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Mucinous adenocarcinoma of the appendix

MedGen UID:
317382
Concept ID:
C1706832
Neoplastic Process
Synonyms: Appendiceal mucinous adenocarcinoma; appendiceal mucinous adenocarcinoma; Appendix Mucinous Adenocarcinoma; appendix mucinous adenocarcinoma; mucinous adenocarcinoma of the appendix; vermiform appendix mucinous adenocarcinoma
 
Monarch Initiative: MONDO:0018330
Orphanet: ORPHA391723

Definition

Mucinous adenocarcinoma of the appendix is a very rare, slow growing, well-differentiated epithelial neoplasm of the appendix characterized by abundant mucin production. Clinically, it presents as acute appendicitis (with abdominal pain, fever, leukocytosis) or as pseudomyxoma peritonei (wide-spread presence of mucin within the peritoneal cavity), however some patients may be completely asymptomatic at the time of diagnosis. In many cases, a second gastrointestinal malignancy is present. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMucinous adenocarcinoma of the appendix

Professional guidelines

PubMed

McClelland PH, Gregory SN, Nah SK, Hernandez JM, Davis JL, Blakely AM
Ann Surg Oncol 2024 Sep;31(9):6237-6251. Epub 2024 Jun 14 doi: 10.1245/s10434-024-15526-z. PMID: 38874874Free PMC Article
McClelland PH, Blakely AM
Ann Surg Oncol 2024 Sep;31(9):6278-6279. Epub 2024 Jun 11 doi: 10.1245/s10434-024-15617-x. PMID: 38862838

Recent clinical studies

Etiology

McClelland PH, Gregory SN, Nah SK, Hernandez JM, Davis JL, Blakely AM
Ann Surg Oncol 2024 Sep;31(9):6237-6251. Epub 2024 Jun 14 doi: 10.1245/s10434-024-15526-z. PMID: 38874874Free PMC Article
Lopez-Ramirez F, Gushchin V, Sittig M, King MC, Baron E, Nikiforchin A, Nieroda C, Sardi A
Ann Surg Oncol 2022 Jun;29(6):3390-3401. Epub 2022 Feb 8 doi: 10.1245/s10434-021-11233-1. PMID: 35133518
Turner KM, Patel SH
Ann Surg Oncol 2022 Apr;29(4):2144-2145. Epub 2022 Jan 10 doi: 10.1245/s10434-021-11229-x. PMID: 35006507
Straker RJ 3rd, Grinberg SZ, Karakousis GC
Ann Surg Oncol 2022 Apr;29(4):2344-2345. Epub 2022 Jan 6 doi: 10.1245/s10434-021-11218-0. PMID: 34993662
Gulmez S, Polat E, Duman U, Senger AS, Uzun O, Ozduman O, Oz A, Subasi IE, Duman M
Langenbecks Arch Surg 2022 May;407(3):1201-1207. Epub 2021 Nov 29 doi: 10.1007/s00423-021-02386-4. PMID: 34845541

Diagnosis

Oyeh E, Nsaful J, Bediako-Bowan A, Gbadamosi H, Mensah YB, Adu-Aryee NA, Nyarko V
Ghana Med J 2024 Mar;58(1):109-114. doi: 10.4314/gmj.v58i1.15. PMID: 38957279Free PMC Article
McClelland PH, Gregory SN, Nah SK, Hernandez JM, Davis JL, Blakely AM
Ann Surg Oncol 2024 Sep;31(9):6237-6251. Epub 2024 Jun 14 doi: 10.1245/s10434-024-15526-z. PMID: 38874874Free PMC Article
Gulmez S, Polat E, Duman U, Senger AS, Uzun O, Ozduman O, Oz A, Subasi IE, Duman M
Langenbecks Arch Surg 2022 May;407(3):1201-1207. Epub 2021 Nov 29 doi: 10.1007/s00423-021-02386-4. PMID: 34845541
Turner G, Sharma P, Gillespie A, Eglinton T
ANZ J Surg 2013 Oct;83(10):790-1. doi: 10.1111/ans.12302. PMID: 24099128
Levy AD, Shaw JC, Sobin LH
Radiographics 2009 Mar-Apr;29(2):347-73. doi: 10.1148/rg.292085189. PMID: 19325052

