Ampullary Carcinoid Tumor Research Articles

Duodenal and Ampullary Carcinoid Tumors: Size Predicts Necessity for Lymphadenectomy

BackgroundUnlike other neuroendocrine tumors of the gastrointestinal tract, management of duodenal and periampullary carcinoids remains controversial. We aimed to determine the metastatic potential and optimal choice of therapy for these neoplasms. MethodsA retrospective review of all patients treated at the Johns Hopkins Hospital between 1996 and 2012 was conducted. Clinicopathologic factors associated with lymph nodal involvement and clinical outcomes were evaluated. ResultsA total of 101 patients were identified. Eighty (79.2%) tumors arose from the duodenum and 21 (20.8%) from the periampullary area. Thirty-five (34.7%) patients underwent pancreaticoduodenectomy (PD), 12 (11.9%) local resection, 38 (37.6%) endoscopic excision, and 16 (15.8%) patients harbored incidental tumors identified in the specimen after PD for another indication. Lymph node (LN) pathologic evaluation was done in 56 patients, among which 27 (48%) had positive LN. Specifically, LN positivity (LN+) for tumors <1 cm in size was 4.5% (1/22), for tumors 1–2 cm 72% (13/18), and for tumors >2 cm 81% (13/16). Tumor size was the only factor associated with LN+ (p = 0.029). ConclusionLymph nodal involvement is common for duodenal and periampullary carcinoid tumors, particularly among those >1 cm in size; therefore, resection with lymphadenectomyfor these larger tumors is recommended.

A Rare Case of Ampullary Carcinoid Tumor: Emphasizing the Need for EUS for Diagnosis

Introduction: Ampullary carcinoids are extremely rare neuroendocrine tumors, comprising <0.05% of all gastrointestinal carcinoids and <2% of all ampullary tumors. Case Report: A 75-year-old white woman presented with nausea/vomiting with abdominal pain for 1 day, preceded by dizzy spells of 2 weeks’ duration. She had prior history significant for coronary bypass surgery almost a decade ago, and hypertension. The vitals were stable and initial lab work, including complete blood count, kidney profile, amylase/lipase, and liver function tests were unremarkable. The patient was unable to get an MRI due to recent shoulder prosthesis, and a CT with a pancreas protocol was obtained, which showed severe intra- and extra-hepatic biliary ductal dilation with a 1.1 x 0.5-cm mass near the ampulla, raising concerns for possible neoplastic process. Endoscopic ultrasound (EUS) imaging revealed a very dilated (1.2 cm) CBD that tapered towards a bulbous ampulla. During ERCP, a sphincterotomy was performed; there was no evidence of any strictures and biopsies of the bulbous ampulla were obtained. Immunohistochemical stains revealed that the tumor cells expressed pancytokeratin AE1/AE3 (patchy), synaptophysin (strong and diffuse), chromogranin (patchy), but congo red stain excluded any evidence of amyloid deposition. There was no increased mitotic activity, and overall findings supported well-differentiated neuroendocrine tumor (carcinoid tumor). Discussion: Ampullary carcinoid tumor was first described in 1888 by Lubarsch. The most common presenting symptoms may include obstructive jaundice (>60%), abdominal pain (<40%), weight loss (<10%), and pancreatitis (˜5%). The sensitivity of octreotide scintigraphy decreases with smaller size of tumor and decreased tumor somatostatin receptor expression. Endoscopic biopsy during EGD has limited value and has a high failure rate due to the sub-epithelial nature of this tumor. Diagnosis can be established by histological and immune-histochemical analysis of lesion with EUS-FNA. EUS is superior to CT and equivalent to MRI for tumor staging. Unlike other midgut carcinoids, the size of the ampullary carcinoid is a poor predictor of metastatic potential, with varying rates of metastasis at different tumor sizes. Since these tumors have a high propensity of lymph node metastasis, unlike other duodenal carcinoids, local resection of ampullary carcinoid is technically difficult and pancreatico-duodenectomy has been suggested for complete resection of the lesion. Predicting metastases among small intestinal carcinoids using a gene expression profiling (GEP)-based mathematical model is an emerging strategy to improve detection accuracy.

Duodenal and ampullary carcinoid tumors: Using size to predict necessity for lymphadenectomy.

