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High Grade Appendix Mucinous Adenocarcinoma

MedGen UID:
474399
•Concept ID:
C3272766
•
Neoplastic Process

Definition

A mucinous adenocarcinoma that arises from the appendix. The malignant cells are numerous and exhibit high grade dysplasia. It may be associated with pseudomyxoma peritonei, but unlike the low grade mucinous neoplasm, it invades other organs and metastasizes to distant anatomic sites. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHigh Grade Appendix Mucinous Adenocarcinoma

Professional guidelines

PubMed

The histopathological classification, diagnosis and differential diagnosis of mucinous appendiceal neoplasms, appendiceal adenocarcinomas and pseudomyxoma peritonei.
Carr NJ, Bibeau F, Bradley RF, Dartigues P, Feakins RM, Geisinger KR, Gui X, Isaac S, Milione M, Misdraji J, Pai RK, Rodriguez-Justo M, Sobin LH, van Velthuysen MF, Yantiss RK
Histopathology 2017 Dec;71(6):847-858. Epub 2017 Sep 19 doi: 10.1111/his.13324. PMID: 28746986
Appendiceal Mucinous Neoplasms: Diagnosis and Management.
Shaib WL, Assi R, Shamseddine A, Alese OB, Staley C 3rd, Memis B, Adsay V, Bekaii-Saab T, El-Rayes BF
Oncologist 2017 Sep;22(9):1107-1116. Epub 2017 Jun 29 doi: 10.1634/theoncologist.2017-0081. PMID: 28663356Free PMC Article
Management of pseudomyxoma peritonei.
Fallis SA, Moran BJ
J BUON 2015 May;20 Suppl 1:S47-55. PMID: 26051332

Recent clinical studies

Etiology

High-Grade Appendiceal Mucinous Neoplasm: Clinicopathologic Findings in 35 Cases.
Gonzalez RS, Carr NJ, Liao H, Pai RK, Agostini-Vulaj D, Misdraji J
Arch Pathol Lab Med 2022 Dec 1;146(12):1471-1478. doi: 10.5858/arpa.2021-0430-OA. PMID: 35472721
Controversies in appendiceal pathology: mucinous and goblet cell neoplasms.
Orr CE, Yantiss RK
Pathology 2022 Mar;54(2):167-176. Epub 2021 Nov 23 doi: 10.1016/j.pathol.2021.09.003. PMID: 34836648
Mutation profile of high-grade appendiceal mucinous neoplasm.
Liao X, Vavinskaya V, Sun K, Hao Y, Li X, Valasek M, Xu R, Polydorides AD, Houldsworth J, Harpaz N
Histopathology 2020 Feb;76(3):461-469. Epub 2019 Dec 23 doi: 10.1111/his.13986. PMID: 31491041
The histopathological classification, diagnosis and differential diagnosis of mucinous appendiceal neoplasms, appendiceal adenocarcinomas and pseudomyxoma peritonei.
Carr NJ, Bibeau F, Bradley RF, Dartigues P, Feakins RM, Geisinger KR, Gui X, Isaac S, Milione M, Misdraji J, Pai RK, Rodriguez-Justo M, Sobin LH, van Velthuysen MF, Yantiss RK
Histopathology 2017 Dec;71(6):847-858. Epub 2017 Sep 19 doi: 10.1111/his.13324. PMID: 28746986
Appendiceal Mucinous Neoplasms: Diagnosis and Management.
Shaib WL, Assi R, Shamseddine A, Alese OB, Staley C 3rd, Memis B, Adsay V, Bekaii-Saab T, El-Rayes BF
Oncologist 2017 Sep;22(9):1107-1116. Epub 2017 Jun 29 doi: 10.1634/theoncologist.2017-0081. PMID: 28663356Free PMC Article

Diagnosis

Controversies in appendiceal pathology: mucinous and goblet cell neoplasms.
Orr CE, Yantiss RK
Pathology 2022 Mar;54(2):167-176. Epub 2021 Nov 23 doi: 10.1016/j.pathol.2021.09.003. PMID: 34836648
The Frontiers of Appendiceal Controversies: Mucinous Neoplasms and Pseudomyxoma Peritonei.
Hissong E, Yantiss RK
Am J Surg Pathol 2022 Jan 1;46(1):e27-e42. doi: 10.1097/PAS.0000000000001662. PMID: 33417324
Updates in Appendix Pathology: The Precarious Cutting Edge.
Carr NJ
Surg Pathol Clin 2020 Sep;13(3):469-484. Epub 2020 Jul 11 doi: 10.1016/j.path.2020.05.006. PMID: 32773195
The histopathological classification, diagnosis and differential diagnosis of mucinous appendiceal neoplasms, appendiceal adenocarcinomas and pseudomyxoma peritonei.
Carr NJ, Bibeau F, Bradley RF, Dartigues P, Feakins RM, Geisinger KR, Gui X, Isaac S, Milione M, Misdraji J, Pai RK, Rodriguez-Justo M, Sobin LH, van Velthuysen MF, Yantiss RK
Histopathology 2017 Dec;71(6):847-858. Epub 2017 Sep 19 doi: 10.1111/his.13324. PMID: 28746986
Appendiceal Mucinous Neoplasms: Diagnosis and Management.
Shaib WL, Assi R, Shamseddine A, Alese OB, Staley C 3rd, Memis B, Adsay V, Bekaii-Saab T, El-Rayes BF
Oncologist 2017 Sep;22(9):1107-1116. Epub 2017 Jun 29 doi: 10.1634/theoncologist.2017-0081. PMID: 28663356Free PMC Article