Therapy

Lopez-Ramirez F, Gushchin V, Sittig M, King MC, Baron E, Nikiforchin A, Nieroda C, Sardi A
Ann Surg Oncol 2022 Jun;29(6):3390-3401. Epub 2022 Feb 8 doi: 10.1245/s10434-021-11233-1. PMID: 35133518
Gulmez S, Polat E, Duman U, Senger AS, Uzun O, Ozduman O, Oz A, Subasi IE, Duman M
Langenbecks Arch Surg 2022 May;407(3):1201-1207. Epub 2021 Nov 29 doi: 10.1007/s00423-021-02386-4. PMID: 34845541
Copur M, Cushman-Vokoun A, Padussis J, Whitney W, Schroeder C, Herold D, Lintel N, Horn A
Oncology (Williston Park) 2021 Jun 23;35(6):335-340. doi: 10.46883/ONC.2021.3506.0335. PMID: 34161053
Sabesan A, Felder S, Feuerlein S, Lam C, McGettigan M, Powers BD, Dessureault S, Dineen SP
Ann Surg Oncol 2020 Jan;27(1):165-170. Epub 2019 Aug 6 doi: 10.1245/s10434-019-07676-2. PMID: 31388776
Nishitani K, Nishitani H, Shimoda Y
J Dermatol 1987 Apr;14(2):167-9. doi: 10.1111/j.1346-8138.1987.tb03552.x. PMID: 3038981

Prognosis

Oyeh E, Nsaful J, Bediako-Bowan A, Gbadamosi H, Mensah YB, Adu-Aryee NA, Nyarko V
Ghana Med J 2024 Mar;58(1):109-114. doi: 10.4314/gmj.v58i1.15. PMID: 38957279Free PMC Article
McClelland PH, Gregory SN, Nah SK, Hernandez JM, Davis JL, Blakely AM
Ann Surg Oncol 2024 Sep;31(9):6237-6251. Epub 2024 Jun 14 doi: 10.1245/s10434-024-15526-z. PMID: 38874874Free PMC Article
McClelland PH, Blakely AM
Ann Surg Oncol 2024 Sep;31(9):6278-6279. Epub 2024 Jun 11 doi: 10.1245/s10434-024-15617-x. PMID: 38862838
Tsagkalidis V, Ecker BL
Ann Surg Oncol 2024 Jul;31(7):4240-4241. Epub 2024 Apr 15 doi: 10.1245/s10434-024-15289-7. PMID: 38622457Free PMC Article
Lopez-Ramirez F, Gushchin V, Sittig M, King MC, Baron E, Nikiforchin A, Nieroda C, Sardi A
Ann Surg Oncol 2022 Jun;29(6):3390-3401. Epub 2022 Feb 8 doi: 10.1245/s10434-021-11233-1. PMID: 35133518

Clinical prediction guides

McClelland PH, Gregory SN, Nah SK, Hernandez JM, Davis JL, Blakely AM
Ann Surg Oncol 2024 Sep;31(9):6237-6251. Epub 2024 Jun 14 doi: 10.1245/s10434-024-15526-z. PMID: 38874874Free PMC Article
Lopez-Ramirez F, Gushchin V, Sittig M, King MC, Baron E, Nikiforchin A, Nieroda C, Sardi A
Ann Surg Oncol 2022 Jun;29(6):3390-3401. Epub 2022 Feb 8 doi: 10.1245/s10434-021-11233-1. PMID: 35133518
Gulmez S, Polat E, Duman U, Senger AS, Uzun O, Ozduman O, Oz A, Subasi IE, Duman M
Langenbecks Arch Surg 2022 May;407(3):1201-1207. Epub 2021 Nov 29 doi: 10.1007/s00423-021-02386-4. PMID: 34845541
Fackche N, Schmocker RK, Kubi B, Cloyd JM, Ahmed A, Grotz T, Leiting J, Fournier K, Lee AJ, Powers B, Dineen S, Veerapong J, Baumgartner JM, Clarke C, Gamblin TC, Patel SH, Dhar V, Hendrix RJ, Lambert L, Abbott DE, Pokrzywa C, Lafaro K, Lee B, Zaidi MY, Maithel SK, Johnston FM, Greer JB
J Gastrointest Surg 2021 Nov;25(11):2908-2919. Epub 2021 Feb 25 doi: 10.1007/s11605-021-04953-y. PMID: 33634422
Milovanov V, Sardi A, Aydin N, Nieroda C, Sittig M, Gushchin V
Ann Surg Oncol 2017 Jul;24(7):1783-1786. Epub 2017 Mar 17 doi: 10.1245/s10434-017-5794-6. PMID: 28315061

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