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[PMID]: 28015049
[Au] Autor:Anthony LB; Stafford S; Cronin M; Grossman A; Woltering E
[Ad] Address:LSUHSC New Orleans, New Orleans, LA; Knoxville Cancer Center, Knoxville, TN.
[Ti] Title:Octreotide LAR doses used in clinical practice: Results from an internet survey and a clinical practice.
[So] Source:J Clin Oncol;22(14_suppl):4274, 2004 Jul 15.
[Is] ISSN:1527-7755
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:4274 Background: Octreotide acetate LAR (LAR) is approved for the control of symptoms from carcinoid syndrome and VIPoma. Recommended LAR doses from the package label are 20 and 30 mg administered monthly. Physicians commonly confront clinical scenarios requiring titration of LAR to doses that control symptoms. We hypothesized that physicians commonly employed doses extending beyond those tested during the registrational period. To test this hypothesis, a physician practice and a patient support group were surveyed. METHODS: An internet survey conducted with a yahoo carcinoid patient support group (N=93) (with no individual subject identifiers) and a survey of a physician office practice database using the ICD-9 code of 259.2 (carcinoid syndrome) were conducted (N=309). RESULTS: Of the 93 patients responding to the internet survey, 28 (30%) were on LAR doses > 30 mg / mo as follows: 13 pts, 40 mg; 1 pt, 50 mg: 13 pts, 60 mg; 1 pt, 80 mg. From 50 of the 309 chart reviews from the clinical practice, 36 patients were receiving LAR at doses ranging from 20 mg to 60 mg every 2 to 4 weeks. The most common dose was 30 mg every 4 wks (N=24) followed by 20 mg q 4 wks (N=6). Other doses ranged from 40 mg every 3 wks to 60 mg every 4 wks with 1 patient each at the following doses: 30 mg q 2 wks; 30 mg q 3 wks; 40 mg q 3 wks; 40 mg q 4 wks; 50 mg q 4 wks and 60 mg q Conclusions: Octreotide acetate LAR is prescribed at doses beyond the package label 17% (single physician practice) to 30% (patient survey) of the time. [Table: see text].
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1612
[Cu] Class update date: 161227
[Lr] Last revision date:161227
[St] Status:In-Data-Review

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[PMID]: 27977628
[Au] Autor:Niu L; Li J; Zeng J; Fang G; Zhou L; Xu K; Zhang K
[Ad] Address:From the *Fuda Cancer Hospital, Jinan University School of Medicine, Guangzhou and †Department of General Surgery, The Second Hospital of Jilin University, Changchun, Jilin, China.
[Ti] Title:Percutaneous Irreversible Electroporation for Pancreatic VIPoma: A Case Report.
[So] Source:Pancreas;46(1):135-137, 2017 Jan.
[Is] ISSN:1536-4828
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:The purpose of this study was to investigate the safety and efficacy of irreversible electroporation (IRE) for the management of unresectable pancreatic vasoactive intestinal peptide tumor (VIPoma) in a 34-year-old male patient. The initial symptom was watery diarrhea, which could not be stopped by fasting. Laboratory tests revealed high vasoactive intestinal peptide (VIP) hormone levels, hypokalemia, and metabolic acidosis. Computed tomography examination showed a 6.0 × 5.0-cm, contrast-enhanced lesion in the neck and body of the pancreas and obliteration of the portal vein. Pathological and immunohistochemical findings were indicative of pancreatic VIPoma. The patient was treated with octreotide and IRE, and had no obvious IRE-related complications, except for moderate pain at the puncture sites. The patient reported that the watery diarrhea had decreased gradually; moreover, the VIP hormone level was normalized 15 days after IRE. Computed tomography scans showed a large area of necrosis in the pancreatic lesion. The findings from this case indicated that IRE could be a feasible and safe technique in controlling pancreatic VIPoma; however, additional follow-up and findings from more cases are required to further confirm the efficacy of IRE ablation therapy for pancreatic VIPoma.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1612
[Cu] Class update date: 161216
[Lr] Last revision date:161216
[St] Status:In-Data-Review

