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Jejunal neuroendocrine tumor, well differentiated, low or intermediate grade

MedGen UID:
1619460
Concept ID:
C4525626
Neoplastic Process
Synonyms: Jejunal neuroendocrine neoplasm; jejunal neuroendocrine neoplasm; Jejunal Neuroendocrine Tumor; jejunal neuroendocrine tumor; Jejunal neuroendocrine tumor; jejunal neuroendocrine tumor, well differentiated, low or intermediate grade; jejunal neuroendocrine tumour
 
Monarch Initiative: MONDO:0015064
Orphanet: ORPHA100077

Definition

Jejunal neuroendocrine tumor is a rare, primary, malignant, epithelial neoplasm of the small intestine arising from enterochromaffin cells in the jejunum. Clinical behavior depends on the histologic grade, but initially it is generally characterized by vague abdominal symptoms (cramping, bloating, diarrhea) with insidious onset, although sometimes it could present with signs of bowel obstruction/perforation or gastrointestinal bleeding. Diagnosis in advanced stages with regional or distant spread is common, but signs of carcinoid syndrome (flushing, sweating, diarrhea) are usually not apparent until hepatic metastasis has occurred. [from ORDO]

Professional guidelines

PubMed

Jacobsen E
Am J Hematol 2022 Dec;97(12):1638-1651. Epub 2022 Oct 18 doi: 10.1002/ajh.26737. PMID: 36255040
Camacho PM, Petak SM, Binkley N, Diab DL, Eldeiry LS, Farooki A, Harris ST, Hurley DL, Kelly J, Lewiecki EM, Pessah-Pollack R, McClung M, Wimalawansa SJ, Watts NB
Endocr Pract 2020 May;26(Suppl 1):1-46. doi: 10.4158/GL-2020-0524SUPPL. PMID: 32427503
Garvey WT, Mechanick JI, Brett EM, Garber AJ, Hurley DL, Jastreboff AM, Nadolsky K, Pessah-Pollack R, Plodkowski R; Reviewers of the AACE/ACE Obesity Clinical Practice Guidelines
Endocr Pract 2016 Jul;22 Suppl 3:1-203. Epub 2016 May 24 doi: 10.4158/EP161365.GL. PMID: 27219496

Recent clinical studies

Etiology

Wang Y, Acs B, Robertson S, Liu B, Solorzano L, Wählby C, Hartman J, Rantalainen M
Ann Oncol 2022 Jan;33(1):89-98. Epub 2021 Sep 29 doi: 10.1016/j.annonc.2021.09.007. PMID: 34756513
Cusimano MC, Vicus D, Pulman K, Maganti M, Bernardini MQ, Bouchard-Fortier G, Laframboise S, May T, Hogen LF, Covens AL, Gien LT, Kupets R, Rouzbahman M, Clarke BA, Mirkovic J, Cesari M, Turashvili G, Zia A, Ene GEV, Ferguson SE
JAMA Surg 2021 Feb 1;156(2):157-164. doi: 10.1001/jamasurg.2020.5060. PMID: 33175109Free PMC Article
Ahdoot M, Wilbur AR, Reese SE, Lebastchi AH, Mehralivand S, Gomella PT, Bloom J, Gurram S, Siddiqui M, Pinsky P, Parnes H, Linehan WM, Merino M, Choyke PL, Shih JH, Turkbey B, Wood BJ, Pinto PA
N Engl J Med 2020 Mar 5;382(10):917-928. doi: 10.1056/NEJMoa1910038. PMID: 32130814Free PMC Article
Epstein JI, Egevad L, Amin MB, Delahunt B, Srigley JR, Humphrey PA; Grading Committee
Am J Surg Pathol 2016 Feb;40(2):244-52. doi: 10.1097/PAS.0000000000000530. PMID: 26492179
Weller M, Wick W, Aldape K, Brada M, Berger M, Pfister SM, Nishikawa R, Rosenthal M, Wen PY, Stupp R, Reifenberger G
Nat Rev Dis Primers 2015 Jul 16;1:15017. doi: 10.1038/nrdp.2015.17. PMID: 27188790