Therapy

Iterative Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Recurrent Mucinous Adenocarcinoma of the Appendix.
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
Mucinous Adenocarcinoma of the Appendix With Histologic Response to Neoadjuvant Chemotherapy: Review of Histologic and Clinical Spectrum of Epithelial Neoplastic Mucinous Lesions of the Appendix.
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
Efficacy of modified FOLFOX6 chemotherapy for patients with unresectable pseudomyxoma peritonei.
Hiraide S, Komine K, Sato Y, Ouchi K, Imai H, Saijo K, Takahashi M, Takahashi S, Shirota H, Takahashi M, Ishioka C
Int J Clin Oncol 2020 Apr;25(4):774-781. Epub 2019 Dec 10 doi: 10.1007/s10147-019-01592-x. PMID: 31823151Free PMC Article
Pseudomyxoma Peritonei of Extra-Appendiceal Origin: A Comparative Study.
Baratti D, Kusamura S, Milione M, Pietrantonio F, Caporale M, Guaglio M, Deraco M
Ann Surg Oncol 2016 Dec;23(13):4222-4230. Epub 2016 Jun 28 doi: 10.1245/s10434-016-5350-9. PMID: 27352203
Management of pseudomyxoma peritonei.
Fallis SA, Moran BJ
J BUON 2015 May;20 Suppl 1:S47-55. PMID: 26051332

Prognosis

High-Grade Appendiceal Mucinous Neoplasm: Clinicopathologic Findings in 35 Cases.
Gonzalez RS, Carr NJ, Liao H, Pai RK, Agostini-Vulaj D, Misdraji J
Arch Pathol Lab Med 2022 Dec 1;146(12):1471-1478. doi: 10.5858/arpa.2021-0430-OA. PMID: 35472721
Controversies in appendiceal pathology: mucinous and goblet cell neoplasms.
Orr CE, Yantiss RK
Pathology 2022 Mar;54(2):167-176. Epub 2021 Nov 23 doi: 10.1016/j.pathol.2021.09.003. PMID: 34836648
The Frontiers of Appendiceal Controversies: Mucinous Neoplasms and Pseudomyxoma Peritonei.
Hissong E, Yantiss RK
Am J Surg Pathol 2022 Jan 1;46(1):e27-e42. doi: 10.1097/PAS.0000000000001662. PMID: 33417324
Updates in Appendix Pathology: The Precarious Cutting Edge.
Carr NJ
Surg Pathol Clin 2020 Sep;13(3):469-484. Epub 2020 Jul 11 doi: 10.1016/j.path.2020.05.006. PMID: 32773195
Management of pseudomyxoma peritonei.
Fallis SA, Moran BJ
J BUON 2015 May;20 Suppl 1:S47-55. PMID: 26051332

Clinical prediction guides

Iterative Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Recurrent Mucinous Adenocarcinoma of the Appendix.
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
Controversies in appendiceal pathology: mucinous and goblet cell neoplasms.
Orr CE, Yantiss RK
Pathology 2022 Mar;54(2):167-176. Epub 2021 Nov 23 doi: 10.1016/j.pathol.2021.09.003. PMID: 34836648
The Frontiers of Appendiceal Controversies: Mucinous Neoplasms and Pseudomyxoma Peritonei.
Hissong E, Yantiss RK
Am J Surg Pathol 2022 Jan 1;46(1):e27-e42. doi: 10.1097/PAS.0000000000001662. PMID: 33417324
Altered linkage pattern of N-glycan sialic acids in pseudomyxoma peritonei.
Nummela P, Heiskanen A, Kytölä S, Haglund C, Lepistö A, Satomaa T, Ristimäki A
Glycobiology 2021 Apr 1;31(3):211-222. doi: 10.1093/glycob/cwaa079. PMID: 33539510
Glycomic Profiling Highlights Increased Fucosylation in Pseudomyxoma Peritonei.
Saarinen L, Nummela P, Leinonen H, Heiskanen A, Thiel A, Haglund C, Lepistö A, Satomaa T, Hautaniemi S, Ristimäki A
Mol Cell Proteomics 2018 Nov;17(11):2107-2118. Epub 2018 Aug 2 doi: 10.1074/mcp.RA118.000615. PMID: 30072579Free PMC Article

Recent systematic reviews

Efficacy and safety in the use of intraperitoneal hyperthermia chemotherapy and peritoneal cytoreductive surgery for pseudomyxoma peritonei from appendiceal neoplasm: A systematic review.
Floriano I, Silvinato A, Reis JC, Cafalli C, Bernardo WM
Clinics (Sao Paulo) 2022;77:100039. Epub 2022 May 13 doi: 10.1016/j.clinsp.2022.100039. PMID: 35576869Free PMC Article
Defining stage in mucinous tumours of the appendix with peritoneal dissemination: the importance of grading terminology: systematic review.
Martín-Román L, Lozano P, Vásquez W, Palencia N, Gómez Y, Fernández-Aceñero MJ, González-Bayón L
BJS Open 2021 Jul 6;5(4) doi: 10.1093/bjsopen/zrab059. PMID: 34355239Free PMC Article

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