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      Periampullary Neuroendocrine Tumor as a Cause of Acute Pancreatitis

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          Abstract

          Patient: Male, 60

          Final Diagnosis: Periampullary neuroendocrine tumor

          Symptoms: Abdominal discomfort

          Medication: —

          Clinical Procedure: —

          Specialty: Surgery

          Objective:

          Rare disease

          Background:

          Duodenal and ampullary carcinoids are very rare tumors accounting respectively for 2% and 0.03% of all carcinoid tumors. Clinical findings vary according to the location of the tumor within the periampullary region; with epigastric pain being the most common presenting symptom in duodenal carcinoids and jaundice the most common clinical finding in ampullary carcinoids. Treatment options include pancreaticoduodenectomy, local excision, and endoscopic excision.

          Case Report:

          In this case report, we present a 60-year-old male who presented with a one-week history of intractable epi-gastric pain. He was diagnosed with duodenal periampullary carcinoid tumor and treated with local excision.

          Conclusions:

          Although duodenal and ampullary carcinoid tumors may have different clinical presentations, as well as histo-chemistry characteristics and metastatic potential, they appear to benefit from the same surgical treatment.

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          Most cited references13

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          Carcinoid tumors.

          Carcinoid tumors are rare, slow-growing neuroendocrine tumors arising from the enterochromaffin cells disseminated throughout the gastrointestinal and bronchopulmonary systems. Though they have been traditionally classified based on embryologic site of origin, morphologic pattern, and silver affinity, newer classification systems have been developed to emphasize the considerable clinical and histopathologic variability of carcinoid tumors found within each embryologic site of origin. These neoplasms pose a diagnostic challenge because they are often innocuous at the time of presentation, emphasizing the need for a multidisciplinary diagnostic approach using biochemical analysis, standard cross-sectional imaging, and newer advances in nuclear medicine. Similarly, treatment of both primary and disseminated carcinoid disease reflects the need for a multidisciplinary approach, with surgery remaining the only curative modality. The prognosis for patients with these tumors is generally favorable; however, it can be quite variable and is related to the location of the primary tumor, extent of metastatic disease at initial presentation, and time of diagnosis.
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            Small-bowel tumors: epidemiologic and clinical characteristics of 1260 cases from the connecticut tumor registry.

            To examine the epidemiology and clinical characteristics of small-bowel cancer. Patients with small-bowel tumors reported between 1980 and 2000, studied retrospectively. Data from the Connecticut Tumor Registry. One thousand sixty small-bowel cancer cases: 628 men (49.84%) and 632 women (50.16%). Mean age at presentation was 65.2 years. The most common location of small-bowel tumors was the ileum (374 cases; 29.7%), followed by the duodenum (320 cases; 25.4%) and the jejunum (193 cases; 15.3%). In 367 patient cases (29.1%: 192 men [30.6%] and 175 women [27.7%]), a prior or subsequent tumor of the gastrointestinal tract was reported. The most prevalent histologic type was carcinoid (417 cases; 33%), followed by adenocarcinoma (341 cases; 27%) and lymphoma (205 cases; 16.3%). The patient population was predominantly white (1159 patients; 92%), followed by African American patients (91 patients; 7.2%). Stratification by consecutive 7-year intervals showed the following: from 1980 to 1986, there were 10.5 cases per 100 000 individuals; from 1987 to 1993, there were 13.05 cases per 100 000 individuals; and from 1994 to 2000, there were 14.86 cases per 100 000 individuals. Men comprised 44.8% of cases from 1980 to 1986, 50.2% of cases from 1987 to 1993, and 53.3% of cases from 1994 to 2000. African American patients accounted for 7.5% of all cases from 1980 to 1986, 5.8% from 1986 to 1993, and 8.2% of cases from 1994 to 2000. In 1106 patients (87.7%), the primary therapy was surgical, including intestinal bypass, radical excision, excisional biopsy, and subtotal or total excision. The incidence of small-bowel tumors in Connecticut has increased during the past 2 decades, with the highest rate of increase in men. Carcinoid tumors are the most common small intestinal cancers identified histologically, followed by adenocarcinomas. The former seems to be more frequently seen in the ileum, the latter in the duodenum. Surgery is the treatment of choice for the cure or palliation of small-bowel cancers.
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              Four Neuroendocrine Tumor Types and Neuroendocrine Carcinoma of the Duodenum: Analysis of 203 Cases

