[A Case of Primary Somatostatin-Producing Tumor of the Duodenum with Liver Metastases with Long-Term Survival of More than 20 Years].

A 52-year-old woman underwent esophagogastroduodenoscopy after an abnormal medical examination, which revealed a mass lesion over half the circumference of the superior duodenal angulus. Immunostaining was diffusely positive for somatostatin, synaptophysin, and chromogranin A. A 3 cm-sized mass in the pancreaticoduodenal region and multiple nodular lesions of a few mm in both lobes of the liver were revealed by CT. The diagnosis is primary somatostatin-producing tumor of the duodenum with multiple liver metastases. She underwent gastric jejunal bypass for impaired transit. Afterwards hepatic infusion and systemic chemotherapy were continued, and 5 years passed without progression. When she stopped chemotherapy for 6 months, she started somatostatin analogue therapy because of the increase of the tumors. The tumors did not increase, and 20 years have passed since the start of treatment. We report a case of primary somatostatin-producing tumor of the duodenum with liver metastases that is still alive for a long period of time, with a review of the literature.

Somatostatin-Immunoreactive Pancreaticoduodenal Neuroendocrine Neoplasms: Twenty-Three Cases Evaluated according to the WHO 2010 Classification.

BACKGROUND/OBJECTIVE: Neuroendocrine neoplasms of the pancreas and duodenum with predominant or exclusive immunoreactivity for somatostatin (pdSOMs) are rare, and knowledge about tumour biology, treatment, survival and prognostic factors is limited. This study aims to describe clinical, pathological and biochemical features as well as treatment and prognosis of pdSOMs. DESIGN: Twenty-three patients with pdSOM (9 duodenal, 12 pancreatic and 2 unknown primary tumours) were identified from our prospective neuroendocrine tumour database, and data according to the study aims were recorded. RESULTS: Among the 9 patients with duodenal SOM, the male/female ratio was 4/5. All males and 1 female had neurofibromatosis type 1. Seven patients had stage 1A/B and 2 had stage 2B disease. The Ki-67 index was 1-5% (median 2%). Plasma somatostatin was elevated in the patients with 2B disease. Of the 14 patients with pancreatic SOM or an unknown primary tumour, the male/female ratio was 2/12. One male had multiple endocrine neoplasia type 1. Five had stage 1A/2B and 9 had stage 4. The Ki-67 index was 1-40% (median 7%). Plasma somatostatin was elevated in 7 patients. Patients reported symptoms related to the somatostatinoma syndrome, but none fulfilled the criteria for a full syndrome. Primary tumour in the pancreas, metastatic disease at diagnosis and higher tumour grade were all associated with significantly poorer survival. CONCLUSION: None of the patients with pdSOM presented with the full somatostatinoma syndrome. Prognostic factors are localisation of the primary tumour, dissemination and tumour grade. A Ki-67 index of 5% may discriminate the course of the disease.

Pancreatic somatostatinoma diagnosed preoperatively: report of a case.

CONTEXT: Somatostatinoma is a rare neoplasm of the pancreas. Preoperative diagnosis is often difficult. CASE REPORT: We report a 72-year-old woman with a pancreatic head tumor measuring 37 mm in diameter, and enlargement of the lymph nodes on the anterior surface of the pancreatic head and the posterior surface of the horizontal part of the duodenum. Laboratory data showed an elevated plasma somatostatin concentration. Examination of a biopsy specimen of the pancreatic head mass obtained by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) showed histopathological features of a neuroendocrine tumor. Immunohistochemical staining showed that the tumor cells were positive for somatostatin, leading to a preoperative diagnosis of pancreatic somatostatinoma. The patient underwent pylorus-preserving pancreaticoduodenectomy. The plasma somatostatin concentration decreased progressively after surgery. CONCLUSIONS: A rare case of pancreatic somatostatinoma with lymph node metastases was presented. Immunohistochemical analysis of a biopsy specimen obtained by EUS-FNA was useful for preoperative diagnosis.

[New considerations in the surgical treatment of pancreatic neuroendocrine tumours]. / A hasnyálmirigy neuroendokrin tumorok sebészi kezelésének újabb szempontjai.

Except insulinomas, pancreatic neuroendocrine tumours are malignant in more than 50% of cases. Treatment of these tumours should be directed against the tumours and the excess of hormones produced by the tumours. Surgical treatment may reduce the mortality and morbidity of patients and, in case of localized lesions, may result in a definitive cure. The aim of this review is to summarize novel developments in the surgical treatment of pancreatic neuroendocrine tumours, including those related to metastatic diseases and MEN1 syndrome.

