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Neuroendocrine tumor of the anal canal

MedGen UID:
474482
Concept ID:
C3272849
Neoplastic Process
Synonyms: Anal Canal NET; anal canal NET; Anal Canal Neuroendocrine Tumor; anal canal neuroendocrine tumor; anal canal neuroendocrine tumour; Anal Canal Well Differentiated Tumor; anal canal well differentiated tumor; Anal Canal Well Differentiated Tumor/Carcinoma; anal canal well differentiated tumor/carcinoma; anal canal well differentiated tumour; Anal NET; anal NET; Anal Neuroendocrine Tumor; anal Neuroendocrine tumor; anal Neuroendocrine tumour; NET of anal canal; NET of the anal canal; neuroendocrine neoplasm of the anal canal; neuroendocrine tumor of anal canal; Neuroendocrine tumor of anal canal; neuroendocrine tumor of the anal canal; neuroendocrine tumour of anal canal
 
Monarch Initiative: MONDO:0015069
Orphanet: ORPHA100082

Definition

A are epithelial tumor of the anal canal arising from enterochromaffin cells in the colorectal-type epithelium above the dentate line and in the anal transition zone. The tumors are slow growing and the majority of cases are diagnosed in later advanced stages. It may present with symptoms related to the anatomical location of the tumor (rectal mass, rectal bleeding and pain, tenesmus or changes in bowel habits), symptoms of carcinoid syndrome (flushing and increased gut motility) or nonspecific symptoms of advanced disease (hepatomegaly, fever, weight loss, anorexia, malaise). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNeuroendocrine tumor of the anal canal

Professional guidelines

PubMed

Ueberroth BE, Liu AJ, Starr JS, Hobday TJ, Ashman JB, Mishra N, Bekaii-Saab TS, Halfdanarson TR, Sonbol MB
Clin Colorectal Cancer 2021 Jun;20(2):e139-e149. Epub 2020 Dec 17 doi: 10.1016/j.clcc.2020.12.001. PMID: 33551318
Naqvi J, Lee A, Lederman A, Kavi A, Osborn VW, Schreiber D
J Gastrointest Cancer 2020 Mar;51(1):211-216. doi: 10.1007/s12029-019-00238-5. PMID: 30982929
Klas JV, Rothenberger DA, Wong WD, Madoff RD
Cancer 1999 Apr 15;85(8):1686-93. doi: 10.1002/(sici)1097-0142(19990415)85:8<1686::aid-cncr7>3.0.co;2-7. PMID: 10223561

Recent clinical studies

Etiology

Okumura T, Noguchi T, Sekiguchi N, Kobayashi T, Kanda S, Ida K, Minagawa T, Tokumaru S, Umemura T, Koizumi T
Chemotherapy 2023;68(2):87-94. Epub 2022 Nov 3 doi: 10.1159/000527377. PMID: 36327945
Ueberroth BE, Liu AJ, Starr JS, Hobday TJ, Ashman JB, Mishra N, Bekaii-Saab TS, Halfdanarson TR, Sonbol MB
Clin Colorectal Cancer 2021 Jun;20(2):e139-e149. Epub 2020 Dec 17 doi: 10.1016/j.clcc.2020.12.001. PMID: 33551318
Naqvi J, Lee A, Lederman A, Kavi A, Osborn VW, Schreiber D
J Gastrointest Cancer 2020 Mar;51(1):211-216. doi: 10.1007/s12029-019-00238-5. PMID: 30982929
Majbar A, Hrora A, Jahid A, Ahallat M, Raiss M
BMC Res Notes 2016 Jun 13;9:304. doi: 10.1186/s13104-016-2108-1. PMID: 27297080Free PMC Article
Klas JV, Rothenberger DA, Wong WD, Madoff RD
Cancer 1999 Apr 15;85(8):1686-93. doi: 10.1002/(sici)1097-0142(19990415)85:8<1686::aid-cncr7>3.0.co;2-7. PMID: 10223561

Diagnosis

Correa Bonito A, Maqueda González R, Rodríguez Sánchez A, Bermejo Marcos E, Blanco Terés L, García Septiem J, Martín Pérez E
Gastroenterol Hepatol 2022 Apr;45 Suppl 1:62-63. Epub 2021 May 21 doi: 10.1016/j.gastrohep.2021.03.009. PMID: 34023472
Pagani W, Sheikh U, Skef W
Clin Gastroenterol Hepatol 2021 May;19(5):A25. Epub 2020 Apr 6 doi: 10.1016/j.cgh.2020.03.061. PMID: 32272246
Sunjaya D, Grieb B, Sweetser S
Gastroenterology 2019 Feb;156(3):560-561. Epub 2018 Sep 18 doi: 10.1053/j.gastro.2018.04.040. PMID: 30240674
Hoff PM, Coudry R, Moniz CM
Surg Oncol Clin N Am 2017 Jan;26(1):57-71. doi: 10.1016/j.soc.2016.07.013. PMID: 27889037
Roy AC, Wattchow D, Astill D, Singh S, Pendlebury S, Gormly K, Segelov E
Surg Oncol Clin N Am 2017 Jan;26(1):143-161. doi: 10.1016/j.soc.2016.07.009. PMID: 27889033