              Background: Several types of neuroendocrine neoplasms (NENs) have been described in the duodenal tract, from low-grade tumors (NETs) to high-grade neuroendocrine carcinomas (NECs). A comprehensive analysis of histology, hormonal profile and prognostic parameters of a sufficiently large duodenal NEN series to cover all main kinds of neoplasms is however lacking. Methods: We collected a retrospective series of 203 duodenal wall and ampullary region NENs, from six specialized endocrine pathology centers. All were characterized histopathologically and histochemically, and 190 were followed for a median of 9 years. Results: Twenty-seven poorly differentiated NECs, mostly from the ampullary region, were identified and shown to lead to patient demise in a median of 10 months. Among 176 NETs, four subtypes were characterized, including 20 gastrinomas, 37 ampullary-type somatostatin-producing NETs (ASTs), 12 gangliocytic paragangliomas (GPs) and 106 nonfunctioning NETs (nfNETs). ASTs and GPs were mostly localized in the ampullary/periampullary region, while gastrinomas and nfNETs were mainly from the proximal duodenum. ASTs and gastrinomas showed high rates of local infiltration (especially lymphoinvasion and deep duodenal wall/pancreatic tissue invasion) and lymph node metastasis, while nfNETs had significantly lower and more size-dependent local invasive potential. Disease-specific survival differed significantly between NETs and NECs, though not among NET subtypes. NET cases with distant metastases (n = 23) were significantly associated with larger size, higher proliferative grade, lymphovascular invasion, deep invasion and local lymph node metastasis. Conclusion: Our careful analysis of a large series of duodenal NENs identified five histologically and prognostically different histotypes of potential clinical relevance.
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                Author and article information

                Journal
                Am J Case Rep
                Am J Case Rep
                amjcaserep
                The American Journal of Case Reports
                International Scientific Literature, Inc.
                1941-5923
                2018
                05 September 2018
                : 19
                : 1063-1067
                Affiliations
                [1 ]Department of Surgery, Lebanese American University Medical Center, Beirut, Lebanon
                [2 ]Department of Surgery, Sacré Coeur Hospital, Beirut, Lebanon
                [3 ]Department of Surgery, Lebanese University, Beirut, Lebanon
                [4 ]Department of Emergency Medicine, Lebanese American University, Beirut, Lebanon
                [5 ]Department of Hematology and Oncology, American University of Beirut Medical Center, Beirut, Lebanon
                [6 ]Mount Lebanon Hospital, Beirut, Lebanon
                [7 ]Department of Surgery, Mount Lebanon Hospital, Beirut, Lebanon
                Author notes

                Authors’ Contribution:

                [A]

                Study Design

                [B]

                Data Collection

                [C]

                Statistical Analysis

                [D]

                Data Interpretation

                [E]

                Manuscript Preparation

                [F]

                Literature Search

                [G]

                Funds Collection

                Conflict of interest: None declared

                Corresponding Author: Gregory Nicolas, e-mail: gregory.nicolas@ 123456lau.edu
                Article
                908205
                10.12659/AJCR.908205
                6135042
                30181528
                02f5bb25-fffe-45b9-bdf3-c7007a29ce41
                © Am J Case Rep, 2018

                This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International ( CC BY-NC-ND 4.0)

                History
                : 24 November 2017
                : 18 April 2018
                Categories
                Articles

                carcinoid tumor,general surgery,pancreatic neoplasms

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