胰腺内副脾误诊为神经内分泌肿瘤病例特点及误诊分析

目的 探讨胰腺内副脾患者的病例特点及误诊原因,以提高对该病的认识,减少误诊及不必要的手术。方法 回顾性分析2012年1月至2022年8月于中国人民解放军总医院第一医学中心肝胆外科及第二医学中心综合外科就诊、术前诊断为胰腺神经内分泌肿瘤、经术后病理确诊为胰腺内副脾的10例患者(共12个病灶)的病例资料,分析其CT、MRI、⁶⁸镓标记正电子发射计算机断层显像(⁶⁸Ga-dotatate PET/CT)图像与病理特点等。结果 10例患者中9例为单发,1例有3个病灶;12个病灶均位于胰尾,与周围组织边界清晰,为富血供结节;病灶最大径0.30～3.00 (1.43±0.75)cm。MRI表现：T1W1均呈低信号,T2W1 8例呈高信号(80%),DWI 7例呈高信号(70%);增强扫描动脉期6例呈均匀强化,只有1例呈“花斑样”不均匀强化。3例行⁶⁸Ga-dotatate PET/CT检查的患者病灶均呈高代谢,最大标准摄取值约为脾脏标准摄取值(SUV)的一半。结论 胰腺内副脾一般位于胰尾,病灶<3 cm,边界清晰;在MRI T...     

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。     

胰腺内副脾误诊为胰腺神经内分泌肿瘤1例报告

<正>1病例资料患者男性,72岁,自觉左上腹痛1个月,于外院行胃镜检查未发现明显异常,后行腹部CT提示胰腺占位性病变,考虑神经内分泌肿瘤,为求进一步诊治就诊于本院。患者病程期间无发热、寒战,无腹泻,无恶心、呕吐,血糖正常,无明显体质量下降。既往体健,无腹部及胰腺外伤史。腹部查体无阳性体征。本院腹部彩超未发现明确胰腺病灶。后又行肝胆胰脾CT平扫+三期增强,提示胰腺尾部见结节样异常强化影,大小约1.3 cm,平扫显示不清,动脉期明显强化     

胰腺异位副脾1例报告并文献复习

胰腺异位副脾临床发病罕见,术前诊断困难,易误诊为胰腺神经内分泌肿瘤。本文报告1例胰腺异位副脾病例,患者因上腹部胀痛不适就诊,完善腹部磁共振检查和CT检查示胰尾部大小约1.4 cm×1.1 cm占位,术前辅助检查结果示：胰腺占位性病变？胰腺神经内分泌肿瘤？胆囊结石伴慢性胆囊炎;胆总管结石伴胆管炎;肝功能不全。择期行内窥镜逆行性胆管胰管造影术+内镜乳头括约肌切开取石术+胆囊切除术+胰腺体尾部切除术,术后病理示：胰腺异位副脾;胆囊结石伴慢性胆囊炎。给予对症处理后患者出院,随访正常。     

胃肠神经内分泌肿瘤和胰腺神经内分泌肿瘤的区别

胃肠胰神经内分泌肿瘤（GEP-NETs）主要来源于胃肠胰系统的神经内分泌细胞,这一细胞系统和肿瘤是以表达细胞类型特异的肽激素和普通标记物（突触囊泡蛋白、铬粒素A）为特征。其特点为：临床上少见;肿瘤通常较小（〈1 cm）;生长速度较慢（数月或数年）,呈阶段性表达,可能数年无症状;通常在出现症状前即有转移,转移部位多为肝脏和骨,而此时肿瘤的体积通常〉2cm。因此这类肿瘤经常被误诊,诊断过程较复杂,不单单依靠临床,往往需要高端的实验室和扫描手段支持。而GEP-NETs中胃肠神经内分泌肿瘤（NET）和胰腺NET,两者在临床表现、诊断和治疗方面也有不同,需要临床医生重视和加以区分。     