Therapy

Temperley HC, O'Sullivan NJ, Keyes A, Kavanagh DO, Larkin JO, Mehigan BJ, McCormick PH, Kelly ME
Langenbecks Arch Surg 2022 Dec;407(8):3193-3200. Epub 2022 Nov 4 doi: 10.1007/s00423-022-02715-1. PMID: 36331615
Ueberroth BE, Liu AJ, Starr JS, Hobday TJ, Ashman JB, Mishra N, Bekaii-Saab TS, Halfdanarson TR, Sonbol MB
Clin Colorectal Cancer 2021 Jun;20(2):e139-e149. Epub 2020 Dec 17 doi: 10.1016/j.clcc.2020.12.001. PMID: 33551318
Voong KR, Rashid A, Crane CH, Minsky BD, Krishnan S, Yao JC, Wolff RA, Skibber JM, Feig BW, Chang GJ, Das P
Am J Clin Oncol 2017 Dec;40(6):555-560. doi: 10.1097/COC.0000000000000211. PMID: 26237193
Sun P, Li YH, Wang W, Chen CQ, Wang LY
J BUON 2014 Jan-Mar;19(1):103-8. PMID: 24659650
Klas JV, Rothenberger DA, Wong WD, Madoff RD
Cancer 1999 Apr 15;85(8):1686-93. doi: 10.1002/(sici)1097-0142(19990415)85:8<1686::aid-cncr7>3.0.co;2-7. PMID: 10223561

Prognosis

Merichal Resina M, Cerdan Santacruz C, Sierra Grañón E, Tarragona Foradada JA, Olsina Kissler JJ
Cir Esp (Engl Ed) 2020 Oct;98(8):491-493. Epub 2019 Dec 31 doi: 10.1016/j.ciresp.2019.11.014. PMID: 31898949
Hoff PM, Coudry R, Moniz CM
Surg Oncol Clin N Am 2017 Jan;26(1):57-71. doi: 10.1016/j.soc.2016.07.013. PMID: 27889037
Roy AC, Wattchow D, Astill D, Singh S, Pendlebury S, Gormly K, Segelov E
Surg Oncol Clin N Am 2017 Jan;26(1):143-161. doi: 10.1016/j.soc.2016.07.009. PMID: 27889033
Garrett K, Kalady MF
Surg Clin North Am 2010 Feb;90(1):147-61, Table of Contents. doi: 10.1016/j.suc.2009.09.008. PMID: 20109639
Quan SH
Int Adv Surg Oncol 1983;6:323-35. PMID: 6307888

Clinical prediction guides

Fløe LE, Aggerholm-Pedersen N, Tabaksblat EM
J Cancer Res Clin Oncol 2024 Mar 6;150(3):114. doi: 10.1007/s00432-024-05635-3. PMID: 38448660Free PMC Article
Temperley HC, O'Sullivan NJ, Keyes A, Kavanagh DO, Larkin JO, Mehigan BJ, McCormick PH, Kelly ME
Langenbecks Arch Surg 2022 Dec;407(8):3193-3200. Epub 2022 Nov 4 doi: 10.1007/s00423-022-02715-1. PMID: 36331615
Mitra D, Rao PK, Nagarajan P, Bishop AJ, Farooqi AS, Gershenwald JE, Wargo J, Keung EZ, Fisher SB, Amaria RN, Davies MA, Ross MI, Guadagnolo BA
Pract Radiat Oncol 2022 Sep-Oct;12(5):437-445. Epub 2022 Mar 9 doi: 10.1016/j.prro.2022.02.012. PMID: 35278716
Hoff PM, Coudry R, Moniz CM
Surg Oncol Clin N Am 2017 Jan;26(1):57-71. doi: 10.1016/j.soc.2016.07.013. PMID: 27889037
Longacre TA, Kong CS, Welton ML
Adv Anat Pathol 2008 Sep;15(5):263-78. doi: 10.1097/PAP.0b013e318183234b. PMID: 18724100

Recent systematic reviews

Temperley HC, O'Sullivan NJ, Keyes A, Kavanagh DO, Larkin JO, Mehigan BJ, McCormick PH, Kelly ME
Langenbecks Arch Surg 2022 Dec;407(8):3193-3200. Epub 2022 Nov 4 doi: 10.1007/s00423-022-02715-1. PMID: 36331615
Martin-Perez B, Andrade-Ribeiro GD, Hunter L, Atallah S
Tech Coloproctol 2014 Sep;18(9):775-88. Epub 2014 May 7 doi: 10.1007/s10151-014-1148-6. PMID: 24848524

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