management

Background and purpose

We present our experience in the treatment of nonfunctioning neuroendocrine pancreatic tumors (NFNPTs) to define the clinical and pathological characteristics and to suggest proper management.

Methods

The records of 17 patients with NFNPTs operated on between 1998 and 2008 were retrospectively reviewed, and all tumors were classified clinicopathologically as benign, uncertain, and malignant, based on the World Health Organization (WHO) classification of neuroendocrine tumors.

Results

There were four benign, six uncertain, and seven malignant NFNPTs. The most frequent symptoms were abdominal pain (five patients) and obstructive jaundice (one patient). Most of these symptomatic patients had malignant tumors. Mean tumor size of benign, uncertain, and malignant tumors were 1.0 ± 0.3, 3.2 ± 1.6, and 5.3 ± 2.4 cm, respectively. Metastatic lesions of malignant tumors were lymph node (six patients), liver (four patients), and adrenal gland (one patient). Six of seven patients with malignant tumors underwent curative rejection. There were recurrences in four of six patients with curatively rejected malignant tumors. Two patients underwent more rejection, three patients received systemic chemotherapy, and two patients underwent radiofrequency ablation and transcatheter arterial chemoembolization for liver metastases. Survival of patients with malignant tumors was significantly shorter than that of patients with benign and uncertain tumors. However, three patients with malignant tumors had long survival of more than 3 years, even with metastases or recurrences.

Conclusions

Aggressive surgical resection should be performed in patients with resectable NFNPTs, even with metastases. Even when a tumor was unresectable or there were recurrences, long-time palliation could be achieved by a multidisciplinary approach.     

Alkaline Phosphatase Predicts Survival in Patients with Metastatic Neuroendocrine Tumors

The clinical course of patients with metastatic neuroendocrine tumors is highly variable. While some patients experience an indolent clinical course over many years, other patients may rapidly succumb to their disease. Little is known about prognostic factors in these patients, making decisions regarding their management more difficult.We performed a retrospective analysis of 137 patients with metastatic neuroendocrine tumors referred to our institution for treatment. Potential prognostic factors were evaluated using multivariate survival analysis. The median overall survival of patients in our cohort was 6.0 years, although the range of survival times was broad (48 days to 23.4 years). Alkaline phosphatase levels above normal were predictive of shorter survival in both univariate and multivariate analysis. Elevated chromogranin A levels were also associated with shorter survival in univariate analysis; in a multivariate analysis, however, this correlation was no longer significant. There was no association between survival and gender, primary tumor site, or presence or absence of carcinoid syndrome. Elevated alkaline phosphatase is a robust adverse prognostic factor for survival in patients with metastatic neuroendocrine tumors and may be superior to chromogranin A in this setting. Close monitoring of alkaline phosphatase levels may be useful when considering initiation or changes of therapy in patients with metastatic neuroendocrine tumors.Matthew Kulke is supported in part by NIH grants K23 CA 093401, K30 HL04095, and gifts from Dr. Raymond and Beverly Sackler, the Caring for Carcinoid Foundation, and the Stephen and Caroline Kaufer Fund for Neuroendocrine Tumor Research.     

Malignant pancreatic tumor other than solid pseudopapillary tumor in pediatric patients: A single-center experience

Pancreatic tumors, except solid pseudopapillary tumors (SPTs), are rare in pediatric patients. Herein, we report various types of pancreatic tumors in pediatric patients and review the literature regarding their treatments and prognosis.We retrospectively reviewed the data of pediatric patients who underwent surgery for pancreatic tumors, excluding SPTs, between January 2009 and December 2019 at Seoul National University Children''s Hospital. A total of 35 pediatric patients were identified as having undergone surgery for pancreatic tumors. Of these patients, 30 were excluded because the tumor was identified as an SPT.The diagnoses of the five remaining (non-SPT) pancreatic tumors were pancreatic neuroendocrine tumor, mixed acinar neuroendocrine carcinoma, kaposiform hemangioendothelioma, and intraductal papillary mucinous neoplasm. All five patients survived; however, recurrence and liver metastasis were observed in one patient. The detailed demographics, treatments, and prognosis of each patient were reviewed.Despite the rarity and low incidence of pancreatic tumors in pediatric patients, four types of non-SPT tumors are reported here. Hence, the possibility of these should not be overlooked, especially since the diagnosis and adjuvant treatment differ vastly between the tumor types.     

Classification,clinicopathologic features and treatment of gastric neuroendocrine tumors

Gastric neuroendocrine tumors(GNETs)are rare lesions characterized by hypergastrinemia that arise from enterochromaffin-like cells of the stomach.GNETs consist of a heterogeneous group of neoplasms comprising tumor types of varying pathogenesis,histomorphologic characteristics,and biological behavior.A classification system has been proposed that distinguishes four types of GNETs;the clinicopathological features of the tumor,its prognosis,and the patient’s survival strictly depend on this classification.Thus,correct management of patients with GNETs can only be proposed when the tumor has been classified by an accurate pathological and clinical evaluation of the patient.Recently developed cancer therapies such as inhibition of angiogenesis or molecular targeting of growth factor receptors have been used to treat GNETs,but the only definitive therapy is the complete resection of the tumor.Here we review the literature on GNETs,and summarize the classification,clinicopathological features(especially prognosis),clinical presentations and current practice of management of GNETs.We also present the latest findings on new gene markers for GNETs,and discuss the effective drugs developed for the diagnosis,prognosis and treatment of GNETs.