The current management of pancreatic neuroendocrine tumors.

PanNETs constitute a rare and heterogeneous group of pancreatic neoplasms whose overall prognosis is better than the more common pancreatic adenocarcinoma. Although surgery is the only treatment that provides a cure, many adjuvant therapies have been explored with some new, exciting, targeted therapies just approved for PanNETs. With growing interest in this type of neoplasm, an increasing number of clinical trials and natural history studies should shed light on the best management for these patients.

Somatostatinoma of the Ampulla of Vater: A Systematic Review.

BACKGROUND AND AIMS: Somatostatinoma of the ampulla of Vater (SAV) is a rare neuroendocrine tumor that usually appears with atypical clinical manifestations and is associated with Von Recklinghausen's disease. The aims of this study were to systematically review the literature regarding SAV and to highlight the clinicopathological characteristics and optimal therapeutic management of this rare entity. METHODS: A systematic search of the literature in PubMed/Medline and Scopus databases was performed by two independent investigators, including all case reports and case series concerning SAVs from 1980 until September 2021. RESULTS: In total, 37 articles were retrieved, including 43 patients, with a male to female ratio of 1.8:1 and a mean age of 46.8 ± 11.3 years (mean, SD). For 23 out of 43 patients (53.5%), Von Recklinghausen's disease was proved. The main clinical manifestations were abdominal pain (41.9%), jaundice (27.9%), weight loss (20.9%) and bowel disorders (20.9%). Typical histological findings included psammoma bodies, nests or clusters of epithelial cells with eosinophilic cytoplasm, while somatostatin staining was positive in 35 patients (81.4%), chromogranin-A in 21 patients (48.8%) and synaptophysin in 18 patients (41.9%). Surgery was the initial therapeutic approach in 34 patients (79.1%), whereas Whipple's procedure was the preferred surgical approach in 23 patients (53.4%). The longest survival among included patients was 13 years and only two postoperative deaths (4.7%) were reported. CONCLUSIONS: Somatostatinomas of the ampulla of Vater are rare malignancies that require increased physicians' suspicion and accurate surgical approach in order to achieve optimal therapeutic results.

Short-term results of 42 endoscopic ampullectomies: a single-center experience.

OBJECTIVE: Benign lesions of the major papilla are rare but raise the problem of their medical care. We studied the efficacy, safety, and histology of the endoscopic ampullectomy. PATIENTS AND METHODS: Forty-two endoscopic resections of the major papilla were undertaken in 23 males and 19 females of a mean age of 63. Five patients (12%) presented with a familial adenomatous polyposis. The assessment of resectability included preoperative histology, and endoscopic ultrasound (EUS) in 26 patients (62%) always showing intra-mucosal lesion. The resection was performed with a duodenoscope, using a diathermic loop with a pure current section. RESULTS: The resection was realized in one piece for 34 patients, in 2-4 fragments for 8 patients. A plastic pancreatic stent was inserted in 26 patients (62%), a plastic biliary stent in 10 patients (24%). There were no deaths but nine complications (21%): six acute pancreatitis (four patients with a pancreatic stent, contrary to the literature), three delayed gastrointestinal bleeding. The final histological result was fibrosis and inflammatory tissue in 7 patients, low-grade dysplasia in 20 patients, high-grade dysplasia or in situ carcinoma in 10 patients, invasive adenocarcinoma in 1 patient, and somatostatinoma in 2 patients (concordance of 72% with the initial histology). The resection was complete in 39 patients (93%). Three patients had additional surgery because of positive margin of resection or bad histology criteria. The median of follow-up in 33 patients with a complete resection was of 15 months, and we did not note any recurrence in 29 patients (88%). CONCLUSION: Endoscopic ampullectomy is an efficient treatment for superficial lesions of the papilla, despite a significant but rarely severe morbidity. Preoperative EUS is mandatory, preoperative histology is advisable. Long-term follow-up is necessary.

Mixed endocrine somatostatinoma of the ampulla of vater associated with a neurofibromatosis type 1: a case report and review of the literature.