胰腺神经内分泌肿瘤诊治进展

胰腺神经内分泌肿瘤(pNEN)是一种较为罕见的胰腺肿瘤，多为散发病例，约10%具有遗传倾向。据其是否分泌功能性激素，可分为功能性和非功能性。pNEN药物治疗可分为控制激素相关临床症状和控制肿瘤生长两类。手术是重要治疗手段之一，此外还可考虑射频消融、放射性核素治疗等方法。因pNEN诊治过程涉及多个科室，推荐多学科诊疗综合评估制定诊治方案。     

胰腺神经内分泌肿瘤的诊治进展

胰腺神经内分泌肿瘤(pancreatic neuroendocrine tumors,PNETs)是源于胰腺多能神经内分泌干细胞的一类肿瘤,故又称胰岛细胞瘤,临床少见或罕见,其发病率约为4~5/100万。该病经积极手术治疗后效果较好,但是由于PNETs自身类型多样,症状复杂多变,临床表现各异,在临床诊疗中极易造成误诊和漏诊。因此,早期正确的诊断是有效治疗PNETs的重要前提。该文针对目前胰腺神经内分泌肿瘤的诊治进展作一系统综述。     

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,...     

胰腺神经内分泌肿瘤

胰腺神经内分泌肿瘤（PNET）临床少见,表现为功能性和无功能性,来源于胰岛细胞,又称为胰岛细胞瘤（ICTs）。PNET是胰腺肿瘤很重要的一种类型,约占临床诊断胰腺肿瘤的2％-4％,包括单发、多发,良性、恶性等多种类型,其中10％～20％伴有多发内分泌肿瘤（MEN）。     

囊性胰腺神经内分泌肿瘤的临床病理特征及预后分析

回顾性分析72例行手术治疗的胰腺神经内分泌肿瘤(pNETs)患者的临床资料,其中囊性pNETs 11例,实性pNETs 61例,比较分析两组患者术前、术后的相关临床资料和病理学特征。与实性pNETs组比较,囊性pNETs多为无功能性pNETs,肿瘤多位于胰尾,体积更大,Ki-67值更低,且较少出现胰周侵犯,神经细胞黏附...     

Epidermoid cyst originating from an intrapancreatic accessory spleen

We report a rare case of an epidermoid cyst originating from an intrapancreatic accessory spleen, in a 40-year-old Japanese man with no clinical symptoms. A cystic tumor in the pancreatic tail was detected incidentally by abdominal ultrasonography. The patient was referred to the KKR Tachikawa Hospital for further examination of the tumor. Preoperative imaging findings suggested that the tumor was an epidermoid cyst originating from an intrapancreatic accessory spleen. On both pre-and post-contrast computed tomography and magnetic resonance images, the solid compartment of the tumor had the same X-ray attenuation and intensity as the spleen. Upon surgical excision, the mass consisted of solid and cystic components that were macroscopically evident on the preoperative images. Microscopic analysis revealed that the solid component was an accessory spleen in the pancreatic tail, whereas the cystic component was lined with stratified epithelium representative of an epidermoid cyst. This is the thirteenth report (in English) of an epidermoid cyst originating from an intrapancreatic accessory spleen, and the first case to be diagnosed prior to surgery.     

Epidermoid cyst in an intrapancreatic accessory spleen: a case report

Ectopic splenic tissue in the abdominal cavity is a common entity, with a reported incidence of 10% in the general population. However, an intrapancreatic accessory spleen is a rare disease, and moreover cyst formation in it is exceedingly rare. A 58-year-old woman with a 25-mm multilocular cyst in the tail of the pancreas detected incidentally by ultrasonography was admitted for further evaluation. Because malignancy could not be ruled out, a spleen-preserving distal pancreatectomy was performed. The cut surface of the surgical specimen showed a multilocular cyst surrounded by brown solid tissue resembling normal spleen. Pathological examination revealed it was stratified squamous epithelium and was surrounded by splenic tissue. The final pathological diagnosis was epidermoid cyst in an accessory spleen in the pancreas. This cyst has no characteristic features on diagnostic imaging. Consequently, it is not possible to make a definite preoperative diagnosis in most cases. Epidermoid cyst in intrapancreatic splenic tissue is another lesion to be considered in the differential diagnosis of pancreatic tail tumors.     

