Appendix cancer
{{Infobox medical condition (new)
| name = Appendix cancer
| synonyms = Appendiceal cancer
| image = Appendiceal carcinoid 1.JPG
| caption = An excised biopsy of an appendiceal carcinoid tumor
| pronounce =
| field = Oncology, general surgery
| symptoms = Bloating, discomfort in lower right abdomen, shortness of breath, loss of appetite[https://www.medstargeorgetowncancer.org/conditions/gastrointestinal-cancer/peritoneal-cancer/appendix-cancer/ MedStar Georgetown Cancer Institute]
| complications =
| onset = ~50-55 years old [https://rarediseases.org/rare-diseases/appendiceal-cancer-tumors/ Rare Diseases article]
| duration =
| types = Colonic-Type Adenocarcinoma, Non-carcinoid Appendix Tumors, Signet-Ring Cell Adenocarcinoma [https://www.medstargeorgetowncancer.org/conditions/gastrointestinal-cancer/peritoneal-cancer/appendix-cancer/ MedStar Georgetown Cancer Institute]
| causes =
| risks = Smoking, family history, Multiple endocrine neoplasia type 1[https://moffitt.org/cancers/appendiceal-appendix-cancer/causes/ Moffitt Cancer Center]
| diagnosis = Biopsy, CT Scan, MRI[https://www.medstargeorgetowncancer.org/conditions/gastrointestinal-cancer/peritoneal-cancer/appendix-cancer/ MedStar Georgetown Cancer Institute]
| differential = Acid reflux, Irritable bowel syndrome, Lactose intolerance, Stomach cancer[https://www.medstargeorgetowncancer.org/conditions/gastrointestinal-cancer/peritoneal-cancer/appendix-cancer/ MedStar Georgetown Cancer Institute]
| prevention =
| treatment = Appendectomy, chemotherapy, radiation therapy[https://www.uchicagomedicine.org/forefront/cancer-articles/2018/may/appendix-cancer-faq University of Chicago Medicine]
| medication =
| prognosis = Five-year survival rate 25-88% (U.S.) [https://www.cancer.net/cancer-types/appendix-cancer/statistics cancer.net]
| frequency = ~1,000 cases per year (U.S.)[https://www.mdanderson.org/cancer-types/appendix-cancer.html MD Anderson]
| deaths = Unknown
}}
Appendix cancer, also known as appendiceal cancer, is a very rare malignant tumor that forms in the vermiform appendix.{{Cite web |date=2016-10-18 |title=What is the Appendix? |url=https://www.news-medical.net/health/What-is-the-Appendix.aspx |access-date=2023-02-08 |website=News-Medical.net |language=en}}
Gastrointestinal stromal tumors are rare tumors with malignant potential.{{Cite journal |last1=Park |first1=Jin Hwa |last2=Kang |first2=Bo-kyeong |last3=Lee |first3=Hang Lak |last4=Yoon |first4=Jai Hoon |last5=Lee |first5=Kang Nyeong |last6=Jun |first6=Dae Won |last7=Lee |first7=Oh Young |last8=Han |first8=Dong Soo |last9=Yoon |first9=Byung Chul |last10=Choi |first10=Ho Soon |date=September 2020 |title=Correlation between Three-Dimensional Volume and Malignant Potential of Gastrointestinal Stromal Tumors (GISTs) |journal=Journal of Clinical Medicine |language=en |volume=9 |issue=9 |pages=2763 |doi=10.3390/jcm9092763 |pmid=32858945 |pmc=7564070 |issn=2077-0383 |doi-access=free }} Primary lymphomas can occur in the appendix. Breast cancer, colon cancer, and tumors of the female genital tract may metastasize to the appendix.{{cite book |last= Rosai |first= Juan |author-link= Juan Rosai |title= Rosai and Ackerman's surgical pathology |edition= 9th | orig-year = 1953 |year= 2004 |publisher= Mosby |chapter= 11. Gastrointestinal tract |pages= 761–769 }}
Diagnosis
File:Appendix neoplasms by incidence and prognosis.png
Carcinoid tumors are the most common tumors of the appendix.{{cite book |title= Sabiston Principles of Surgery |edition= 20th |page= 1308}} Other common forms are mucinous adenocarcinomas, adenocarcinoma not otherwise specified (NOS), and signet ring cell adenocarcinoma listed from highest to lowest incidence.