CONTEXT: Mixed endocrine tumors are double neoplasms with both glandular and endocrine components; these tumors are rare, especially those arising in the ampulla of Vater. Ampullary somatostatinomas are classically associated with neurofibromatosis type 1. We herein describe the first reported case of a mixed endocrine somatostatinoma of the ampulla of Vater associated with neurofibromatosis type 1; we also present a review of the literature of the 7 mixed endocrine tumors of the ampulla which have been reported so far. CASE REPORT: A 49-year-old woman presented with atypical abdominal pain. Endoscopic examination revealed a tumor involving the ampulla of Vater and a CT scan identified stenoses of both the distal common bile duct and the main pancreatic duct. A pancreaticoduodenectomy was performed and pathological examination revealed two tumor components, exocrine (high grade adenoma with infiltrative adenocarcinoma) and endocrine (expressing somatostatin hormone) with lymph node metastases originating from both types. The patient was treated with adjuvant chemotherapy and has had no recurrence for 3 years. DISCUSSION: In ampullary somatostatinomas, psammoma bodies are pathognomonic and chromogranin A is rarely expressed: these features should alert the pathologist to an association with neurofibromatosis type 1. The management of patients with mixed tumors is challenging. The treatment of choice is surgery, and adjuvant chemotherapy should be adapted to the most aggressive component, i.e. the exocrine one. CONCLUSION: Because of their rarity, the diagnosis of ampullary mixed endocrine tumors is difficult. Our case points out the characteristic features of these neoplasms and their possible association with neurofibromatosis type 1.

Duodenal somatostatinoma presenting as obstructive jaundice with the coexistence of a gastrointestinal stromal tumour in neurofibromatosis type 1: a case with review of the literature.

Somatostatinomas are rare neuroendocrine tumours, mostly located in the pancreas or duodenum, with an estimated incidence of 1 in 40 million. Duodenal somatostatinomas (DSs) are usually found in association with neurofibromatosis type 1 (NF1), tuberous sclerosis and Von Hippel-Lindau syndrome. Gastrointestinal stromal tumours (GIST) have also been described in NF1, but the association with somatostatinoma is very uncommon. We report the case of a patient with NF1 who presented with obstructive jaundice due to multiple firm nodules around the ampulla of Vater. A pancreaticoduodenectomy was performed and revealed a 1 cm duodenal/ampullary mass which stained positive for somatostatin, together with a GIST also found on the duodenal wall. Despite its rarity, ampullary somatostatinomas should be considered in the differential diagnosis of biliary tract dilation in patients with NF1.

Large somatostatin-producing endocrine carcinoma of the ampulla of vater in association with GIST in a patient with von Recklinghausen's disease. Case report and review of the literature.

CONTEXT: Somatostatin-producing endocrine tumors of the duodenum are very rare neoplasms of the gastrointestinal tract. These tumors may be associated with von Recklinghausen's disease. CASE REPORT: We present the case of a 49-year-old female patient with von Recklinghausen's disease and an incidentally diagnosed ampullary neoplasm. The patient was treated with a classical pancreaticoduodenectomy. At surgery, a mass was found in the greater curve of the stomach which was resected using the classic Whipple procedure. Histology and immunohistochemistry showed that the duodenal tumor was an ampullary somatostatin-producing endocrine carcinoma while the gastric tumor was a gastrointestinal stromal tumor (GIST). The postoperative course was uneventful and the patient is alive, without tumor recurrence, six years after surgery. CONCLUSION: Somatostatin-producing endocrine tumors of the duodenum are rare tumors, often associated with von Recklinghausen's disease; these neoplasms should be treated aggressively using radical surgical resection. Although local resection may be appropriate for small duodenal somatostatin-producing tumors, a pancreaticoduodenectomy is usually required for larger tumors.

Diagnostic usefulness of FDG-PET for malignant somatostatinoma of the pancreas.

A 24-year-old female complaining of diarrhea and back pain was admitted to hospital where a tumor of the pancreatic head was revealed on a computed tomography (CT) scan. Abdominal ultrasonography, CT and celiac angiography revealed a hypervascular lesion on the pancreas. An endocrine tumor, particularly a somatostatinoma, was suspected and hormone levels in the blood were examined. Serum hormone levels were normal, so FDG-PET was performed. An abnormally high accumulation of FDG was detected on the FDG-PET image at the head of the pancreas, and the SUV of the lesion was 3.2, so the mass was considered to be malignant on FDG-PET. Pylorus-preserving pancreatoduodenectomy was performed with a preoperative diagnosis of malignant endocrine tumor. The resected specimen revealed a massive, yellowish-white tumor of the pancreas head measuring 50x 45x38mm. Histologically, the tumor was a malignant islet-cell tumor, and immunohistochemically the tumor stained with an anti-somatostatin antibody, but not with antibodies against glucagons, insulin or vasoactive intestinal polypeptide. The patient was discharged after the operation and has remained well without recurrence for 5 years. Liver metastatic tumors, however, appeared 6 years after the operation.

[Mucinous gastric adenocarcinoma and duodenal somato-statinoma. Case report]. / Adenocarcinoma gastrico di tipo mucinoso associato a somatostatinoma duodenale. Case report.