Epidermoid cyst of the intrapancreatic accessory spleen producing CA19‐9

We report a rare case of an epidermoid cyst in an accessory spleen at the pancreatic tail with producing CA19‐9. A 55‐year‐old female was admitted to our hospital, Cancer Research Institute, Kanazawa University, for close examination of a cystic lesion at the pancreatic tail and a high serum CA19‐9‐value (176 U/mL). There were almost no abdominal symptoms related to the cystic lesion. A cystic tumor approximately 3 cm in diameter and composed of multilocular cysts without a protruding portion of the inner surface was found at the pancreatic tail by ultrasound sonography, computed tomography, and magnetic resonance imaging. Endoscopic retrograde pancreatography revealed that the main pancreatic duct shifted at the pancreatic tail and there was no communication between the main pancreatic duct and cystic lesion. Based on a preoperative diagnosis of mucinous cystic tumor, distal pancreatectomy with splenectomy was performed. Histological ?ndings suggested an epidermoid cyst (3.5 × 3.0 cm) originating from an intrapancreatic accessory spleen. Immunohistochemical analysis of CA19‐9 in the epidermoid cyst showed clear staining of the inner epithelium of the cyst and amorphous or hyalinous cystic contents. The serum CA19‐9 value was con?rmed to decline to normal 2 months after resection. Physicians should not forget this disease during differential diagnosis related to pancreatic cystic lesions with elevated levels of serum tumor markers, such as CA19‐9 or carcinoembryonic antigen, although this disease is extremely rare.     

胰腺异位副脾的MDCT特征

目的 总结胰腺异位副脾(IPAS)的多层螺旋CT(MDCT)征象,提高对该病的认识和诊断正确率.方法 收集术前行MDCT检查并经手术和病理确诊的7例IPAS患者资料,回顾性分析IPAS的MDCT特征性表现,包括病变的位置、大小、形态、边界、密度、强化特点.结果 本组男性4例,女性3例,平均年龄49岁.所有病灶均位于胰腺尾部被膜下胰腺实质内偏背侧.病灶呈类圆形3例,椭圆形4例,边界清晰.病变均为实性结构,内无坏死区,未见钙化灶.病灶最大直径0.9～1.8 cm,平均为1.4 cm.与胰腺实质相比,CT平扫时肿块实质密度均较均匀,动脉期呈不均匀稍高密度3例,均匀稍高密度4例,门脉期均呈均匀稍高密度.平扫时肿块CT值范围50～ 61 HU,平均55 HU;动脉期CT值范围80～110 HU,平均97 HU;门脉期CT值99～ 120 HU,平均102 HU.行血管成像的3例患者,不管是动脉还是静脉均未见受压、被侵犯征象,未见有血管与病灶相连.结论 IPAS具有特征性的MDCT影像表现,对于胰腺尾部较小实性病灶,其密度及强化方式与脾脏相似时应考虑IPAS的可能.     

Epidermoid cyst in intrapancreatic accessory spleen: A systematic review

Background/Objectives

Due to its rarity, epidermoid cyst in intrapancreatic accessory spleen (ECIPAS) is still a diagnostic dilemma during clinical practice. The aim of this review was to summarize the epidemiologic features and management of ECIPAS.