  3 / 547 MEDLINE  
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[PMID]: 27583474
[Au] Autor:Nilubol N; Freedman EM; Quezado MM; Patel D; Kebebew E
[Ad] Address:Endocrine Oncology Branch (N.N., E.M.F., D.P., E.K.) and Laboratory of Pathology (M.M.Q.), Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892.
[Ti] Title:Pancreatic Neuroendocrine Tumor Secreting Vasoactive Intestinal Peptide and Dopamine With Pulmonary Emboli: A Case Report.
[So] Source:J Clin Endocrinol Metab;101(10):3564-3567, 2016 Oct.
[Is] ISSN:1945-7197
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:CONTEXT: The vasoactive intestinal peptide-secreting neuroendocrine tumor (VIPoma) is a very rare pancreatic tumor. We report the first case of a patient with VIPoma that co-secreted dopamine and had pulmonary emboli. CASE DESCRIPTION: A 67-year-old woman presented with 2 months of watery diarrhea, severe generalized weakness,6.8 kg of weight loss, a facial rash, and hypokalemia. Colonoscopy did not reveal the cause of the chronic diarrhea. Initial biochemical testing showed markedly elevated serum vasoactive intestinal peptide (VIP) and pancreatic polypeptide. Computed tomography scan of the abdomen and pelvis revealed a 5.4-cm distal pancreatic mass. Octreoscan showed an intense uptake in the area of the pancreatic mass. Incidental pulmonary emboli were found and treated. Additional biochemical testing revealed a markedly elevated urinary dopamine level. The patient received preoperative α-blockade and octreotide. She underwent a successful laparoscopic distal pancreatectomy. Postoperative urinary dopamine and pancreatic polypeptide were within normal limits. Serum VIP decreased by half but remained elevated. Pathology confirmed a grade 1 pancreatic neuroendocrine tumor without lymph node metastasis. The patient's symptoms resolved and no longer required octreotide. Metastatic workup including computed tomography, F18-fluorodeoxglucose positron emission tomography, and Ga68-DOTATATE scans were negative during 4 years of follow-up. CONCLUSIONS: VIPoma is a rare subtype of pancreatic neuroendocrine tumor that can secrete dopamine and can be associated with thromboembolism.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1609
[Cu] Class update date: 161102
[Lr] Last revision date:161102
[St] Status:In-Data-Review

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[PMID]: 27583030
[Au] Autor:Dréanic J; Lepère C; El Hajjam M; Gouya H; Rougier P; Coriat R
[Ad] Address:Gastroenterology and Endoscopy Unit, Hôpital Cochin, 27, Rue du Faubourg, Saint Jacques F75014, Paris, France.
[Ti] Title:Emergency therapy for liver metastases from advanced VIPoma: surgery or transarterial chemoembolization?
[So] Source:Ther Adv Med Oncol;8(5):383-7, 2016 Sep.
[Is] ISSN:1758-8340
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:VIPoma is a rare neuroendocrine tumor (NET) with a high potential to develop hepatic metastases and poor prognosis. The primitive tumor is nonsymptomatic and usually localized within the pancreas. Liver metastasis drives the prognosis and induces profuse watery diarrhea or renal failure. We herein present severe renal failure or diarrhea in two patients hospitalized in intensive care justifying emergency treatment of liver metastasis. The two patients experienced severe diarrhea due to a hypersecretion of vasoactive intestinal peptide (VIP) from liver metastasis released into the blood circulation. Therapeutic management was discussed and liver transarterial chemoembolization (TACE) was performed with chemotherapy-loaded embospheres, which cause necrosis of tumor lesions. TACE controlled the hormonal syndrome and made patients eligible for curative surgery. Tumor necrosis occurred and VIP levels collapsed. Surgery was performed in one of the two cases after TACE and the patient was considered in remission. Both patients were still alive after 3 years of follow up. Thus, TACE is feasible and appears to be an effective emergency treatment in patients with a VIP-hormonal syndrome due to liver metastases. Despite the biological disorder due to the hormonal secretion, an aggressive approach is warranted in VIP liver metastasis.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1609
[Cu] Class update date: 160904
[Lr] Last revision date:160904
[Da] Date of entry for processing:160901
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.1177/1758834016656495