316 Background: The metastatic potential and choice of therapy of duodenal and ampullary carcinoid tumors are poorly understood. We evaluated the local management and outcomes in patients with these uncommon tumors and determined factors predicting risk of nodal involvement. Methods: 117 patients were identified with duodenal or ampullary carcinoid tumors between 1996 and 2012, who were treated in a single high-volume center. Clinicopathologic data and overall survival by local treatment modality were analyzed. Results: Among all patients, 64 (55%) were treated surgically, including 34 (29%) who underwent pancreaticoduodenectomy (PD), 14 (12%) who underwent local resection (partial duodenectomy), and 16 (13.6%) where a carcinoid tumor was found incidentally after PD for another indication. The remaining 53 patients (45%) underwent endoscopic excision. The average tumor size was 1.8 cm (0.1-8.5) and the majority were of duodenal origin (n=93, 80%). Surgical management was more commonly performed for ampullary tumors compared to tumors of duodenal origin (83% vs. 47%, p=0.002), and endoscopic excision was more common with smaller tumors (p=0.001). Most carcinoids were well-differentiated (94%) and 55% were T1/T2. Yet, among the 55 patients in whom lymph nodes were histologically assessed (PD or lymph node sampling), 51% had positive nodes (N+). In addition, on multivariate analysis nodal involvement was strongly associated with tumor size (OR: 9.9, p=0.001). Specifically, tumors larger than 1-cm had positive nodes in more than 70% of cases whereas those ≤1cm had a 5% risk of nodal involvement (1 of 21 patients) (Table). Similar rates of N+ were observed for both duodenal and ampullary carcinoids. With long-term follow-up, only one recurrence was observed (1%). The overall survival was similar among all treatment groups (median=139 months). Conclusions: Lymph node involvement is common in patients with duodenal and ampullary carcinoid tumor, particularly among tumors &gt;1 cm in size. When possible, surgical resection with lymphadenectomy is recommended for such tumors. [Table: see text]

Extra Gastrointestinal Stromal Tumor treated with imatinib in a patient with Neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1), also known as Von- Recklinghausen’s disease is one of the most commonly transmitted hereditary autosomal dominant diseases, with an estimated birth incidence of 1:3,000. The pathogenesis is thought to be due to a mutation in the NF1 tumor suppressor gene that is found on chromosome 17. This mutation leads to the loss of tumor suppressor function, which then results in the development of benign and malignant tumors. In addition to cutaneous, soft tissue, and visceral (plexiform) neurofibromas, this syndrome is connected with several types of gastrointestinal (GI) and abdominal tumors. Examples of such include the following: neuronal hyperplasia (neuromas), ampullary carcinoid, pheochromocytoma, and gastrointestinal stromal tumor (GIST). The latter has been suggested to be the most common NF1-associated GI tumor. Here we present the case of a NF1 patient who was found to have extra gastrointestinal stromal tumor (EGIST) which is seen in <5% cases of GIST.

Ampullary carcinoid tumor: An atypical location

Ampullary carcinoid tumor: An atypical location

Ampullary carcinoid tumors diagnosed by endoscopic ultrasound-guided fine needle aspiration in two patients with biliary and pancreatic duct obstruction

Ampullary carcinoid tumors diagnosed by endoscopic ultrasound-guided fine needle aspiration in two patients with biliary and pancreatic duct obstruction

Coincidence of GIST and pancreatic endocrine neoplasm in neurofibromatosis

Carcinoids of the ampulla of Vater are infrequent tumors of which a quarter of cases have been detected in patients with type I neurofibromatosis. This hereditary disease is also associated with gastrointestinal stromal tumors (GIST). However, the coincidence of these three entities together have only been formerly detected in five cases. A 53 year-old female patient, diagnosed with type I neurofibromatosis, with a malignant carcinoid of ampulla of Vater and multiple gastrointestinal stromal tumors in the duodenum and jejunum, was treated with total pancreatectomy and the excision of her intestinal tumors. Five-years on, a follow-up showed the patient to be well, and free from tumor recurrence. The coexistence of an ampullary carcinoid tumor, GIST and neurofibramatosis is very rare. Radical curative surgical resection is a good treatment option, but the optimal management of this is not yet well established.

The Rare Malignancy of the Hepatobiliary System: Ampullary Carcinoid Tumor

Introduction. Carcinoid tumors are low-grade tumors originating from endoderm and mostly involving the gastrointestinal system. However; they may be seen in any site within the gastrointestinal system. Case Presentation. A 69-year-old female patient. The results of blood tests were observed to be consistent with obstructive jaundice. A mass appearance was not encountered on tomographic examination. Papilla that was tumor-like macroscopically was seen in the second part of the duodenum in diagnostic endoscopy. Pylorus—preserving pancreaticoduodenectomy surgical procedure was applied. On pathological examination of the mass, a tumoral mass was detected in ampulla vateri localization, 1.5 × 1 × 0.8 cm in size, which, in immunohistochemical staining, was evaluated as a neuroendocrine tumor. Also, Metastasis was observed. Conclusion. The rarest type of carcinoid tumor is ampullary located carcinoid tumor, and tumor size is not a reliable indicator for tumor aggressivity in ampullary carcinoid tumors.

