Duodenal Carcinoid Research Articles

S1050 An Experience of Endoscopic Band Ligation Without Resection (EBL-WR) for Grade One, Well Differentiated Duodenal and Rectal Carcinoid Tumors

S1050 An Experience of Endoscopic Band Ligation Without Resection (EBL-WR) for Grade One, Well Differentiated Duodenal and Rectal Carcinoid Tumors

A case report of duodenal carcinoid tumor causing gastric outlet obstruction

Carcinoids are tumours of neuroendocrine origin. Commonly found in gastrointestinal and respiratory tracts, however, duodenal carcinoids among them are comparatively rare. Duodenal carcinoids presenting as acute gastric outlet obstruction are even rarer. Clinically difficult to diagnose due to their non-specific presentation. Endoscopic ultrasound (EUS), computed tomography (CT) and immunohistochemistry (IHC) for markers like chromogranin A, neuron specific enolase (NSE), synaptophysin helps in making a definitive diagnosis. Management is influenced by multiple factors like size, site, metastases and regional lymph node involvement. Here is a case report of solitary duodenal carcinoid complicated with acute gastric outlet obstruction. A middle-aged female with chronic history of intractable dyspepsia, bloating and occasional vomiting, on thorough evaluation with upper GI endoscopy, EUS guided biopsy, CT scan and histopathological examination was diagnosed of a solitary 2.2×1.2×1.6 cm sized duodenal carcinoid tumour. Patient had no past, family or genetic history supporting the diagnosis. Patient presented with features of acute gastric outlet obstruction 1 week after the diagnosis which required a distal gastrectomy with resection of first part of duodenum followed by a Roux-en-Y gastrojejunostomy with an uneventful 6 month follow up. As the size in this case was more than 2 cm with AJCC staging of T2N0M0, distal gastrectomy with Roux-en-Y gastrojejunostomy was done to ensure an R0 resection and to relieve the gastric outlet obstruction. Duodenal carcinoids presenting as gastric outlet obstruction are not common. Early management is essential to prevent complications like gastric outlet obstruction despite of indolent course of the disease.

ID: 3526378 COMPARISON OF RESECTION METHODS OF CARCINOID TUMORS OF THE DUODENUM AND RECTUM: A MULTICENTER EXPERIENCE

Historically, surgical resection has been the mainstay for treatment of duodenal and rectal carcinoid lesions. Recently, endoscopic therapy has emerged as a minimally invasive alternative. The aim of our study was to determine and compare the safety and efficacy of endoscopic and surgical resection of duodenal and rectal carcinoids.

Ressecção endoscópica de tumor neuroendócrino duodenal utilizando Padlock (Over-The-Scope clipe): relato de caso em um hospital de atenção terciária

Introdução: Com a evolução das técnicas endoscópicas e a realização de procedimentos cada vez mais invasivos, o surgimento da ressecção endoscópica de espessura total permitiu o diagnóstico e o tratamento definitivo de lesões envolvendo qualquer camada do trato gastrointestinal. O Padlock é um novo dispositivo da categoria de clipe over-the-scope (OTSCs), que foi introduzido recentemente na prática clínica. O objetivo desse estudo é relatar nossa experiência e documentar a eficácia do Padlock para a realização de ressecção endoscópica de lesões duodenais. Resumo do caso: Homem, 69 anos, apresentando quadro de dor abdominal. Endoscopia digestiva alta revelou tumor neuroendócrino de 12 mm em duodeno. A lesão foi removida por ressecção endoscópica de espessura total com auxílio do Padlock e o anatomopatológico evidenciou margens livres. Realizada revisão de literatura com discussão sobre a viabilidade, eficácia e segurança deste procedimento. Conclusão: A ressecção endoscópica de espessura total de carcinoide duodenal utilizando Padlock parece ser um procedimento viável e eficaz. No entanto, mais estudos prospectivos e randomizados são necessários para fornecer comparações significativas com as técnicas utilizadas convencionalmente e para estabelecer a segurança clínica desta nova técnica.