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[PMID]: 27461388
[Au] Autor:Dimitriadis GK; Weickert MO; Randeva HS; Kaltsas G; Grossman A
[Ad] Address:The Arden NET CoEWarwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism, University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK Division of Translational and Experimental MedicineWarwick Medical School, University of Warwick, Coventry, UK Division of Endocr
[Ti] Title:Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours.
[So] Source:Endocr Relat Cancer;23(9):R423-36, 2016 Sep.
[Is] ISSN:1479-6821
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Although recent epidemiological evidence indicates that the prevalence of non-functioning gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) is rising, a significant number of GEP-NETs still present with symptoms related to the secretion of biologically active substances leading to the development of distinct clinical syndromes. In the past, these syndromes were associated with substantial morbidity and mortality due to the lack of specific therapies; however, since the introduction of long-acting somatostatin analogues and medications such as proton pump inhibitors, their control has been greatly improved. As a result, nowadays, the main cause of morbidity and mortality in GEP-NETs is mostly directly related to tumour growth and the extent of metastatic disease. However, in some patients with functioning tumours and extensive disease, control of the secretory syndrome still remains problematic, necessitating the employment of several cytoreductive techniques, which may not always be sufficient. Recently, new agents directed against tumour growth, or exerting increased binding activity to receptors expressed in these tumours, or interfering with the synthetic pathway of some of the compounds secreted by these tumours, have been developed. Since there are no specific guidelines addressing the totality of the management of the secretory syndromes related to GEP-NETs, this review aims at critically analysing the medical management of previously recognised secretory syndromes; it also addresses areas of uncertainty, assesses the newer therapeutic developments and also addresses recently described but poorly characterised secretory syndromes related to GEP-NETs.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1608
[Cu] Class update date: 160826
[Lr] Last revision date:160826
[Js] Journal subset:IM
[St] Status:In-Data-Review
[do] DOI:10.1530/ERC-16-0200

  6 / 547 MEDLINE  
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[PMID]: 27404266
[Au] Autor:Parbhu SK; Adler DG
[Ad] Address:a Department of Internal Medicine, Division of Gastroenterology and Hepatology , University of Utah School of Medicine, Huntsman Cancer Center , Salt Lake City , Utah , USA.
[Ti] Title:Pancreatic neuroendocrine tumors: contemporary diagnosis and management.
[So] Source:Hosp Pract (1995);44(3):109-19, 2016 Aug.
[Is] ISSN:2154-8331
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Pancreatic neuroendocrine tumors (PNETs) are neoplasms that arise from the hormone producing cells of the islets of Langerhans, also known as pancreatic islet cells. PNETs are considered a subgroup of neuroendocrine tumors, and have unique biology, natural history and clinical management. These tumors are classified as 'functional' or 'non-functional' depending on whether they release peptide hormones that produce specific hormone- related symptoms, usually in established patterns based on tumor subtype. This manuscript will review pancreatic neuroendocrine tumor subtypes, syndromes, diagnosis, and clinical management.
[Mh] MeSH terms primary: Neuroendocrine Tumors/diagnosis
Neuroendocrine Tumors/therapy
Pancreatic Neoplasms/diagnosis
Pancreatic Neoplasms/therapy
[Mh] MeSH terms secundary: Carcinoma, Islet Cell/physiopathology
Carcinoma, Islet Cell/therapy
Continental Population Groups
Humans
Insulinoma/physiopathology
Insulinoma/therapy
Islets of Langerhans
Multiple Endocrine Neoplasia Type 1/physiopathology
Multiple Endocrine Neoplasia Type 1/therapy
Neoplasm Grading
Neoplasm Staging
Neuroendocrine Tumors/physiopathology
Pancreatic Hormones
Pancreatic Neoplasms/physiopathology
Peptide Hormones
Sex Factors
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Nm] Name of substance:0 (Pancreatic Hormones); 0 (Peptide Hormones)
[Em] Entry month:1702
[Cu] Class update date: 170206
[Lr] Last revision date:170206
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:160803
[St] Status:MEDLINE
[do] DOI:10.1080/21548331.2016.1210474