Ampullary Carcinoid Tumors: Unsuspected Diagnosis in Two Patients with Biliary and Pancreatic Ductal Obstruction Diagnosed by EUS-FNA

Purpose: Background: Ampullary carcinoid tumors are rare and often pose a diagnostic challenge due to the limitations of conventional endomucosal biopsies in confirming the diagnosis. Preoperative endoscopic ultrasound with fine needle aspiration (EUS-FNA) has not been studied extensively for this disease entity. Case Series: 1-An asymptomatic and previously healthy 46-year-old male was referred for evaluation of microcytic anemia. Physical exam was unremarkable. Colonoscopy was performed and was normal. Upper endoscopy showed an enlarged Ampulla of Vater. Mucosal biopsies showed non-specific inflammatory changes. EUS revealed a round hypoechoic 23 mm ampullary subepithelial mass. Both common bile duct and pancreatic duct were dilated up to 4 and 7 mm respectively. This was staged as T2N1Mx on EUS. FNA of the mass revealed a low grade neuroendocrine tumor. The patient was referred for pancreaticoduodenectomy (PD), and final pathology revealed carcinoid tumor staged as T2N1M0. Immunostaining for cytokeratin and synaptophysin was positive. Staining for chromogranin was negative. The postoperative course was unremarkable, and imaging and clinical follow up after six months were unremarkable for tumor recurrence. 2-A 53-year-old previously healthy female presented with four-week history of painless jaundice, pruritis, and 20-pound weight loss. Physical exam revealed scleral icterus, with mild hepatomegaly. Abdominal ultrasound and CT scan showed dilated intra and extrahepatic biliary ducts without a pancreatic mass. EUS showed a small 18 mm subepithelial lesion in the ampulla originating form the submusocsa and extending to the muscularis propria causing both pancreatic and biliary duct dilation staged as T3N1Mx. FNA of the lesion showed high grade neuroendocrine tumor with positive immunostaining for cytokeratin, synaptophysin, and chromogranin. The patient underwent pylorus-sparing PD. Final pathology confirmed a high grade neuroendocrine carcinoma, staged as T3N1M0. Discussion: Ampullary carcinoid tumors are extremely rare, and only 100 cases have been reported in the literature. Few reported cases emphasized the role of EUS-FNA for the diagnosis and staging of this disease in patients presenting with biliary and pancreatic duct obstruction, where preoperative diagnosis is challenging. Compared to other enteropancreatic carcinoids, ampullary carcinoids often display malignant behavior which is not predicted by primary tumor size. Potentially curative therapy involves complete resection with adequate regional lymphadenectomy. EUS with FNA provides accurate diagnostic and staging information for directing appropriate therapy.

Management of ampullary carcinoid tumors with pancreaticoduodenectomy.

A 66 year old female was found to have an ampullary mass on endoscopic examination. A pancreaticoduodenectomy was performed and the pathology was consistent with carcinoid tumor. Carcinoid tumors of the ampulla of Vater are rare tumors of the gastrointestinal tract, accounting for 0.3-1% of gastrointestinal carcinoids. Management of ampullary carcinoid tumor with pancreaticoduodenectomy is in accordance with current recommendations.

Ampullary Carcinoid Tumor: Diagnostic Challenges and Update on Management

Purpose: A 65-year-old woman was referred with non-specific abdominal pain, slightly elevated amylase, MRCP findings of an ampullary mass (8×9 mm) with dilated intra-hepatic duct, common bile duct (CBD) and pancreatic duct (PD). An EUS revealed a bulging ampulla with submucosal hypoechoeic lesion, dilated CBD and PD, both tapering towards the ampulla. EUS-FNA was non diagnostic. Unable to cannulate during ERCP; pinch biopsies obtained from the ampulla (Figure 1) showed nests of cells positive for synaptophysin, suggestive of a carcinoid tumor. Patient underwent a Whipple procedure. Pathological evaluation revealed a 2×1.5 cm, well-differentiated neuroendocrine carcinoma (Figure 2) with tumor free margins. One of seven lymph nodes was positive for carcinoma. Two months after the surgery, patient was tolerating a regular diet with normal bowel function. Amongst diagnostic challenges, obtaining accurate tissue biopsies might be difficult due to submucosal proliferation of the tumor with normal epithelium. The sensitivity of an octreotide scintigraphy decreases with smaller size and decreased tumor somatostatin receptor expression. Diagnostic material obtained by EUS-FNA could be non-diagnostic. Unlike other midgut carcinoids, size of the ampullary carcinoid is a poor predictor of metastatic potential with varying rates of metastasis at different tumor sizes. To achieve a cure, a Whipple procedure with patient specific modifications is needed irrespective of the size of the ampullary carcinoid. Poor surgical candidates can be offered endoscopic or surgical ampullectomy. A recent study showed 100% sensitivity and specificity for predicting metastases among small intestinal carcinoids using gene expression profiling (GEP). Tumor risk stratification using GEP could potentially rescue some patients from undergoing a radical surgery.Figure: Two views of the bulging, enlarged ampulla of Vater.Figure: Ampullary carcinoid tumor showing nests and trabeculae of neuroendocrine cells with admixed dense fibrous stroma (H&E, 200X).

