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[PMID]: 29394839
[Au] Autor:Ueda Y; Toyama H; Terai S; Mukubou H; Shirakawa S; Yamashita H; Nanno Y; Mizumoto T; Lee D; Kinoshita H; Tanaka M; Ueno K; Ajiki T; Kido M; Fukumoto T
[Ad] Address:Division of Hepato-Biliary-Pancreatic Surgery, Dept. of Surgery, Kobe University Graduate School of Medicine.
[Ti] Title:[A Surgical Resected Case of VIPoma with Para-Aortic Lymph Node Involvement].
[So] Source:Gan To Kagaku Ryoho;44(12):1976-1978, 2017 Nov.
[Is] ISSN:0385-0684
[Cp] Country of publication:Japan
[La] Language:jpn
[Ab] Abstract:A 72-year-old woman had severe watery diarrhea and weight loss. Computed tomography demonstrated a 55mm tumor in pancreatic tail with enlargement of para-aortic lymph nodes. There was no apparent liver metastasis. Endoscopic ultrasound demonstrated a well-circumscribed heterogenous tumor, which was diagnosed neuroendocrine tumor by endoscopic ultrasound-guided fine needle aspiration biopsy. For suspected VIPoma with para-aortic lymph node involvement, distal pancreatectomy and para-aortic lymphadenectomy was performed. The tumor was diagnosed as VIPoma by immunohistochemistry. The diarrhea improved after surgery. No evidence of recurrence was detected after a follow-up of 11 months.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180203
[Lr] Last revision date:180203
[St] Status:In-Data-Review

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[PMID]: 29384277
[Au] Autor:André R; Koessler T; Polet D; Roth A; Ritz M; Kherad O
[Ad] Address:Service de médecine interne, Hôpital de la Tour, avenue J.-D. Maillard 3, 1217 Genève.
[Ti] Title:VIPome : étiologie rare d'une diarrhée hypokaliémiante. [VIPoma : a rare etiology of diarrhea with hypokalemia].
[So] Source:Rev Med Suisse;14(592):289-293, 2018 Jan 31.
[Is] ISSN:1660-9379
[Cp] Country of publication:Switzerland
[La] Language:fre
[Ab] Abstract:VIPoma or Verner Morrison syndrome is a very rare disease with an incidence rate of 1 case per 10 000 000 person-years. It is a neuroendocrine tumor issue from ß-pancreatic islets leading to profuse diarrhea, hypokalemia and gastric achlorydria due to secretion of vasoactive intestinal polypeptide (VIP) hormone. Diagnosis is based on histology of tumor and the dosage of VIP in a blood sample. Somatostatin analog is a simple and efficient treatment for diarrhea. Curative treatment with surgery could be proposed for a localized disease. For disseminated disease, there are different treatments and a multimodal assessment that should be discussed in a multidisciplinary team might be curative.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180131
[Lr] Last revision date:180131
[St] Status:In-Data-Review

  3 / 548 MEDLINE  
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[PMID]: 28730220
[Au] Autor:Belei OA; Heredea ER; Boeriu E; Marcovici TM; Cerbu S; Marginean O; Iacob ER; Iacob D; Motoc AGM; Boia ES
[Ad] Address:Department of Pediatric Surgery, "Victor Babes" University of Medicine and Pharmacy, Timisoara, Romania; radueiacob@umft.ro, radueiacob@yahoo.com; Department of Neonatology, "Victor Babes" University of Medicine and Pharmacy, Timisoara, Romania; danielariacob@yahoo.com.
[Ti] Title:Verner-Morrison syndrome. Literature review.
[So] Source:Rom J Morphol Embryol;58(2):371-376, 2017.
[Is] ISSN:1220-0522
[Cp] Country of publication:Romania
[La] Language:eng
[Ab] Abstract:Chronic diarrhea in infants is a common condition for addressability to pediatric gastroenterologists. The causes are multiple and the delay in reaching the final diagnosis can lead to complications in the general condition of the child. The purpose of this review is to present the bio-clinical and histogenetic particularities of a rare clinical entity, characterized by tumoral causes of chronic diarrhea. VIPomas are neuroendocrine tumors that autonomously secrete vasoactive intestinal peptide (VIP). Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by VIP-producing tumors only rarely occurs in adult patients with non-pancreatic disease. In pediatric patients, it is extremely rare for a VIPoma to originate in the pancreas; instead, WDHA syndrome is usually associated with VIP-secreting neurogenic tumors involving the retroperitoneum or mediastinum. The majority of VIP secreting tumors in pediatric patients are represented by ganglioneuroblastomas or ganglioneuromas originating in the adrenal medulla or sympathetic neural crest. This syndrome of watery diarrhea associated with hypokalemia and achlorhydria was first described by Verner and Morrison, in 1958, and has been assumed to be due to hypersecretion of VIP. In children, as well as in adult patients, the most likely explanation for persistent secretory diarrhea may be an occult VIPoma. In conclusion, the physicians should be aware that there are some rare tumoral causes of chronic diarrhea, often under-diagnosed. If the diagnosis is not considered, extensive gastrointestinal investigations will be undertaken, delaying the diagnosis and avoidable morbidity will occur.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1707
[Cu] Class update date: 170721
[Lr] Last revision date:170721
[St] Status:In-Process

