Giant insulinoma in a 15-year-old man: A case report

IntroductionGiant insulinomas are extremely rare pancreatic neuroendocrine tumor.Presentation of caseA 15-year-old man presenting with acute onset of lightheadedness was found to have serum glucose of 1.5 mmol/L. The blood collected from the hypoglycemic episode showed an inappropriately high insulin and C-peptide level. Abdominal computerized tomography showed a 12.5 cm well-defined, lobulated hypervascular mass at pancreatic tail, without any evidence of metastasis. En bloc resection with distal pancreatectomy, and splenectomy was successfully performed. The pathological examination confirmed insulinoma, with benign characteristics. Follow-up after the procedure revealed neither hypoglycemic, nor hyperglycemia.ConclusionWe report the youngest case of a giant insulinoma. Despite the size of the tumor, the pathological report confirmed the benign characteristics. However, long-term follow-up is still essential to detect recurrence in the future.     

Laparoscopic enucleation of a pancreatic insulinoma

Insulinoma is the most common functional neuroendocrine tumor of the pancreas [2]. In most cases the lesions are benign, solitary, and located within the pancreatic parenchyma. Because of these characteristics, the majority of these lesions can be treated with simple enucleation [2]. Advances in laparoscopic techniques have recently enabled the safe resection of pancreatic islet cell tumors [1] and may provide patients with the benefits of minimally invasive surgery. This video demonstrates the technique of laparoscopic enucleation of a pancreatic insulinoma. The case presentation is that of a 40-year-old man who had symptoms of neuroglycopenia and was found to have elevated proinsulin levels during a 72-hour fast. Further evaluation included a CT scan, which revealed a 1.5 cm lesion on the posterior surface of the midbody of the pancreas. The video shows the operative technique of enucleation of the lesion, including positioning and trocar placement, performance of intraoperative ultrasound for tumor localization, and the use of specialized instruments (laparoscopic freer-elevator with a spatulated tip) that allowed enucleation of the lesion without excess handling of the tumor itself. The operation was performed in 105 minutes with minimal blood loss. The patient was fed clear liquid diet on the day after surgery and was discharged home on the third postoperative day. He had an uneventful recovery and has experienced no further symptoms. This multimedia article (video) has been published online and is available for viewing at http://www.springerlink.com. Its abstract is presented here. As a subscriber to Surgical Endoscopy you have access to our SpringerLink electronic service, including Online First.     

Pancreatic insulinoma combined with glucagon positive cell: A case report

We present a 70-year-old man who was referred for surgery with uncontrollable hypoglycemia. Ultrasonography and abdominal contrast computed tomography revealed a hypervascular tumor of 1 cm in diameter in the pancreatic tail. With a diagnosis of insulinoma, we performed a distal pancreatectomy. The patient showed a good postoperative course without any complications. The patient’s early morning fasting hypoglycemia disappeared. The respective levels of C-peptide and insulin dropped from 14.9 ng/mL and 4860 μIU/mL preoperatively to 5.3 ng/mL and 553 μIU/mL after surgery. A histopathological examination demonstrated that the tumor was a pancreatic neuroendocrine tumor, grade 1. Immunostaining was negative for insulin and positive for CD56, chromogranin A, synaptophysin and glucagon. These findings suggested that the tumor was clinically an insulinoma but histopathologically a glucagonoma. Among all insulinoma cases reported between 1985 and 2010, only 5 cases were associated with independent glucagonoma. In this report, we characterize and discuss this rare type of insulinoma by describing the case we experienced in detail.     

Post-splenectomy intrapancreatic accessory spleen mimicking endocrine tumor of the pancreas

INTRODUCTION

Intrapancreatic accessory spleen is an uncommon congenital abnormality of the spleen with no indication for surgical intervention. Among the few cases reported, IPAS coexisted with a normal spleen. We here report the first case of IPAS arising a couple years after splenectomy with the appearance of an endocrine tumor of the pancreas.

PRESENTATION OF CASE

A 62-year-old female presented with a one-week history of left upper quadrant discomfort. She had splenectomy for the treatment of hypersplenism caused by cirrhotic portal hypertension two years before this admission. Her physical examination was unremarkable and laboratory data was within the normal range. Both the ultrasonography and magnetic resonance image revealed a small oval-shaped mass in the tail of her pancreas with the diameter 2 cm or less. A distal pancreatectomy was performed for the suspection of malignant neuroendocrine tumor of the pancreas. An intrapancreatic accessory spleen was confirmed by the pathologic examination.