Minute ampullary carcinoid tumor with lymph node metastases: a case report and review of literature

BackgroundCarcinoid tumors are usually considered to have a low degree of malignancy and show slow progression. One of the factors indicating the malignancy of these tumors is their size, and small ampullary carcinoid tumors have been sometimes treated by endoscopic resection.Case presentationWe report a case of a 63-year-old woman with a minute ampullary carcinoid tumor that was 7 mm in diameter, but was associated with 2 peripancreatic lymph node metastases. Mild elevation of liver enzymes was found at her regular medical check-up. Computed tomography (CT) revealed a markedly dilated common bile duct (CBD) and two enlarged peripancreatic lymph nodes. Endoscopy showed that the ampulla was slightly enlarged by a submucosal tumor. The biopsy specimen revealed tumor cells that showed monotonous proliferation suggestive of a carcinoid tumor. She underwent a pylorus-preserving whipple resection with lymph node dissection. The resected lesion was a small submucosal tumor (7 mm in diameter) at the ampulla, with metastasis to 2 peripancreatic lymph nodes, and it was diagnosed as a malignant carcinoid tumor.ConclusionRecently there have been some reports of endoscopic ampullectomy for small carcinoid tumors. However, this case suggests that attention should be paid to the possibility of lymph node metastases as well as that of regional infiltration of the tumor even for minute ampullary carcinoid tumors to provide the best chance for cure.

Radical Surgical Resection for Carcinoid Tumors of the Ampulla

Ampullary carcinoid tumors are extremely rare. The present study describes the clinicopathological features and outcomes for 10 ampullary carcinoid patients who underwent radical resection from 1998 to 2005. During this study period, 294 patients underwent pancreatoduodenectomy for ampullary neoplasms in our institution. The mean patient age was 58.0±13.4 years, and seven were male. Initial clinical manifestations were jaundice in four patients, nonspecific gastrointestinal symptoms in five, and completely asymptomatic in one. Standard pancreatoduodenectomy was performed in three patients, and pylorus-preserving pancreatoduodenectomy in seven, and there were no major complications. The mean tumor size and volume were 2.1±1.3 cm and 4.1±6.9 ml, respectively. Synaptophysin staining was positive in ten patients and chromogranin staining positive in eight. R0 resection was achieved in all ten patients. Overall and disease-free survival rates were 90 and 80% at 1 year, and 64 and 56% at 3 years, respectively. The liver was the most common site of initial metastasis after curative resection. Univariate analyses revealed that a maximal tumor diameter ≥2 cm and tumor extension beyond the ampulla were risk factors for tumor recurrence. In conclusion, while the majority of ampullary carcinoids are indolent, this tumor is associated with a relatively poor prognosis. We believe that radical resection, with the aim of complete tumor removal and cure, is the treatment of choice.

A Carcinoid Tumor of the Ampulla of Vater Treated by Endoscopic Snare Papillectomy

Here, a case of a patient with incidental finding of a carcinoid tumor of the ampulla of Vater, who was treated with endoscopic snare papillectomy, is reported. A 62-year-old male was admitted to our hospital due to a carcinoid tumor of the ampulla of Vater, which was found during follow-up endoscopy after an endoscopic mucosal resection of early gastric cancer. No lymphadenopathy or visceral metastasis was found on an abdominal CT scan, In-111 octerotide scan and EUS. The ampulla was then en bloc removed by endoscopic snare papillectomy. The resected specimen revealed a 0.7×0.5×0.1 cm sized carcinoid tumor. All margins of resection were negative for tumor. After six months of follow-up, there was no evidence of recurrence and metastasis, either endoscopically or radiologically. To our knowledge, this case is the first report of an ampullary carcinoid tumor treated by endoscopic snare papillectomy in Korea.