Diagnosis

Weller M, Wen PY, Chang SM, Dirven L, Lim M, Monje M, Reifenberger G
Nat Rev Dis Primers 2024 May 9;10(1):33. doi: 10.1038/s41572-024-00516-y. PMID: 38724526
Cusimano MC, Vicus D, Pulman K, Maganti M, Bernardini MQ, Bouchard-Fortier G, Laframboise S, May T, Hogen LF, Covens AL, Gien LT, Kupets R, Rouzbahman M, Clarke BA, Mirkovic J, Cesari M, Turashvili G, Zia A, Ene GEV, Ferguson SE
JAMA Surg 2021 Feb 1;156(2):157-164. doi: 10.1001/jamasurg.2020.5060. PMID: 33175109Free PMC Article
Ahdoot M, Wilbur AR, Reese SE, Lebastchi AH, Mehralivand S, Gomella PT, Bloom J, Gurram S, Siddiqui M, Pinsky P, Parnes H, Linehan WM, Merino M, Choyke PL, Shih JH, Turkbey B, Wood BJ, Pinto PA
N Engl J Med 2020 Mar 5;382(10):917-928. doi: 10.1056/NEJMoa1910038. PMID: 32130814Free PMC Article
Oronsky B, Ma PC, Morgensztern D, Carter CA
Neoplasia 2017 Dec;19(12):991-1002. Epub 2017 Nov 5 doi: 10.1016/j.neo.2017.09.002. PMID: 29091800Free PMC Article
Epstein JI, Egevad L, Amin MB, Delahunt B, Srigley JR, Humphrey PA; Grading Committee
Am J Surg Pathol 2016 Feb;40(2):244-52. doi: 10.1097/PAS.0000000000000530. PMID: 26492179

Therapy

Chu AWL, Wong MM, Rayner DG, Guyatt GH, Díaz Martinez JP, Ceccacci R, Zhao IX, McMullen E, Srivastava A, Wang J, Wen A, Wang FC, Brignardello-Petersen R, Izcovich A, Oykhman P, Wheeler KE, Wang J, Spergel JM, Singh JA, Silverberg JI, Ong PY, O'Brien M, Martin SA, Lio PA, Lind ML, LeBovidge J, Kim E, Huynh J, Greenhawt M, Gardner DD, Frazier WT, Ellison K, Chen L, Capozza K, De Benedetto A, Boguniewicz M, Smith Begolka W, Asiniwasis RN, Schneider LC, Chu DK
J Allergy Clin Immunol 2023 Dec;152(6):1470-1492. Epub 2023 Sep 9 doi: 10.1016/j.jaci.2023.08.029. PMID: 37678577
Freedman A, Jacobsen E
Am J Hematol 2020 Mar;95(3):316-327. Epub 2019 Dec 22 doi: 10.1002/ajh.25696. PMID: 31814159
Barbui T, Tefferi A, Vannucchi AM, Passamonti F, Silver RT, Hoffman R, Verstovsek S, Mesa R, Kiladjian JJ, Hehlmann R, Reiter A, Cervantes F, Harrison C, Mc Mullin MF, Hasselbalch HC, Koschmieder S, Marchetti M, Bacigalupo A, Finazzi G, Kroeger N, Griesshammer M, Birgegard G, Barosi G
Leukemia 2018 May;32(5):1057-1069. Epub 2018 Feb 27 doi: 10.1038/s41375-018-0077-1. PMID: 29515238Free PMC Article
Garvey WT, Mechanick JI, Brett EM, Garber AJ, Hurley DL, Jastreboff AM, Nadolsky K, Pessah-Pollack R, Plodkowski R; Reviewers of the AACE/ACE Obesity Clinical Practice Guidelines
Endocr Pract 2016 Jul;22 Suppl 3:1-203. Epub 2016 May 24 doi: 10.4158/EP161365.GL. PMID: 27219496
Gross A, Kay TM, Paquin JP, Blanchette S, Lalonde P, Christie T, Dupont G, Graham N, Burnie SJ, Gelley G, Goldsmith CH, Forget M, Hoving JL, Brønfort G, Santaguida PL; Cervical Overview Group
Cochrane Database Syst Rev 2015 Jan 28;1(1):CD004250. doi: 10.1002/14651858.CD004250.pub5. PMID: 25629215Free PMC Article

Prognosis

Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Rogers L, Zhang P, Vogelbaum MA, Perry A, Ashby LS, Modi JM, Alleman AM, Galvin J, Brachman D, Jenrette JM, De Groot J, Bovi JA, Werner-Wasik M, Knisely JPS, Mehta MP
J Neurosurg 2018 Jul;129(1):35-47. Epub 2017 Oct 6 doi: 10.3171/2016.11.JNS161170. PMID: 28984517Free PMC Article
Hemmingsen B, Gimenez-Perez G, Mauricio D, Roqué I Figuls M, Metzendorf MI, Richter B
Cochrane Database Syst Rev 2017 Dec 4;12(12):CD003054. doi: 10.1002/14651858.CD003054.pub4. PMID: 29205264Free PMC Article
Hurria A, Togawa K, Mohile SG, Owusu C, Klepin HD, Gross CP, Lichtman SM, Gajra A, Bhatia S, Katheria V, Klapper S, Hansen K, Ramani R, Lachs M, Wong FL, Tew WP
J Clin Oncol 2011 Sep 1;29(25):3457-65. Epub 2011 Aug 1 doi: 10.1200/JCO.2011.34.7625. PMID: 21810685Free PMC Article
International Non-Hodgkin's Lymphoma Prognostic Factors Project
N Engl J Med 1993 Sep 30;329(14):987-94. doi: 10.1056/NEJM199309303291402. PMID: 8141877