Surgical strategies and predictors of outcome for malignant neuroendocrine tumors of the pancreas

BACKGROUND AND AIM: Neuroendocrine pancreatic carcinomas are rare and little information on factors influencing the clinical course and prognosis is presently available. The aim of this study was to retrospectively review all patients who underwent pancreatic resection for malignant neuroendocrine tumor of the pancreas at the one department from 1989 to 2003. METHODS: Eleven male and eight female patients with a mean age of 51 years (range 13-76 years) underwent surgery for malignant neuroendocrine tumor of the pancreas. The prognostic relevance for long-term survival was investigated for intrapancreatic localization of the primary, histological classification including proliferation index (Ki67), lymph node involvement, surgical treatment and long-term survival after resection. The clinical course after resection was also evaluated. Statistical analysis was performed using multivariate analysis and Kaplan-Meier method. RESULTS: Functional or non-functional tumors occurred in six (32%) and 13 (68%) patients, respectively. The tumors were located in the pancreatic head in 10 patients (53%), body in three (15%) and tail in two (11%). Multilocular tumors were found in five (26%). Surgical procedures performed were six pylorus preserving pancreaticoduodenectomies (32%), four standard pancreaticoduodenectomies (21%), four distal pancreatectomies (21%), three total pancreatectomies (15%) and two segmental resections (11%). Multivariate analysis showed sex (P = 0.018), Ki67 proliferation index (P = 0.023), tumor diameter (P = 0.02) and tumor site (P = 0.011) as significant predictors of outcome. CONCLUSION: Malignant neuroendocrine tumors of the pancreas are associated with poor prognosis. Surgical resection is an appropriate and safe procedure with low morbidity rates. The prognosis seems to be determined by various biological factors. However, with regard to the principles of surgical oncology, tumor-free resection margins are important and radical surgical procedures are justified in selected patients.     

Giant epithelial cyst of the accessory spleen

Splenic cysts are uncommon, and cysts derived from the accessory spleen are rare. We report a case of a huge splenic cyst derived from the accessory spleen in the omentum, concomitant with multiple epithelial cysts of the primary spleen. Both serum and cystic fluid concentrations of carbohydrate antigen (CA)19-9 were markedly elevated. A huge monolocular cyst occupied the entire abdomen. A total of 7?l of aspirated cystic fluid was turbid and yellowish-brown. The cyst wall, which consisted of CA19-9-positive squamous epithelium, contained approximately 2 × 3 × 1?cm of splenic tissue. There was a separate multicystic 16 × 8-cm spleen in the normal position. The CA19-9 serum level returned to normal postoperatively.     

Preliminary results of a Japanese nationwide survey of neuroendocrine gastrointestinal tumors

Background We conducted a nationwide survey to estimate the incidence of neuroendocrine gastrointestinal tumors (NETs) newly diagnosed in Japan from 2002 through 2004. Methods Data on 1541 patients, 514 pancreatic endocrine tumors (PETs) and 1027 gastrointestinal carcinoids (GICs), were collected and analyzed. Results Nonfunctioning tumors (NF-PET) constituted 47.7% of PETs. Next in frequency were insulinoma (31.7%) and gastrinoma (8.6%). Malignancy was frequent in NF-PETs (46.1%) and gastrinomas (45.5%), but only 7.4% of insulinomas were malignant. The incidence of multiple endocrine neoplasia type-1 associated with PETs was 7.4%. The incidence of GICs was 28.8%, 5.2%, and 66.0% in foregut, midgut, and hindgut, respectively. Carcinoid syndrome and metastases were observed in only 1.7% and 5.6% of GICs, respectively. Conclusions The incidence of NETs in Japan was clarified by this preliminary study. Comparatively large differences in GICs between Japan and Western nations were present with regard to the location, symptomatic status, and prevalence of malignancy.     

Genetics of pancreatic neuroendocrine tumors: implications for the clinic

Pancreatic neuroendocrine tumors (PanNETs) are a common and deadly neoplasm of the pancreas. Although the importance of genetic alterations in PanNETs has been known for many years, recent comprehensive sequencing studies have greatly expanded our knowledge of neuroendocrine tumorigenesis in the pancreas. These studies have identified specific cellular processes that are altered in PanNETs, highlighted alterations with prognostic implications, and pointed to pathways for targeted therapies. In this review, we will discuss the genetic alterations that play a key role in PanNET tumorigenesis, with a specific focus on those alterations with the potential to change the way patients with these neoplasms are diagnosed and treated.