Risk factors for lymph node metastasis and survival of patients with nonampullary duodenal carcinoid tumors treated with endoscopic therapy versus surgical resection: analysis of the Surveillance, Epidemiology, and End Results program

Endoscopic therapy (ET) has been used to treat nonampullary duodenal neuroendocrine tumors (NAD-NETs)≤10mm in size, but data on long-term outcomes are limited. In addition, management of 11- to 19-mm NAD-NETs is not well defined because of variable estimates of risk of metastasis. We aimed to determine the prevalence and risk factors of metastasis of NAD-NETs≤19mm and evaluate the long-term survival of patients after ET as compared with radical surgery. The Surveillance Epidemiology and End Result database was used to identify 1243 patients with T1-2 histologically confirmed NAD-NETs≤19mm in size. Cancer-specific survival (CSS) and overall survival (OS) were calculated. Overall, 4.8% of cases had metastasis at the time of diagnosis, with lower prevalence in≤10-mm lesions (3.1%) versus 11- to 19-mm lesions (11.7%, P< .001). The risk factors for metastases included invasion to the muscularis propria (odds ratio, 25.95; 95% confidence interval, 9.01-76.70), age<65 years (odds ratio, 1.93), submucosal involvement (odds ratio, 3.1), and 11 to 19mm in size (vs≤10mm). In patients with well- to moderately differentiated T1-2N0M0 NAD-NETs≤19mm confined to the mucosa/submucosa who underwent ET or surgery, the 5-year CSS was 100%. The 5-year OS was similar between the≤10-mm and 11- to 19-mm groups (86.6% vs 91.0%, P= .31) and the ET and surgery groups (87.4% vs 87.5%, P= .823). In NAD-NETs, invasion to the muscularis propria is the strongest risk factor for metastasis. In the absence of metastasis, in lesions with well/moderate differentiation and without muscle invasion, ET is adequate for NAD-NETs≤10mm and is a viable option for 11- to 19-mm lesions.

Carcinoid Syndrome in a Patient with Duodenal Carcinoid Tumor

Duodenal carcinoid tumors accounts for 5% of all Gastrointestinal Neuroendocrine Tumors (GI-NETs). Only 4% of all duodenal carcinoid tumors present with a full-blown carcinoid syndrome. We report a case of duodenal carcinoid tumor presenting as carcinoid syndrome in a 58 year old man, who presented with upper abdominal discomfort, diarrhoea, hot flushes and occasional wheezing. Histopathology following endoscopic resection of the tumor and 24 hour urinary 5-Hydroxyindolacetic acid (5-HIAA) confirmed the diagnosis. Duodenal carcinoid tumors are one of the rarest tumors of gastrointestinal tract and their association with a typical carcinoid syndrome is not that common. A high level of suspicion is required for an early diagnosis. With proper resection (endoscopic or surgical) of a localized tumor, recurrence and progression of the disease can be halted. Symptoms of carcinoid syndrome should be controlled by antidiarrheal agents, inhaled β-adrenergic agonist bronchodilators and somatostatin analogs (octreotide, lanreotide). Patients should be advised to avoid stress and conditions or substances that precipitate these symptoms. Dietary supplementation with nicotinamide can help in this regard. Patients with hepatic metastases are treated with synthetic analogues of somatostatin (octreotide, lanreotide). Systemic chemotherapy is not recommended in metastatic disease by the current guidelines. Patients should be followed up by monitoring serum chromogranin and urinary 5-HIAA. EUS, CT, MRI & somatostatin receptor scintigraphy can also be used for following up the patients.

Clip band ligation ablation.

Band ligation–assisted endoscopic mucosal resection (BL-EMR) is an established procedure to remove esophageal, gastric, and rectal mucosal and submucosal lesions.1 Not infrequently, small duodenal carcinoids or neuroendocrine tumors (NETs) are found during routine endoscopy. These tumors are located in the submucosal and mucosal layers. Although there are reports of managing small duodenal NETs with BL-EMR,2 there are risks of perforation.3

S2912 Abdominal Pain as the Only Symptom of a Metastatic Duodenal Carcinoid Tumor: A Case Presentation

S2912 Abdominal Pain as the Only Symptom of a Metastatic Duodenal Carcinoid Tumor: A Case Presentation

Disección endoscópica submucosa de tumor neuroendocrino primario de duodeno. Reporte de caso y revisión de la literatura