=Carcinoid=
File:Histopathology of appendiceal carcinoid.jpg
A carcinoid is a neuroendocrine tumor (NET) of the intestines.{{Cite web |title=Carcinoid Tumors: Symptoms, Diagnosis & Treatment |url=https://my.clevelandclinic.org/health/diseases/22414-carcinoid-tumors |access-date=2023-02-08 |website=Cleveland Clinic |language=en}} Incidence rates among carcinoids occur at about 0.15 per 100,000 per year. This subgroup makes up a large amount of neoplasias both malignant and benign. Almost 3 out of 4 of these tumors are associated with the region at the end of the appendix, and tend to be diagnosed in the 4th to 5th decades in life. Both women and Caucasian individuals show a minor prevalence regarding neuroendocrine tumor diagnosis without an explanation.{{cite journal |last1=Teixeira |first1=Frederico José Ribeiro |last2=Couto Netto |first2=Sérgio Dias do |last3=Akaishi |first3=Eduardo Hiroshi |last4=Utiyama |first4=Edivaldo Massazo |last5=Menegozzo |first5=Carlos Augusto Metidieri |last6=Rocha |first6=Marcelo Cristiano |title=Acute appendicitis, inflammatory appendiceal mass and the risk of a hidden malignant tumor: a systematic review of the literature |journal=World Journal of Emergency Surgery |date=9 March 2017 |volume=12 |issue=1 |page=12 |doi=10.1186/s13017-017-0122-9|pmid=28286544 |pmc=5343298 |doi-access=free }} Prognosis of 5 year survival rates of carcinoids averages between 70 and 80% for typical cases. Advanced cases for 5 year survival range from 12 to 28%.
=Mucinous neoplasm=
File:Histopathology of low-grade appendiceal mucinous neoplasm.jpg
Mucinous cystadenoma is an obsolete term for appendiceal mucinous neoplasm.{{cite web|url=http://www.pathologyoutlines.com/topic/appendixmucinousneoplasm.html|title=Appendix - Other tumors - Mucinous neoplasms (LAMN and HAMN)|author= Michael Feely, Raul S. Gonzalez}} Topic Completed: 1 October 2017. Revised: 11 December 2019
Treatment
Small neuroendocrine tumors{{cite journal|last1=Griniatsos|first1=J|last2=Michail|first2=O|title=Appendiceal neuroendocrine tumors: recent insights and clinical implications.|journal=World Journal of Gastrointestinal Oncology|date=2010|volume=2|issue=4|pages=192–196|doi=10.4251/wjgo.v2.i4.192|pmc=2999180|pmid=21160597|doi-access=free}} (<2 cm) without features of malignancy may be treated by appendectomy if complete removal is possible. Other neuroendocrine tumors and adenocarcinomas may require right hemicolectomy.
Pseudomyxoma peritonei treatment includes cytoreductive surgery which includes the removal of visible tumor and affected essential organs within the abdomen and pelvis. The peritoneal cavity is infused with heated chemotherapy known as HIPEC in an attempt to eradicate residual disease. The surgery may or may not be preceded or followed with intravenous chemotherapy or HIPEC.{{cite book| last1 = M. Townsend | first1 = Courtney| title = Sabiston | edition = 18th| orig-year = 1969| year = 2012| publisher = Elsevier| pages = 1289| chapter = 51. The appendix }}
Epidemiology
A study of primary malignancies in the United States found a rate of 0.12 cases per 1,000,000 population per year. Carcinoids that were not identified as malignant were not included in this data.{{Cite journal | last1 = McCusker | first1 = M. E. | last2 = Coté | first2 = T. R. | last3 = Clegg | first3 = L. X. | last4 = Sobin | first4 = L. H. | title = Primary malignant neoplasms of the appendix | journal = Cancer | volume = 94 | issue = 12 | pages = 3307–3312 | year = 2002 | pmid = 12115365 | doi = 10.1002/cncr.10589| s2cid = 40814989 | doi-access = free }} Carcinoid is found in roughly 1 in 300-400 appendectomies for acute appendicitis.{{cite book |title= Bailey and Love's Short Practice of Surgery |edition= 27th |page= 1315}}
In a systematic literature review where 4765 appendiceal cancer patients were identified, the incidence of appendiceal cancer was shown to have increased regardless of the type of tumor, age, sex, and stage of appendiceal cancer.{{cite journal |last1=Marmor |first1=Schelomo |last2=Portschy |first2=Pamela R. |last3=Tuttle |first3=Todd M. |last4=Virnig |first4=Beth A. |title=The Rise in Appendiceal Cancer Incidence: 2000–2009 |journal=Journal of Gastrointestinal Surgery |date=6 January 2015 |volume=19 |issue=4 |pages=743–750 |doi=10.1007/s11605-014-2726-7|pmid=25560182 |s2cid=24206562 }} Roughly 75% of appendiceal cases listed in the review had some form of metastases occurring. No observed trends have been noticed as to why this increase is occurring. One theory proposed is the increased use of computed tomography imaging in emergency departments since the early 1990s allowing for detection to occur before a surgery may be performed.
Malignancies in the appendix may also cause Pseudomyxoma peritonei.