Clinical prediction guides

de la Fouchardiere A, Blokx W, van Kempen LC, Luzar B, Piperno-Neumann S, Puig S, Alos L, Calonje E, Massi D; ESP Dermatopathology Working Group; EORTC Melanoma Group; EURACAN
Virchows Arch 2021 Jul;479(1):3-11. Epub 2021 Jan 12 doi: 10.1007/s00428-020-03005-1. PMID: 33432480
Cusimano MC, Vicus D, Pulman K, Maganti M, Bernardini MQ, Bouchard-Fortier G, Laframboise S, May T, Hogen LF, Covens AL, Gien LT, Kupets R, Rouzbahman M, Clarke BA, Mirkovic J, Cesari M, Turashvili G, Zia A, Ene GEV, Ferguson SE
JAMA Surg 2021 Feb 1;156(2):157-164. doi: 10.1001/jamasurg.2020.5060. PMID: 33175109Free PMC Article
Epstein JI, Egevad L, Amin MB, Delahunt B, Srigley JR, Humphrey PA; Grading Committee
Am J Surg Pathol 2016 Feb;40(2):244-52. doi: 10.1097/PAS.0000000000000530. PMID: 26492179
Basturk O, Hong SM, Wood LD, Adsay NV, Albores-Saavedra J, Biankin AV, Brosens LA, Fukushima N, Goggins M, Hruban RH, Kato Y, Klimstra DS, Klöppel G, Krasinskas A, Longnecker DS, Matthaei H, Offerhaus GJ, Shimizu M, Takaori K, Terris B, Yachida S, Esposito I, Furukawa T; Baltimore Consensus Meeting
Am J Surg Pathol 2015 Dec;39(12):1730-41. doi: 10.1097/PAS.0000000000000533. PMID: 26559377Free PMC Article
Hurria A, Togawa K, Mohile SG, Owusu C, Klepin HD, Gross CP, Lichtman SM, Gajra A, Bhatia S, Katheria V, Klapper S, Hansen K, Ramani R, Lachs M, Wong FL, Tew WP
J Clin Oncol 2011 Sep 1;29(25):3457-65. Epub 2011 Aug 1 doi: 10.1200/JCO.2011.34.7625. PMID: 21810685Free PMC Article

Recent systematic reviews

Chu AWL, Wong MM, Rayner DG, Guyatt GH, Díaz Martinez JP, Ceccacci R, Zhao IX, McMullen E, Srivastava A, Wang J, Wen A, Wang FC, Brignardello-Petersen R, Izcovich A, Oykhman P, Wheeler KE, Wang J, Spergel JM, Singh JA, Silverberg JI, Ong PY, O'Brien M, Martin SA, Lio PA, Lind ML, LeBovidge J, Kim E, Huynh J, Greenhawt M, Gardner DD, Frazier WT, Ellison K, Chen L, Capozza K, De Benedetto A, Boguniewicz M, Smith Begolka W, Asiniwasis RN, Schneider LC, Chu DK
J Allergy Clin Immunol 2023 Dec;152(6):1470-1492. Epub 2023 Sep 9 doi: 10.1016/j.jaci.2023.08.029. PMID: 37678577
Shen Y, Shi Q, Nong K, Li S, Yue J, Huang J, Dong B, Beauchamp M, Hao Q
J Cachexia Sarcopenia Muscle 2023 Jun;14(3):1199-1211. Epub 2023 Apr 14 doi: 10.1002/jcsm.13225. PMID: 37057640Free PMC Article
Wong CK, Mak RY, Kwok TS, Tsang JS, Leung MY, Funabashi M, Macedo LG, Dennett L, Wong AY
J Pain 2022 Apr;23(4):509-534. Epub 2021 Aug 24 doi: 10.1016/j.jpain.2021.07.012. PMID: 34450274
Conte MS, Bradbury AW, Kolh P, White JV, Dick F, Fitridge R, Mills JL, Ricco JB, Suresh KR, Murad MH; GVG Writing Group
J Vasc Surg 2019 Jun;69(6S):3S-125S.e40. Epub 2019 May 28 doi: 10.1016/j.jvs.2019.02.016. PMID: 31159978Free PMC Article
Gross A, Kay TM, Paquin JP, Blanchette S, Lalonde P, Christie T, Dupont G, Graham N, Burnie SJ, Gelley G, Goldsmith CH, Forget M, Hoving JL, Brønfort G, Santaguida PL; Cervical Overview Group
Cochrane Database Syst Rev 2015 Jan 28;1(1):CD004250. doi: 10.1002/14651858.CD004250.pub5. PMID: 25629215Free PMC Article

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