Background. Duodenal neuroendocrine tumours are very rare, if they are limited to the mucosal layer and have no nodal involvement, endoscopic resection may be curative. Case report. We present the case of a Mexican 52 years old female with a duodenal neuroendocrine tumour who underwent successful endoscopic submucosal dissection with no complications. Pathology of the specimen revealed a complete R0 resection with negative resection margins. Endoscopic biopsies 4 weeks later showed no malignant cells. Conclusions. Because of their low frequency the management of primary duodenal carcinoid tumours is controversial; for tumours less than 1 cm endoscopic resection is recommended, for those bigger than 2 cm surgical resection is advised. Management of tumours between 1 and 2 cm is controversial and should be individualized.

Duodenal carcinoid tumour – a case report

Duodenal carcinoids are rare tumours of the small intestine with heterogenous clinical and pathological characteristics. The long-term prognosis is very good if discovered in the early stages. We present the case of a patient with a non-functional duodenal carcinoid tumour discovered incidentally during an upper gastrointestinal endoscopy. The diagnosis was confirmed through immunohistochemistry. Treatment consisted of the endoscopic resection of the tumour and the surveillance of the patient for the following 2 years, with no signs of recurrence. We have conducted a literature review regarding the clinical manifestations, diagnosis, treatment, and follow-up of patients with this type of tumours.

A Rare Case of Duodenal Carcinoid Tumors in a Patient With Atypical Symptoms

Gastrointestinal neuroendocrine tumors (NETs) are a common, yet relatively less understood subset of gastrointestinal tumors with a myriad of clinical presentations and prognostic outcomes. Although most NETs are more indolent than carcinomas, prognostic outcomes are still heavily reliant on several variables such as tumor location, stage, differentiation and metastasis amongst others. Additionally, NETs also present with a range of clinical symptoms, both localized and systemic. In this case report we discuss a patient, who had a longstanding symptom at presentation and was found to have a duodenal polyp upon endoscopy. Further evaluation established the presence of a well differentiated NET that was later resected surgically. J Curr Surg. 2020;10(1-2):7-9 doi: https://doi.org/10.14740/jcs397

Laparoscopic resection of duodenal carcinoid: A feasible method: Single institute case series

Background:Duodenal carcinoids (neuroendocrine tumour) are rare tumour, but recently, increase in incidence has been noted. Various techniques for excision of tumour have been described in literature, but very few case reports and case series have mentioned about laparoscopic management of carcinoid tumour. We describe a case series of seven cases of duodenal non-periampullary carcinoids which was managed by laparoscopic method.Aims:The aim of the study was to check feasibility of laparoscopic management of duodenal carcinoid and technique of surgery.Settings and Design:This study design was a case series and prospective data were retrospectively collected.Materials and Methods:A total of 7 patients were operated for carcinoid tumour of duodenum mainly involving first part by laparoscopic method from February 2016 to January 2017. All patients were followed up for minimum 1-year period and various pre-operative, intra-operative findings and post-operative outcome were noted.Results:Out of seven patients, 6 patient were managed by laparoscopic duodenotomy and transduodenal excision whereas one patient required duodenectomy of first part. Mean operative time was 99 min, mean intraoperative blood loss was 55.7, mean hospital stay was 99.7 and no recurrence in a 1-year follow-up.Conclusions:Laparoscopic excision of carcinoid tumour is safe, technically reproducible and feasible method.

931 Surveillance of Duodenal Carcinoids and the Importance of Tumor Margins: A Single Center Retrospective Study at the Moffit Cancer Center