DISCUSSION

Intrapancreatic accessory spleen is one kind of congenital ectopic spleen without indication for operative intervention. We present the case to support that intrapancreatic accessory spleen may enlarge through a compensatory mechanism, and raise the awareness of this intrapacreatic entity to avoid unnecessary surgical operation.

CONCLUSION

IPAS should be highly considered as a differential diagnosis while the lesion is no more than 2.5 cm in diameter and/or other accessory spleens show around the splenic hilum.     

全球1999—2021年间关于胰岛素瘤的文献计量分析

背景与目的：胰岛素瘤是胰腺最常见的功能性内分泌肿瘤。然而很少有关于胰岛素瘤的文献计量学研究。因此本研究将通过文献计量分析这一方法描述近20年来胰岛素瘤领域研究的热点和趋势。方法：通过Web of Science核心数据库（WoSCC）检索1999—2021年间与胰岛素瘤相关的出版文献，并以纯文本格式导入VOSviewer和CiteSpace软件。数据使用文献计量方法进行处理，对作者、国家、机构、高被引文献、共被引、关键词和参考文献进行文献可视化分析。结果：共检索到3 863篇文献，包括19 310位作者，3 268个组织，83个国家/地区和1 005种期刊，文献共引用了来自7 494种期刊合计55 619位作者的85 078篇文章。其中，相关研究主要在美国进行，Lernmark A是最高产的作者，华盛顿大学是最主要的贡献机构，Journal of Biological Chemistry是胰岛素瘤领域发文的主要期刊。关键词分析显示，目前重点主要集中在“Pancreatic Neuroendocrine Tumor”“ENTS Consensus Guideline”“Marker”“M...     

Laparoscopic enucleation of a solitary pancreatic insulinoma

Insulinomas are usually small, benign tumors of the pancreas, often found in obese patients, which require an incision that is out of all proportion to the size of the lesion. A laparoscopic technique for enucleation of a pancreatic insulinoma is described. Received: 4 October 1994/Accepted: 18 September 1995     

Mixed Duct-Acinar-Islet Cell Tumor of the Pancreas: Report of a Case

A 72-year-old Japanese woman presented at our hospital complaining of altered consciousness on arising every morning. The laboratory findings showed hypoglycemia and hyperinsulinemia. Abdominal ultrasonography revealed a tumor in the body of the pancreas. With a diagnosis of insulinoma, a surgical excision of the tumor was performed. A light microscopic examination and an immunohistochemical study revealed the tumor to consist of duct, acinar, and islet cell components. Mixed tumors of the pancreas are rare, and their clinical features and pathogenesis remain unclear. A further accumulation of clinical cases as well as a large number of histopathological studies on these rare mixed tumors is needed. Received: March 2, 2000 / Accepted: July 25, 2000     

Pancreatic schwannoma: A rare case and a brief literature review

IntroductionPancreatic schwannoma (PS) is an extremly rare benign tumor. Less than 50 cases of pancreatic schwannoma have been described in the English literature over the past thirty years.Presentation of case reportA 63-year-old female underwent left modified radical mastectomy 2 years ago due to breast cancer. During her routine check-up, a 65 × 63 × 55 mm measured calcified, well-demarcated, cystic-mass having septations and calcifications that localized to the pancreatic head was detected by abdominal computerized tomography. She was asymptomatic and her tumor markers were in normal ranges. A standard Whipple procedure was performed, and the histo-pathological diagnosis of the resected specimen was reported as ancient schwannoma with clear surgical margins. Patient’s postoperative course was eventful. She had a biliary leakage after surgery which was managed conservatively. She is under follow-up.DiscussionPancreatic schwannoma also known as neurilemoma or neuroma is a slowly growing, encapsulated, mostly benign tumor with smooth well-delineated margins that originates from myelin producing schwann cells located on the nerve sheath of the peripheral epineurium of either the sympathetic or parasympathetic autonomic fibers. PS’s are extremly rare. The head of pancreas being involved in the vast majority of cases (40%), followed by its body (20%). Management of pancreatic schwannomas remains largely controversial. Both enucleation and radical surgical resections have revealed great therapeutic efficiency. with a well prognosis without recurrences.ConclusionAlthough rare, PS’s should be considered in the differential diagnosis of the other solid or cystic masses of the pancreas.     