Notable cases
- Actress Audrey Hepburn was diagnosed with appendiceal cancer and died of the disease in 1993.{{Cite journal |last= Selim |first= Jocelyn |title= The Fairest of All |url= http://www.crmagazine.org/archive/Fall2009/Pages/AudreyHepburnAppendixCancer.aspx |periodical= CR |volume= 4 |issue= 4 |date= Fall 2009 |publisher=American Association for Cancer Research |location= Philadelphia |access-date= 26 May 2023 |archive-url= https://web.archive.org/web/20100419051634/http://www.crmagazine.org/archive/Fall2009/Pages/AudreyHepburnAppendixCancer.aspx |archive-date= 19 April 2010 |url-status= dead }}
- In 2007, ESPN sportscast anchor Stuart Scott was diagnosed with appendiceal cancer and died of the disease in 2015.{{cite news|title=Stuart Scott Says, 'F U, Cancer!'|url=http://www.menshealth.com/health/stuart-scott-cancer?fullpage=true|access-date=7 January 2015|work=Men's Health}}{{cite news|title=Stuart Scott, ESPN's Voice of Exuberance, Dies at 49|url=https://www.nytimes.com/2015/01/05/sports/stuart-scott-espn-sportscaster-is-dead-at-49.html?_r=0|access-date=26 May 2023|url-access=subscription |work=New York Times| date=4 January 2015 | last1=Sandomir | first1=Richard }}
- Serbian musician Vlada Divljan was diagnosed in 2012, and died of subsequent complications in 2015.{{cite news|last= Nikolić|first=Aleksandar|url=http://www.blic.rs/Kultura/Vesti/530809/Vlada-Divljan-porucio-Nisam-zivotno-ugrozen-osecam-se-vrlo-dobro |title=Vlada Divljan poručio: Nisam životno ugrožen, osećam se vrlo dobro |website=Blic |date= 30 January 2015 | trans-title=Vlada Divljan said: I am not in danger, I feel very well|access-date=26 May 2023|language=sr}}
- Mexican actor Adan Canto died of appendiceal cancer at the age of 42 in 2024.{{cite news |last1=Weiss |first1=Haley |title=What to Know About Appendiceal Cancer |url=https://time.com/6554058/what-is-appendiceal-cancer-adan-canto/ |access-date=11 March 2024 |work=TIME |date=10 January 2024 |language=en}}
References
{{reflist}}
Further reading
- {{cite book |editor1-last= Hamilton |editor1-first= S.R. |editor2-last= Aaltonen |editor2-first= L.A. |year= 2000 |title= World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System |publisher= International Agency for Research on Cancer |location= Lyon |chapter= Ch. 5 Tumours of the Appendix |url= http://w2.iarc.fr/en/publications/pdfs-online/pat-gen/bb2/bb2-chap5.pdf |access-date= 2014-05-18 |archive-url= https://web.archive.org/web/20140518172831/http://w2.iarc.fr/en/publications/pdfs-online/pat-gen/bb2/bb2-chap5.pdf |archive-date= 2014-05-18 |url-status= dead }}
- {{cite journal |last1= McGory |first1= Marcia L. |last2= Maggard |first2= Melinda A. |last3= Kang |first3= Hakjung |last4= O'Connell |first4= Jessica B. |last5=Ko |first5= Clifford Y. |display-authors= 4 |title= Malignancies of the appendix: Beyond case series reports |journal= Diseases of the Colon & Rectum |volume= 48 |issue= 12 |year= 2005 |pages=2264–71 |pmid= 16258711 |doi= 10.1007/s10350-005-0196-4|s2cid= 23839281 }}
- {{cite journal |last1= O'Donnell |first1= M.E. |last2= Carson |first2= J. |last3= Garstin |first3= W.I.H. |title= Surgical treatment of malignant carcinoid tumours of the appendix |url= http://www.medscape.com/viewarticle/554797 |via= Medscape |journal= International Journal of Clinical Practice |volume= 61 |issue= 3 |year= 2006 |pages= 431–7 |pmid= 16911574 |doi= 10.1111/j.1742-1241.2006.00875.x|s2cid= 24142127 |doi-access= free }}
- {{cite journal |last1= Goede |first1= A.C. |last2= Caplin |first2= M.E. |last3= Winslet |first3= M.C. |title= Carcinoid tumour of the appendix |journal= British Journal of Surgery |volume= 90 |issue= 11 |year= 2003 |pages= 1317–22 |pmid= 14598408 |doi= 10.1002/bjs.4375|s2cid= 36569061 |doi-access= free }}
- {{cite journal |last1= Stinner |first1= B. |last2= Rothmund |first2= M. |title= Neuroendocrine tumours (carcinoids) of the appendix |journal= Best Practice & Research: Clinical Gastroenterology |volume= 19 |issue= 5 |year= 2005 |pages= 729–38 |pmid= 16253897 |doi= 10.1016/j.bpg.2005.06.003}}
External links
{{Medical resources
| DiseasesDB =
| ICD10 = {{ICD10|C|18|1|c|15}}, {{ICD10|C|78|5|c|76}}
| ICD9 = {{ICD9|153.5}}
| ICDO =
| OMIM =
| MedlinePlus =
| eMedicineSubj =
| eMedicineTopic =
| MeshID = D001063
}}
- [http://www.cancer.net/cancer-types/appendix-cancer Cancer.Net: Appendix Cancer]
{{Digestive system neoplasia}}
{{DEFAULTSORT:Appendix Cancer}}