INTRODUCTION: The incidence of gastrointestinal neuroendocrine tumors (GI-NETs) is increasing in recent times reflecting the widespread use of endoscopy, and an increased awareness among clinicians. Duodenal carcinoids are rare and accounting for 1%-3% of primary duodenal tumors. Duodenal carcinoids &lt; 10 mm in size and limited to the submucosal layer are amenable for endoscopic resection. Despite the increase in incidence, the natural history of duodenal carcinoid tumors has not yet been well defined. The aim of our study was to evaluate the recurrence rate following endoscopic resection of well differentiated duodenal carcinoid tumors from 2006-2016 at our institution. METHODS: We performed a retrospective analysis on 39 patients who underwent endoscopic resection of duodenal carcinoids from 2006-2016 at the Moffitt Cancer Institute. The indications for endoscopic resection were lesions ≤ 10 mm in diameter, confined to the submucosal layer, and without lymph node or distant metastasis. Endoscopic resection was accomplished using endoscopic mucosal resection (EMR) and biopsy forceps. The patients were followed for a mean of 3 years. RESULTS: Among the 39 patients meeting criteria for endoscopic resection, 28 patients underwent surveillance endoscopies post EMR and were included in final analysis . EMR was performed in 12 tumors while biopsy forceps resection in 11 patients. Pathological complete resection(R0) was achieved in 20 patients (71%), without evidence of recurrence in 9 (45%) patients followed for at least 3 years and in the 11(55%) patients followed for lesser than 3 years. On the contrary, in the 8 patients (28%) had recurrence in the 3 year follow up period of which that was recurrence,6 (75%) had either positive lateral or deep margins on initial resection (Table 1). There were no post procedural complications in any of the patients in the follow up period. CONCLUSION: Endoscopic resection appears to be a safe and effective treatment for duodenal carcinoid tumors measuring ≤ 10 mm in diameter and confined to the submucosal layer. This retrospective study showed that the main predictor of recurrence is the presence of positive tumor margins at initial resection. Patients with negative and margins had no recurrences in the 3 year follow up period, thus alleviating the need for long term surveillance in this patient population. Larger multicenter studies are required to determine the optimal surveillance stragey in patients undergoing endoscopic resection of duodenal carcinoid tumors.

2524 Rare Case of Numerous Duodenal Neuroendocrine Tumors Encompassing Entire Proximal Duodenum

INTRODUCTION: Gastrointestinal neuroendocrine tumors (GI-NETs) are rare neoplasms, but incidence is on the rise, in part due to increased detection. GI NETs can be found from the esophagus to the rectum. The appendix is the most common location.1,2 Primary duodenal NETs (D-Nets) are rare and account for less than 2% of tumors1. We report a case of a rare multiple duodenal NET. CASE DESCRIPTION/METHODS: 44-year-old female who presented for anemia complaining of belching, flatus, and loose stools. EGD found the post-bulbar area studded with numerous 1 cm nodules, covering the entire proximal colon. Endoscopic biopsy revealed a spectrum of proliferations, which followed the distribution of Brunner glands. Many of the proliferations turned in the full-blown D-NETs positive for synaptophysin and chromogranin, the Ki-67 index was less than 3%, consent with neuroendocrine neoplasm grade 1 (NET tumor), in the 1st through the 3rd portion of the duodenum. Octrotide scan was negative. The patient was sent for a Whipple procedure. MEN type 1, NF, TSH, and 24 hr urine 5-HIAA were normal. DISCUSSION: GI NETs are often diagnosed in the 5th–6th decade of life and are common in African Americans and females.3 Primary tumors in the duodenum are rare and their frequency decreases toward the distal duodenum.4 Symptoms are non-specific, presenting with dyspepsia and epigastric pain. EGD is the best method for preoperative diagnosis, neuroendocrine markers such as serum chromogranin A and urine 5-HIAA, are supportive.4 In duodenal carcinoid tumors, ∼4% are associated with carcinoid syndrome.5 Duodenal carcinoids can be associated with clinical syndromes, such as Zollinger-Ellison syndrome or Multiple endocrine neoplasia-1 3. Carcinoid tumors, with metastasis express, have a high affinity for octreotide, and can be visualized with an OctreoScan.2,3 Duodenal tumors can be removed endoscopically or with resection. Our patient underwent Whipple's pancreaticoduodenectomy due to the peri-ampullary location of the tumors (Figure 1). The current study describes a rare case of multiple D-NETs carpeting the proximal duodenum, in a patient without elevated neuroendocrine markers, ZES, or MEN. Proliferation in the distribution of the Brunner glands indicates an underlying genetic alteration in the neuroendocrine cells associated with THE Brunner glands. From our literature search, we were only able to find two other case reporting multiple D-NETs and no cases reporting D-NETs proliferations from Brunner glands, thus a rare presentation.

