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Low Grade Appendix Mucinous Neoplasm

MedGen UID:
317841
•Concept ID:
C1708747
•
Neoplastic Process
Synonyms: Appendix Well Differentiated Mucinous Adenocarcinoma; Low Grade Appendiceal Mucinous Neoplasm; Low grade appendiceal mucinous neoplasm; Low-Grade Appendiceal Mucinous Neoplasm; Low-Grade Appendix Mucinous Neoplasm
SNOMED CT: Low grade appendiceal mucinous neoplasm (450896006)

Definition

A well differentiated mucinous neoplasm that arises from the appendix. It is characterized by slow growth and it is associated with the development of pseudomyxoma peritonei. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLow Grade Appendix Mucinous Neoplasm

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
Pseudomyxoma peritonei: natural history and treatment.
Mittal R, Chandramohan A, Moran B
Int J Hyperthermia 2017 Aug;33(5):511-519. doi: 10.1080/02656736.2017.1310938. PMID: 28540829

Recent clinical studies

Etiology

Cystic neoplasm of the appendix. Report of an unusual clinical case at the South Central High Specialty Hospital PEMEX.
Chama-Naranjo A, Chávez-Villanueva U, Farell-Rivas J
Cir Cir 2020;88(Suppl 2):21-23. doi: 10.24875/CIRU.20000134. PMID: 33284271
Diagnostic concordance in cases of appendiceal mucinous neoplasia referred to a tertiary referral centre.
Amin A, Carr N
J Clin Pathol 2019 Sep;72(9):639-641. Epub 2019 Jun 19 doi: 10.1136/jclinpath-2019-205945. PMID: 31217254
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
Malignant pseudomyxoma peritonei of colonic origin. Natural history and presentation of a curative approach to treatment.
Sugarbaker PH, Kern K, Lack E
Dis Colon Rectum 1987 Oct;30(10):772-9. doi: 10.1007/BF02554625. PMID: 2820671

Diagnosis

Low-grade appendiceal mucinous neoplasm.
Dong J, Tian Y
Clin Res Hepatol Gastroenterol 2021 May;45(3):101647. Epub 2021 Feb 12 doi: 10.1016/j.clinre.2021.101647. PMID: 33588100
Cystic neoplasm of the appendix. Report of an unusual clinical case at the South Central High Specialty Hospital PEMEX.
Chama-Naranjo A, Chávez-Villanueva U, Farell-Rivas J
Cir Cir 2020;88(Suppl 2):21-23. doi: 10.24875/CIRU.20000134. PMID: 33284271
A case report of a giant appendiceal mucocele and literature review.
Motsumi MJ, Motlaleselelo P, Ayane G, Sesay SO, Valdes JR
Pan Afr Med J 2017;28:106. Epub 2017 Oct 4 doi: 10.11604/pamj.2017.28.106.13832. PMID: 29515724Free PMC Article
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

Pathogenesis of histologic variations of appendiceal mucinous neoplasms.
Sugarbaker PH, Chang D, Liang J
Eur J Surg Oncol 2023 May;49(5):895-901. Epub 2023 Feb 27 doi: 10.1016/j.ejso.2023.02.014. PMID: 36863914
Management of pseudomyxoma peritonei.
Fallis SA, Moran BJ
J BUON 2015 May;20 Suppl 1:S47-55. PMID: 26051332
The Diagnostic Significance of a Mucocele of the Appendix.
Craig DH, Ruddy JM, Pullatt RC, Hardie AD, Camp ER
J S C Med Assoc 2014 Mar-Apr;110(1):12-3. PMID: 27125005
Pseudomyxoma peritonei of appendiceal origin. The Memorial Sloan-Kettering Cancer Center experience.
Smith JW, Kemeny N, Caldwell C, Banner P, Sigurdson E, Huvos A
Cancer 1992 Jul 15;70(2):396-401. doi: 10.1002/1097-0142(19920715)70:2<396::aid-cncr2820700205>3.0.co;2-a. PMID: 1319813
Malignant pseudomyxoma peritonei of colonic origin. Natural history and presentation of a curative approach to treatment.
Sugarbaker PH, Kern K, Lack E
Dis Colon Rectum 1987 Oct;30(10):772-9. doi: 10.1007/BF02554625. PMID: 2820671

Prognosis

Cystic neoplasm of the appendix. Report of an unusual clinical case at the South Central High Specialty Hospital PEMEX.
Chama-Naranjo A, Chávez-Villanueva U, Farell-Rivas J
Cir Cir 2020;88(Suppl 2):21-23. doi: 10.24875/CIRU.20000134. PMID: 33284271
Diagnostic concordance in cases of appendiceal mucinous neoplasia referred to a tertiary referral centre.
Amin A, Carr N
J Clin Pathol 2019 Sep;72(9):639-641. Epub 2019 Jun 19 doi: 10.1136/jclinpath-2019-205945. PMID: 31217254
Radical appendectomy as an alternative to right colon resection in patients with epithelial appendiceal neoplasms.
González-Moreno S, Sugarbaker PH
Surg Oncol 2017 Mar;26(1):86-90. Epub 2017 Feb 1 doi: 10.1016/j.suronc.2017.01.006. PMID: 28317590
Management of pseudomyxoma peritonei.
Fallis SA, Moran BJ
J BUON 2015 May;20 Suppl 1:S47-55. PMID: 26051332
Pseudomyxoma peritonei of appendiceal origin. The Memorial Sloan-Kettering Cancer Center experience.
Smith JW, Kemeny N, Caldwell C, Banner P, Sigurdson E, Huvos A
Cancer 1992 Jul 15;70(2):396-401. doi: 10.1002/1097-0142(19920715)70:2<396::aid-cncr2820700205>3.0.co;2-a. PMID: 1319813

Clinical prediction guides

Mucinous appendiceal neoplasms and pseudomyxoma peritonei: Morphological and clinical findings, differential diagnosis, and prognosis.
Akay E, Arslan A, Eren SK, Özhan N, Karaman H
Indian J Pathol Microbiol 2022 Jul-Sep;65(3):565-571. doi: 10.4103/ijpm.ijpm_827_21. PMID: 35900483
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
Diagnostic concordance in cases of appendiceal mucinous neoplasia referred to a tertiary referral centre.
Amin A, Carr N
J Clin Pathol 2019 Sep;72(9):639-641. Epub 2019 Jun 19 doi: 10.1136/jclinpath-2019-205945. PMID: 31217254
Ileocecal Intussusception Caused by an Appendiceal Neoplasm.
Chua TC, Gill P, Gill AJ, Samra JS
J Gastrointest Surg 2016 Apr;20(4):867-8. Epub 2016 Jan 29 doi: 10.1007/s11605-015-3037-3. PMID: 26831058

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
Clinicopathological Features and Management of Appendiceal Mucoceles: A Systematic Review.
Morano WF, Gleeson EM, Sullivan SH, Padmanaban V, Mapow BL, Shewokis PA, Esquivel J, Bowne WB
Am Surg 2018 Feb 1;84(2):273-281. PMID: 29580358

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