胰岛素瘤64例的诊断与外科治疗

目的 总结胰岛素瘤的诊断与外科治疗方法.方法 回顾性分析64例胰岛素瘤的临床资料.结果 64例均表现Whipple三联征.术前BUS、CT及强化CT、MRI、DSA诊断阳性率分别为46.9%(30/64),58.2%(23/39),66.7%(18/27),91.7%(11/12).IOUS诊断阳性率为92%(23/25).单个肿瘤58例,多发肿瘤6例.单发者位于胰头19例,其中直径4cm 1例,胰体17例,胰尾22例;多发者6例均为2枚肿瘤,4例位于胰体,2例分别位于胰体和胰尾各1枚.治疗行肿瘤局部切除39例,胰体尾切除13例加作脾切除6例,胰尾切除8例加作脾切除4例,胰体表面肿瘤直径2cm行腹腔镜下单纯肿瘤摘除1例,自左向右分段切除(盲切法)2例,行胰头十二指肠切除1例.良性肿瘤62例,恶性2例.术后胰瘘3例、急性胰腺炎4例均经非手术治愈.64例术后低血糖症状消失.62例良性胰岛素瘤术后随诊1～5年血糖正常,其中2例分别于术后4年和5年复发,再次手术发现胰尾近脾门处分别有直径1cm和1.5cm肿瘤,经胰尾切除后治愈.45例随访8年血糖正常,17例失访.2例恶性胰岛素瘤分别于术后3年和4年复发,因肝转移死亡.结论 Whipple三联征和测定IRI/G＞0.3是定性诊断的依据.术中触诊联合IOUS是最有效的肿瘤定位诊断方法.胰岛素瘤切除术是最佳的治疗方法.     

胰岛素瘤诊治分析：附72例报告

目的:探讨胰岛素瘤的诊断和外科治疗方法。方法:回顾性分析我院31年余诊治的72例胰岛素瘤的临床资料。结果:84.72%有典型的Whipple三联征表现。80.56%血浆免疫反应性胰岛素/血糖(IRI/G)比值>0.3。术前定位诊断方法的阳性率分别为:腹部超声78.13%,CT(平扫或增强)60.97%,多排螺旋CT胰腺灌注100%,MRI 37.50%,内镜超声(EUS)72.73%,选择性动脉造影(DSA)28.57%。术中超声联合扪诊诊断阳性率92.31%。肿瘤最大直径≤2 cm者占88.75%。37.5%的肿瘤位于胰头颈部,27.50%位于胰体部,35.0%位于胰尾部。81.58%的病例可行胰岛素瘤局部剜除术。病理诊断均为胰岛素瘤,65例(90.28%)为功能性胰岛素瘤,7例(9.72%))为无功能性胰岛素瘤;4例(5.56%)为多发性肿瘤,2例(2.78%)恶性倾向,1例(1.39%)合并多发性内分泌肿瘤1型(MEN-1)。结论:Whipple三联征和IRI/G>0.3可作为胰岛素瘤定性诊断的主要依据。应联合应用超声,CT,多排螺旋CT胰腺灌注,MRI,内镜超声和DSA多种方法进行术前定位。术中超声联合扪诊是简单有效的定位诊断方法。肿瘤的局部剜除术是多数胰岛素瘤的最佳手术治疗方式。     

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目的总结胰岛素瘤的外科治疗经验。方法回顾性分析1983～2002年收治的128例胰岛素瘤的临床资料。结果肿瘤局部摘除术适用于75.8％的病人；胰瘘是胰岛素瘤手术后最常见的并发症。结论胰岛素瘤的手术方式应该根据病变的性质、部位、数量等因素决定；必要的术前定位检查有助于术中选择合理的手术方式。     

Pancreatic neuroendocrine tumor with splenic vein tumor thrombus: A case report

INTRODUCTION

Pancreatic neuroendocrine tumors (PNET) are rare, often indolent malignancies. PNET are classified as functional or nonfunctional based on the secretion of hormones without a negative feedback loop; the latter account for up to 60% of PNET. Although PNET are associated with a better prognosis compared to pancreatic adenocarcinomas, they are often diagnosed in advanced stages, making them a significant source of morbidity for patients. Here we present a rare case of venous tumor thrombus arising from a nonfunctional PNET.

PRESENTATION OF CASE

A 44-year-old woman was referred for evaluation and treatment of a possible tail of pancreas PNET discovered during work-up for a 9 year history of intermittent subcostal pain. Previous endoscopic ultrasound with fine needle aspiration revealed a 3.5 cm × 3 cm mass, with cytological diagnosis of neuroendocrine tumor. Patient was scheduled for laparoscopic distal pancreatectomy. During surgery the mass was found to encase the splenic vein leading the surgeon to perform an en bloc distal pancreatectomy and splenectomy. Pathologic analysis revealed a 1.8 cm × 5 cm tumor thrombus lodged in the splenic vein.

DISCUSSION

Nonfunctional PNET usually present in advanced stages and can be associated with venous tumor thrombi. Preoperative imaging may not accurately predict the presence of venous tumor thrombi.