2663 A Rare Presentation of Duodenal Carcinoid Tumor Complicated by Hemorrhagic Shock

INTRODUCTION: A carcinoid tumor is a tumor that develops from enterochromaffin cells. These cells produce hormones and chemicals that have a profound impact on the circulatory system, gastrointestinal tract, and the lungs. Duodenal carcinoid tumors are rarely encountered in the clinical setting making up only 2–4% of all carcinoid tumors. They are also difficult to diagnose due to their nonspecific clinical presentation which include symptoms such as abdominal pain, jaundice, pancreatitis and weight loss. According to the general guidelines for the treatment of duodenal carcinoid tumors, endoscopic therapy is recommended for tumors measuring less than 1 cm in diameter, whereas surgical resection may be preferable for those measuring 1 cm or more. However, treatment guidelines for duodenal carcinoids are not yet standardized as a result of the lack of consistent data. The most feared complication of carcinoid tumors include carcinoid crisis but milder presentations may include anemia, shortness of breath and diarrhea. CASE DESCRIPTION/METHODS: A 79 year old male with a history of hypertension, hyperlipidemia, spinal stenosis, aortic stenosis and carcinoid tumor at the gastric bulb who previously refused interventional surgery presented to the hospital with a primary complaint of shortness of breath. Initial hemoglobin on presentation was 5.1 and patient noted to be in hemorrhagic shock with vital signs that included heart rate of 116 and blood pressure of 91/52. Patient was subsequently admitted to the intensive care unit and transfused a total of 6 units of packed red blood cells over the course of his 8-day hospital course. An octreotide scan performed during the hospital stay showed a carcinoid tumor at the gastric bulb that was not actively bleeding. This may be explained by the patient’s late presentation to the hospital after the carcinoid tumor bleeding had resolved. The patient at the conclusion of the hospital stay continued to decline any surgical intervention. DISCUSSION: This case highlights an unusual presentation of a patient with a history of carcinoid tumor that presented in hemorrhagic shock secondary to a duodenal carcinoid tumor. Previous research has shown complications of carcinoid tumors that range from diarrhea and shortness of breath to carcinoid crisis. However, hemorrhagic shock has not been demonstrated to be an association with carcinoid tumor. Further large scale retrospective studies may be needed to better evaluate to the full scope of carcinoid tumor complications.

P-047 - Finding the options of managing duodenal carcinoids in Pakistan. a retrospective study in a tertiary care setup

P-047 - Finding the options of managing duodenal carcinoids in Pakistan. a retrospective study in a tertiary care setup

Ligation assisted endoscopic mucosal resection of neuroendocrine tumor of duodenal bulb: a case report with a follow up of 24 months

Duodenal neuroendocrine tumor (NET) is a rare solitary lesion arising from the mucosa and submucosa of the duodenum, which is found incidentally during upper gastrointestinal endoscopy. Eendoscopic Mucosal Resection (EMR) has been the commonly used endoscopic procedure for duodenal carcinoid tumors, but the conventional EMR done to resect duodenal NET s is likely to have positive vertical margins. However, the ligation assisted EMR has recently been shown to be a promising technique for the treatment of duodenal NET that can have a negative free margin. In our study, we present a patient of 51-year-old male, who presented with pain over epigastrium and upper gastrointestinal endoscopy revealed a small submucosal lesion of 10 mm in the duodenal bulb. The endoscopic ultrasound showed the lesion arising from the echo layer three. The biopsy was taken which showed the duodenal NET. The computed tomography of abdomen did not show any evidence of distant metastasis. The EMR of duodenal NET was done by band ligation technique. The biopsy from the resected duodenal lesion confirmed the duodenal NET with the margin free of the tumor. The patient was followed at 6, 12, 18 and 24 months. During the follow up visits, the repeat upper gastrointestinal endoscopy did not show recurrence of the lesion and there was no any evidence of distant metastasis either. Ligation assisted EMR is an acceptable treatment in the hands of expert for small duodenal NET without the evidence of metastasis and can ensure the complete removal of the lesion with vertical free margin.