  4 / 548 MEDLINE  
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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-Process

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[PMID]: 27501174
[Au] Autor:Chey WY; Frankel WL; Roy S; Datta S; Sen CK; Dillhoff M; Muscarella P; Soergel KH; Tompkins RK; Chang TM; Bradley EL; Ellison EC
[Ad] Address:*Department of Surgery, The Ohio State University Wexner Medical Center, Columbus, OH †Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH ‡Department of Surgery, Center for Regenerative Medicine and Cell Based Therapies, Comprehensive Wound Center, Laser Capture Molecular Core, The Ohio State University Wexner Medical Center, Columbus, OH §Division of Gastroenterology, Medical College of Wisconsin, Milwaukee, WI ¶Department of Surgery, University of California at Los Angeles, School of Medicine, Los Angeles, CA ||Department of Surgery, Florida State University, Tallahassee, FL **William and Sheila Konar Center for Digestive and Liver Diseases, Division of Gastroenterology and Hepatology, University of Rochester Medical Center, Rochester, NY.
[Ti] Title:Primary Pancreatic Secretinoma: Further Evidence Supporting Secretin as a Diarrheogenic Hormone.
[So] Source:Ann Surg;266(2):346-352, 2017 Aug.
[Is] ISSN:1528-1140
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:OBJECTIVES: To document the existence of primary pancreatic secretinoma in patients with watery diarrhea syndrome (WDS) and achlorhydria and establish secretin as a diarrheogenic hormone. BACKGROUND: Vasoactive intestinal peptide (VIP) has been widely accepted as the main mediator of WDS. However, in 1968, Zollinger et al reported 2 female patients with pancreatic neuroendocrine tumors, WDS, and achlorhydria. During surgery on the first, a 24-year-old patient, they noticed distended duodenum filled with fluid and a dilated gallbladder containing dilute bile with high bicarbonate concentration. After excision of the tumor, WDS ceased and gastric acid secretion returned. The second, a 47-year-old, patient's metastatic tumor extract given intravenously in dogs, produced significantly increased pancreatic and biliary fluid rich in bicarbonate. They suggested a secretin-like hormone of islet cell origin explains WDS and achlorhydria. These observations, however, predated radioimmunoassay, immunohistochemical staining, and other molecular studies. METHODS: The first patient's tumor tissue was investigated for secretin and VIP. Using both immunohistochemistry and laser microdissection and pressure catapulting technique for RNA isolation and subsequent reverse transcription polymerase chain reaction, the expression levels of secretin, and VIP were measured. RESULTS: Immunoreactive secretin and its mRNA were predominantly found in the tumor tissue whereas VIP and its mRNA were scarce. CONCLUSIONS: The findings strongly support that the WDS and achlorhydria in this patient may have been caused by secretin as originally proposed in 1968 and that secretin may act as a diarrheogenic hormone.
[Mh] MeSH terms primary: Pancreatic Neoplasms/secretion
Secretin/secretion
Vipoma/secretion
[Mh] MeSH terms secundary: Adult
Bicarbonates/metabolism
Body Water/metabolism
Female
Humans
Immunohistochemistry
Intestinal Mucosa/metabolism
Intestine, Small/metabolism
Laser Capture Microdissection
Male
Middle Aged
RNA, Messenger/analysis
RNA, Messenger/secretion
Real-Time Polymerase Chain Reaction
Secretin/analysis
[Pt] Publication type:JOURNAL ARTICLE
[Nm] Name of substance:0 (Bicarbonates); 0 (RNA, Messenger); 1393-25-5 (Secretin)
[Em] Entry month:1708
[Cu] Class update date: 170823
[Lr] Last revision date:170823
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:160809
[St] Status:MEDLINE
[do] DOI:10.1097/SLA.0000000000001938

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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.
[Mh] MeSH terms primary: Dopamine/secretion
Pancreatic Neoplasms/secretion
Pulmonary Embolism/diagnostic imaging
Vasoactive Intestinal Peptide/secretion
Vipoma/secretion
[Mh] MeSH terms secundary: Aged
Female
Humans
Pancreatectomy
Pancreatic Neoplasms/diagnostic imaging
Pancreatic Neoplasms/surgery
Vipoma/diagnostic imaging
Vipoma/surgery
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:37221-79-7 (Vasoactive Intestinal Peptide); VTD58H1Z2X (Dopamine)
[Em] Entry month:1706
[Cu] Class update date: 171001
[Lr] Last revision date:171001
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:160902
[St] Status:MEDLINE

  7 / 548 MEDLINE  
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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: 170220
[Lr] Last revision date:170220
[Da] Date of entry for processing:160902
[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:1607
[Cu] Class update date: 160826
[Lr] Last revision date:160826
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.1530/ERC-16-0200

  9 / 548 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:160713
[St] Status:MEDLINE
[do] DOI:10.1080/21548331.2016.1210474

  10 / 548 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:160513
[St] Status:MEDLINE
[do] DOI:10.1111/ajco.12498


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