The Authors present a rare association of gastric adenocarcinoma and somatostatin-producing duodenal carcinoid. The pre-operative abdominal CT scan revealed the gastric lesions and a duodenal polypoid lesion, giving an important indication to perform a subtotal gastrectomy and a duodenal resection. The definitive diagnosis was possible with histological examination.

[Malignant somatostatinoma (brief overview and a case review)]. / Maligní somatostatinom (strucný prehled a kazuistika).

INTRODUCTION: Somatostatinoma is a rare, slowly growing tumor with malignity potential, most commonly located in the pancreas or duodenum. By the time its diagnosis is established, it is usually large and liver metastases are present. Increased concentrations of somatostatin, produced by pancreatic D cells or by intestinal mucosa, inihibit secretion of a number of GIT hormones, endocrine and exocrine pancreatic secretion. The clinical findings include symptoms of diabetes, dyspepsia, diarrhoea, steatorhoea, abdominal pain, hypochlorhydria, anemia. Sometimes, the finding is accidental. A CASE REVIEW: The authors present a case of somatostatinoma, detected accidentaly in a 72-year-old male. The tumor originated from the pancreatic head, partially spreading invasively into surrounding organs (duodenum), invading blood vessels and metastazing into peripancreatic lymphonodes. The tumor was asymptomatic and was detected as an accidental finding on ultrasound and CT abdominal examinations for the patient's urological disorder (vesicolithiasis). Pylorus saving hemipancreatoduodenectomy was performed and, on microscopic and subsequent immunohistochemical examination of the resecate, a well- differentiated endocrine, somatostatin- producing pancreatic carcinoma was verified.

[Long term outcome following 26 surgical ampullectomies]. / Résultat à long terme de 26 ampullectomies chirurgicales.

BACKGROUND: Pancreaticoduodenectomy (PD) is the standard surgical treatment for malignant ampullomas but is still associated with a mortality and morbidity still ranging from 0 to 10% and from 15 to 40%, respectively. Ampullectomy is an alternative to PD for benign ampulloma or, in high-risk patients, for invasive carcinoma. The aim of this study was to report early and long term results of surgical ampullectomy for presumed benign ampullomas. PATIENTS AND METHODS: From 1981 to 2004, 26 patients from two institutions underwent surgical ampullectomy. Of the 26 patients, 8 had familial adenomatous polyposis (FAP). Surgical ampullectomy was indicated on a multisciplinary basis. RESULTS: Final pathological examination revealed 15 adenomas, 4 in situ adenocarcinomas, 2 endocrine tumors, and 5 other benign lesions. There was no postoperative mortality. Specific morbidity was 8% (N=2). Mean follow-up was 86+/-70 months (range: 3-204). Actuarial overall 5-year survival was 92%. There were 4 local recurrences (none in patients with FAP). Four patients died during follow-up (including 3 from initial disease). CONCLUSION: Ampullectomy is a good alternative to PD in case of benign or non-invasive malignant ampullary lesion, including in selected cases of FAP.

[Type I neurofibromatosis, pheochromocytoma and somatostatinoma of the ampulla. Literature review]. / Association neurofibromatose de type I, phéochromocytome et somatostatinome de l'ampoule de Vater. Revue de la littérature.

Gastrointestinal involvement in Von Recklinghausen's neurofibromatosis (autosomal dominant disease) is observed in 25% of patients and is frequently represented by duodenal carcinoids. We report a case of somatostatinoma of the ampulla in a 42-year-old female with neurofibromatosis and pheochromocytoma and show a literature review about the association of neurofibromatosis and somatostatinoma. This pathological association appears non hasardous and surgical ampullectomy offers a good alternative to pancreaticoduodenectomy in localised tumors treatment.

The Coexistence of Somatostatinoma and Gastrointestinal Stromal Tumor in the Duodenum of a Patient with Von Recklinghausen's Disease.

We herein describe a case of somatostatinoma coexisting with a gastrointestinal stromal tumor (GIST) in the duodenum of an 81-year-old woman with Von Recklinghausen's disease (VRD) and common bile duct stone who presented with diarrhea of three months in duration. Gastroduodenoscopy revealed an ulcer on the second part of the duodenum. A 2.1-cm enhancing tumor was observed to extend from the ulcer on an abdominal computed tomography scan. Subtotal stomach-preserving pancreaticoduodenectomy revealed a somatostatinoma on the papilla of the vater and duodenal GIST. There have been only eight reports on VRD associated with ampullary somatostatinoma and GIST. An awareness of this possibility in patients with gastrointestinal lesions is necessary for proper treatment and patient management.