CONCLUSION

En bloc resection of primary tumor, involved organs and thrombus is the recommended treatment option and often results in long term survival. New multi-modality strategies are needed for detection of venous involvement in nonfunctional PNET to better assist with preoperative planning and counseling.     

胰岛素瘤的诊断和治疗：附137例报告

目的总结胰岛素瘤的诊断方法和外科治疗效果。方法回顾性分析近26年来收治的胰岛素瘤137例患者的临床资料。结果男77例,女60例。137例均具有典型的Whipple三联症。B超,CT,MRI检查的阳性率分别为35.1%,67.9%,58.1%。126例手术治疗患者中,102例(80.9%)行胰岛素瘤摘除术,4例(3.2%)行胰十二指肠切除术,16例(12.7%)行胰体尾切除术,另外4例(3.2%)行开腹探查术。切除的122例肿瘤中,良性占96.7%,恶性3.3%;单发肿瘤98.4%,多发1.6%。86.9%肿瘤直径≤2.0cm。13.1%肿瘤位于胰头;位于胰体和胰尾者分别为46.7%和40.2%。结论Whipple三联症,结合IRI,IGR,C-肽,以及胰岛素原的检测水平,可作为胰岛素瘤的定性诊断依据。胰岛素瘤的定位诊断仍很困难,联合应用多种影像学检查方法有助于提高检出率。治疗首选肿瘤摘除术,如果术中探查未能找到肿瘤,不宜盲目行胰体尾切除术,应排除其他原因所致低血糖症,同时进一步完善影像学检查方法,明确定位后,再考虑二次手术治疗。     

胰岛素瘤定位诊断方法的选择与评价

目的分析胰岛素瘤定位诊断的方法，探讨合理选用的策略。方法对1990年1月至2005年2月北京协和医院收治的137例胰岛素瘤的临床资料进行回顾性分析。结果本组126例行手术治疗，除2例外术中均发现肿瘤，包括11例定性诊断明确，但无定位诊断的病人。术前BUS、EUS、CT、MRI、DSA和ASVS定位的阳性率分别为18．3％（23／126）、68．4％（13／19）、48．5％（48／99）、20％（3／15）、83．3％（70／84）和88．2％（15／17）。结论胰岛素瘤术前定位诊断有助于手术方式的选择，可首选相对无创的增强CT和EUS检查，仍然无法获得定位的病人可选择DSA或ASVS。对定性诊断明确的病人，无论能否获得定位诊断，均应及早手术探查，以免延误治疗。     

无创性检查在胰岛素瘤定位诊断中的价值

目的 探讨无创性检查方法在胰岛素瘤定位诊断中的价值.方法 回顾性分析北京协和医院2005年1月至2008年11月手术治疗的88例胰岛索瘤患者的临床资料.其中男性40例,女性48例;年龄15～74岁,平均46.5岁.结果 B超、增强CT、多排螺旋CT胰腺灌注、磁共振成像、奥曲肽显像、超声内镜、腹腔镜超声和术中超声的诊断阳性率分别为19.3%(17/88)、52.4%(11/21)、95.5%(64/67)、1/6、30.0%(6/20)、83.9%(26/31)、8/8和5/5.其中8例多发胰岛素瘤患者共切除肿瘤31个,多排螺旋CT胰腺灌注、术中超声的定位诊断准确率分别为48.4%(15/31)和100%(14/14).结论 目前胰岛素瘤的术前定位诊断已进入无创检查时代,应首选多排螺旋CT胰腺灌注.对于多发性胰岛素瘤的患者,术中超声在定位诊断中具有重要价值.     

胰岛素瘤腹腔镜外科治疗体会

目的 评估腹腔镜胰岛素瘤切除术的可行性和安全性。方法 2002年6月至2004年6月25例胰岛素瘤患者,分别行腹腔镜胰岛素瘤切除术（腹腔镜组,10例）和开腹胰岛素瘤切除术（开腹手术组,15例）,比较2组手术时间、术中出血量、术后住院天数和并发症发生率差异是否有统计学意义。结果 肿瘤发生部位、大小差异无统计学意义,手术时间、术中出血量和术后平均住院天数等差异均无统计学意义（P〉0．05）。并发症发生率方面,腹腔镜手术组仅1例并发胰瘘,开腹手术组3例并发胰瘘、2例并发腹腔感染、5例并发胸腔积液,开腹手术组并发症发生率显著高于腹腔镜手术组（P〈0．01）。结论 位于胰体或尾部的胰岛素瘤行腹腔镜下胰岛素瘤切除术是安全可行的,并且并发症发生率低于经典的开腹手术。     