  7 / 547 MEDLINE  
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[PMID]: 27170574
[Au] Autor:Guo LJ; Wang CH; Tang CW
[Ad] Address:Department of Gastroenterology, West China Hospital of SiChuan University, ChengDu, SiChuan Province, China.
[Ti] Title:Epidemiological features of gastroenteropancreatic neuroendocrine tumors in Chengdu city with a population of 14 million based on data from a single institution.
[So] Source:Asia Pac J Clin Oncol;12(3):284-8, 2016 Sep.
[Is] ISSN:1743-7563
[Cp] Country of publication:Australia
[La] Language:eng
[Ab] Abstract:AIM: Recent studies on gastroenteropancreatic neuroendocrine tumors (GEP-NETs) in the United States as well as the European studies demonstrate an increasing GEP-NETs incidence. Most information on the epidemiology of neuroendocrine tumors comes from western countries. However, the epidemiological profile of GEP-NETs in West China is still unclear. The aim of study was to reflect the regional features of GEP-NETs in Chengdu city of West China based on data from a single institution. METHODS: West China Hospital (WCH), the largest university hospital located in Chengdu (West China) with population of 14.04 million, has established a serial of databases in recent years. According to the data from Medical Records Section of WCH and Chengdu Health Bureau, the total patients per year in WCH covered about 25.6-28% patients of Chengdu city during the 5 years. Therefore, we have used GEP-NETs diagnosed in WCH from 2009 to 2013 to reflect the regional epidemiological profile of GEP-NETs. RESULTS: GEP-NETs proportion in WCH increased 1.6-folds during past 5 years from 1.28/10(5) to 2.03/10(5) , P < 0.05. The average duration of symptom before diagnosis was 16.8 months. About 46.6% (115/248) of GEP-NETs were metastatic. Seventy-seven percent (190/248) of patients were over 40 years. Proportions of GEP-NETs from primary sites were rectum 30.6% (76/248), pancreas 23.4% (58/248), gastric 13.3% (33/248) and esophagus 11.3% (28/248). Proportions of insulinoma, vipoma and nonfunctional pancreatic neuroendocrine tumors (P-NETs) were 43.1% (25/58), 1.7% (1/58) and 55.2% (32/58) separately in the P-NETs. CONCLUSIONS: There is a distinct epidemiologic profile between West China and western countries based on a single institution data. The delayed diagnosis reflects inadequate disease awareness of GEP-NETs and paucity of research funding.
[Mh] MeSH terms primary: Intestinal Neoplasms/epidemiology
Neuroendocrine Tumors/epidemiology
Pancreatic Neoplasms/epidemiology
Stomach Neoplasms/epidemiology
[Mh] MeSH terms secundary: Adult
Aged
China/epidemiology
Female
Humans
Male
Middle Aged
United States
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1701
[Cu] Class update date: 170119
[Lr] Last revision date:170119
[Js] Journal subset:IM
[Da] Date of entry for processing:160822
[St] Status:MEDLINE
[do] DOI:10.1111/ajco.12498

  8 / 547 MEDLINE  
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[PMID]: 27071757
[Au] Autor:Cavalli T; Giudici F; Santi R; Nesi G; Brandi ML; Tonelli F
[Ad] Address:Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50139, Florence, Italy. tiziana.cavalli@gmail.com.
[Ti] Title:Ventricular fibrillation resulting from electrolyte imbalance reveals vipoma in MEN1 syndrome.
[So] Source:Fam Cancer;15(4):645-9, 2016 Oct.
[Is] ISSN:1573-7292
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:Sporadic VIPoma is an exceedingly rare tumor with an annual incidence of 1:10 million people worldwide, yet it is described in approximately 5 % of MEN1 patients. The majority of VIPomas are malignant and radical surgery is the best therapeutic option. A 58-year-old man presented with cardiocirculatory arrest due to ventricular fibrillation. The patient had a 3-month history of epigastric pain with diarrhea. After reanimation, laboratory data revealed severe hypokalemia and hypercalcemia. Further investigations showed hyperparathyroidism, left adrenal adenoma and pituitary microprolactinoma and genetic diagnosis of MEN1 syndrome was made. Abdominal computed tomography revealed a 45 × 30 mm mass of the pancreatic head and two hepatic lesions, which proved to be neuroendocrine after 68 Ga PET and needle biopsy. Vasoactive intestinal peptide (VIP) serum level had increased. Subsequently the patient underwent pylorus-preserving pancreaticoduodenectomy and hepatic resection. Intraoperative VIP returned to normal values. Histopathology confirmed a pancreatic VIPoma metastatic to the liver. The postoperative course was unremarkable and the patient is well with no evidence of disease at a 48 months follow-up. Even in case of anusual presentation, when two or more main clinical findings of MEN1 related tumors are present, unrespectively to the presence of MEN1 mutation, MEN1 syndrome should be suspected. Surgery in MEN1 pancreatic neuroendocrine tumors is indicated both to treat symptoms and to avoid oncological progression even in advanced cases.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1609
[Cu] Class update date: 160905
[Lr] Last revision date:160905
[Js] Journal subset:IM
[St] Status:In-Data-Review
[do] DOI:10.1007/s10689-016-9906-4