A rare case of duodenal carcinoid presenting as ectopic Cushing’s syndrome

ACTH-dependent Cushing syndrome (CS) due to an ectopic source is responsible for approximately 10-15% cases of Cushing’s syndrome. It is associated with various tumors such as small cell lung cancer and well-differentiated bronchial or gastrointestinal neuroendocrine tumors. Many a times ectopic ACTH production is difficult to manage, and identification of the source may take many years. Hormonal diagnostics include assessments in basic conditions as well as dynamic tests, such as the high-dose dexamethasone suppression test and corticotrophin releasing hormone (CRH) stimulation test. Treatment selection depends on the type of tumor and its extent. In the case of neuroendocrine tumors, the main treatments are surgery and administration of somatostatin analogues or bilateral adrenalectomy in refractory cases and if the source remains unidentified. Here, we report a case who presented with features of Cushing’s syndrome which eventually through workup led us to a diagnosis of duodenal carcinoid producing ectopic ACTH which is extremely rare and was successfully treated.

Carcinoids of the ampulla: Long-term follow-up after endoscopic resection.

430 Background: Neuroendocrine tumors (NET) or carcinoids of the ampulla are exceedingly rare in comparison to duodenal NET. Surgical management is widely accepted as the treatment of choice, but for patients that refuse surgery or are poor operative candidates, endoscopic resection may be option. We present a consecutive case series at a tertiary care center describing our experience with endoscopic resection of ampullary NET. Methods: This is a restrospective review with a long-term follow-up of patients with ampullary NET that were endoscopically resected. Outcomes were analyzed based on the histopathologic classification system proposed by the World Health Organization in 2000. Results: Twelve patients (9 male, 3 female), ranging in age from 41 to 86 (mean 59) underwent endoscopic ampullectomy for ampullary NET, with a mean follow-up time of 5 years. Patients had refused surgery or were poor surgical candidates. All, but one incidentally found case, were symptomatic on presentation, with gastrointestinal bleeding being the main symptom. No patients had a hormonal syndrome. The mean size of the lesions was 21 mm (6 mm to 35 cm). Six (50%) patients had a well-differentiated, benign lesion, 6 (50%) patients had a well-differentiated NET with unknown malignant potential (gangliocytic paragangliomas). Eight (67%) were completely excised during the initial endoscopy with 4 requiring re-excision. Only 2 patients developed recurrence, after 2.5 and 10 years and this necessitated a pancreaticoduodenectomy. Five patients had complications (2 for bleeding and 3 for post-ERCP pancreatitis), with zero deaths. Conclusions: Unlike duodenal carcinoids, ampullary NET are rare. Pancreaticoduodenectomy can be offered to fit patients except for gangliocytic paragangliomas that do not require an aggressive operation. However, for those that refuse surgery or are poor candidates, endoscopic ampullectomy can be an option with acceptable short and long-term outcomes.

Clinical outcomes of ligation-assisted endoscopic resection for duodenal neuroendocrine tumors.

Duodenal carcinoid tumors, a type of neuroendocrine tumors, are relatively rare and are usually found incidentally during endoscopy. Small duodenal carcinoid tumors (≤10–20 mm), embedded in the submucosa, can be resected endoscopically because of the low risk of metastasis. The aim of this study was to assess the safety and efficacy of ligation-assisted endoscopic mucosal resection (EMR) for the treatment of small duodenal carcinoid tumors. The clinical outcomes of the endoscopic procedures were also evaluated.Between November 2008 and November 2017, a total of 15 duodenal carcinoid tumors embedded in the submucosa were resected using EMR. Two types of EMR (conventional EMR and ligation-assisted EMR) were performed according to tumor morphology (narrow-based and broad-based).The mean tumor size was 6.6 ± 3.9 mm and the mean procedure time was 11.0 ± 11.2 minutes. Most of the lesions (80.0%) were located in the duodenal 1st portion. Broad-based tumors were more common than narrow-based tumors (66.7% vs 33.3%). All broad-based tumors were resected successfully using ligation-assisted EMR. Although en-bloc resection and complete resection rates were higher in ligation-assisted EMR than in conventional EMR ([100% vs 87.5%], and [85.7% vs 62.5%], respectively), the difference was not significant (P = .333 and P = .310, respectively). Moreover, there was no evidence of local or distant metastasis during the follow-up (26.1 ± 20.7 months).Ligation-assisted EMR showed a higher complete resection rate than conventional EMR. Ligation-assisted EMR may be an optimal treatment option for duodenal carcinoid tumors with a broad base.