胰岛素瘤外科诊断与治疗的变革

Insulinoma is derived from beta cells, and the yearly incidence of insulinoma is 1-4 per one million. Insulinoma patients were often misdiagnosed with epilepsy or cerebrovascular diseases because of the clinical and epidemiological features of insulinoma. The diagnosis of the insulinoma is usually made biochemically with the presence of low blood glucose ( <2.5 mmol/L), elevated insulin ( ≥6 mU/L) and C-peptide levels ( ≥ 200 pmol/L), and no sulfonylureas in the blood.Supervised 72-hour fasting test has been verified as the gold standard in establishing a biochemical diagnosis of insulinoma.Localization of insulinoma is useful for selecting surgical procedures, and the methods for localization can be divided into noninvasive (transabdominal ultrasound, computed tomography,magnetic resonance imaging and endoscopic ultrasound), invasive (angiography and arterial stimulation venous sampling) and intraoperative diagnosis. Surgical treatment is the only curative method at present, and the common approaches include enuclea tion, partial pancreatic resection, resection of the body and tail of pancreas and duodenum-preserving pancreatic head resection.Most patients with sporadic insulinoma had long-term survival after the surgery. For insulinoma patients with multiple endocrine neoplasia type 1, an aggressive surgical approach is recommended.     

胰岛素瘤诊治分析：附22例报告

目的：探讨胰岛素瘤的诊断和治疗。方法：对2008年1月—2013年1月收治并手术的22例胰岛素瘤患者的临床资料进行回顾性分析。结果：良性胰岛素瘤21例（95.5%）,恶性1例（4.5%）;患者均有不同程度的Whipple三联征表现,4例有不同程度的精神神经症状;检测空腹血胰岛素15例,胰岛素释放指数均>0.3;术前B超,CT,MRI,生长抑素受体显像的肿瘤检出情况分别为22.7%（5/22）,54.5%（12/22）,53.8%（7/13）,100%（1/1）,术中B超检出率为100%（10/10）。行肿瘤摘除术18例,胰体尾切除2例,胰体尾+脾切除2例。术后21例患者血糖恢复正常,1例术后仍有持续的低血糖症状,3个月后行二次手术探查发现位于胰腺钩突一肿瘤,切除后血糖恢复正常;l例术后仍留有精神神经症状;发生胰瘘1例,经充分引流和抑制胰腺分泌治疗于3周后痊愈。结论：Whipple三联征和胰岛素释放指数>0.3是胰岛素瘤定性诊断的主要依据。术前CT和MRI等检查定位+手术探查、术中B超定位是有效的定位诊断方法。手术是治愈胰岛素瘤最重要的方法。

     

Clinicopathological features and long-term prognosis of purely cystic pancreatic neuroendocrine tumors: A single-center experience

BackgroundPancreatic neuroendocrine tumors (pNETs) are heterogenous neoplasms, of which the prognosis varies widely. Purely cystic pancreatic neuroendocrine tumors (C-pNETs) are a small subset of pNETs in which data are extremely rare. This study aimed to compare clinicopathological and long-term survival differences between C-pNETs and solid pNETs (S-pNETs).MethodsA retrospective review of 242 patients with pNETs underwent resection in our institution from 2009 to 2019 was conducted. Demography characteristics, clinicopathological features and long-term outcomes of them were analyzed.ResultsSixteen out of 242 patients (6.6%) were identified as C-pNETs. Compared with S-pNETs, C-pNETs were more frequently non-functional (75% vs 45%, P = 0.02), and the median tumor diameter of C-pNETs was smaller (36 mm vs. 47 mm, P = 0.001). And the accuracy of preoperative diagnosis of C-pNETs was significantly lower (31% vs 78%, P = 0.001). Of note, the majority of C-pNETs were well-differentiated with G1 (81% vs 35%, P = 0.001). And there were no G3 (0 vs 7%, P = 0.001) in C-pNETs. No T4 stage or R1/R2 surgical margin detected in C-pNETs. And only one C-pNETs (6%) had regional lymph node metastasis (N) or synchronous distant metastasis (M). Additionally, only one patient with C-pNETs (6%) suffered tumor recurrence, compared with 24 (13%) for S-pNETs. And survival analysis showed the patients with C-pNETs seemed to be with better disease-free survival (P = 0.26).ConclusionC-pNETs are rare subtype with possibly less aggressive behavior comparing with their solid counterparts. Recurrence and tumor-related death still occurs in patients with resected C-pNETs, although they tend to be with more favorable prognosis.