  9 / 547 MEDLINE  
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[PMID]: 27013370
[Au] Autor:Anderson CW; Bennett JJ
[Ad] Address:Department of Surgery, Helen F. Graham Cancer Center, 4701 Ogletown-Stanton Road, S-4000, Newark, DE 19713, USA. Electronic address: Carinne.Wright@gmail.com.
[Ti] Title:Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors.
[So] Source:Surg Oncol Clin N Am;25(2):363-74, 2016 Apr.
[Is] ISSN:1558-5042
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Pancreatic neuroendocrine tumors are a rare group of neoplasms that arise from multipotent stem cells in the pancreatic ductal epithelium. Although they comprise only 1% to 2% of pancreatic neoplasms, their incidence is increasing. Most pancreatic neuroendocrine tumors are nonfunctioning, but they can secrete various hormones resulting in unique clinical syndromes. Clinicians must be aware of the diverse manifestations of this disease, as the key step to management of these rare tumors is to first suspect the diagnosis.
[Mh] MeSH terms primary: Neuroendocrine Tumors/diagnosis
Pancreatic Neoplasms/diagnosis
[Mh] MeSH terms secundary: Humans
Incidence
Neoplasm Staging
Neuroendocrine Tumors/classification
Neuroendocrine Tumors/epidemiology
Pancreatic Neoplasms/classification
Pancreatic Neoplasms/epidemiology
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1612
[Cu] Class update date: 161230
[Lr] Last revision date:161230
[Js] Journal subset:IM
[Da] Date of entry for processing:160325
[St] Status:MEDLINE

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[PMID]: 26997993
[Au] Autor:Zhang X; Zhou L; Liu Y; Li W; Gao H; Wang Y; Yao B; Jiang D; Hu P
[Ad] Address:Department of Endocrinology, Peking University International Hospital, Beijing 102206, P.R. China.
[Ti] Title:Surgical resection of vasoactive intestinal peptideoma with hepatic metastasis aids symptom palliation: A case report.
[So] Source:Exp Ther Med;11(3):783-787, 2016 Mar.
[Is] ISSN:1792-0981
[Cp] Country of publication:Greece
[La] Language:eng
[Ab] Abstract:Vasoactive intestinal peptideoma (VIPoma) is a rare pancreatic endocrine tumor associated with a well-defined clinical syndrome characterized by watery diarrhea, hypokalemia and metabolic acidosis. In adults, VIPoma is most commonly found in the pancreas, with 80% of the tumors occurring in the body and tail and 20% occurring in the pancreatic head. VIPomas can represent a significant diagnostic challenge due to their nonspecific clinical presentation, which can result in the misdiagnosis of a VIPoma as another condition, such as laxative overdose or a carcinoid secreting tumor. Surgical clearance of the tumor is the first-line treatment, even in cases with metastasis. The present study describes the case of a patient who presented with chronic watery diarrhea and hypokalemia due to a tumor in the pancreatic head, which was confirmed to contain immunoreactive vasoactive intestinal polypeptide via immunohistochemistry. A hepatic metastasis lesion was diagnosed following computed tomography. Stable control of symptoms was achieved after surgery and drug treatment. The study additionally reviews the clinical, histological, radiological and diagnostic features of the condition, as well as the therapeutic modalities that can be used to treat VIPoma in the pancreatic head with hepatic metastasis.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1603
[Cu] Class update date: 160323
[Lr] Last revision date:160323
[St] Status:Publisher


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