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Appendix cancer

Appendix cancer, also known as appendiceal cancer, is a very rare malignant tumor that forms in the vermiform appendix.[7]

Appendix cancer
Other namesAppendiceal cancer
An excised biopsy of an appendiceal carcinoid tumor
SpecialtyOncology, general surgery
SymptomsBloating, discomfort in lower right abdomen, shortness of breath, loss of appetite[1]
Usual onset~50-55 years old [2]
TypesColonic-Type Adenocarcinoma, Non-carcinoid Appendix Tumors, Signet-Ring Cell Adenocarcinoma [1]
Risk factorsSmoking, family history, Multiple endocrine neoplasia type 1[3]
Diagnostic methodBiopsy, CT Scan, MRI[1]
Differential diagnosisAcid reflux, Irritable bowel syndrome, Lactose intolerance, Stomach cancer[1]
TreatmentAppendectomy, chemotherapy, radiation therapy[4]
PrognosisFive-year survival rate 25-88% (U.S.) [5]
Frequency~1,000 cases per year (U.S.)[6]
DeathsUnknown

Gastrointestinal stromal tumors are rare tumors with malignant potential.[8] Primary lymphomas can occur in the appendix. Breast cancer, colon cancer, and tumors of the female genital tract may metastasize to the appendix.[9]

Diagnosis edit

 
Appendix neoplasms by incidence and prognosis.

Carcinoid tumors are the most common tumors of the appendix.[10] Other common forms are mucinous adenocarcinomas, adenocarcinoma not otherwise specified (NOS), and signet ring cell adenocarcinoma listed from highest to lowest incidence.[11]

Carcinoid edit

 
Histopathology of an appendiceal carcinoid. The arrow points out a cluster of neuroendocrine cells. There are also inflammatory cells consistent with acute appendicitis.

A carcinoid is a neuroendocrine tumor (NET) of the intestines.[12] Incidence rates among carcinoids occur at about .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.[13] 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 edit

 
Low-grade appendiceal mucinous neoplasm: Minimal cytological atypia of the epithelial cells.[14]

Mucinous cystadenoma is an obsolete term for appendiceal mucinous neoplasm.[15]

Treatment edit

Small neuroendocrine tumors.[16](<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.[17]

Epidemiology edit

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.[18] Carcinoid is found in roughly 1 in 300-400 appendectomies for acute appendicitis.[19]

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.[11] 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.

Notable cases edit

  • Actress Audrey Hepburn was diagnosed with appendiceal cancer and died of the disease in 1993.[20]
  • In 2007, ESPN sportscast anchor Stuart Scott was diagnosed with appendiceal cancer and died of the disease in 2015.[21][22]
  • Serbian musician Vlada Divljan was diagnosed in 2012, and died of subsequent complications in 2015.[23]
  • Mexican actor Adan Canto died of appendiceal cancer at the age of 42 in 2024.[24]

References edit

  1. ^ a b c d MedStar Georgetown Cancer Institute
  2. ^ Rare Diseases article
  3. ^ Moffitt Cancer Center
  4. ^ University of Chicago Medicine
  5. ^ cancer.net
  6. ^ MD Anderson
  7. ^ "What is the Appendix?". News-Medical.net. 2016-10-18. Retrieved 2023-02-08.
  8. ^ Park, Jin Hwa; Kang, Bo-kyeong; Lee, Hang Lak; Yoon, Jai Hoon; Lee, Kang Nyeong; Jun, Dae Won; Lee, Oh Young; Han, Dong Soo; Yoon, Byung Chul; Choi, Ho Soon (September 2020). "Correlation between Three-Dimensional Volume and Malignant Potential of Gastrointestinal Stromal Tumors (GISTs)". Journal of Clinical Medicine. 9 (9): 2763. doi:10.3390/jcm9092763. ISSN 2077-0383. PMC 7564070. PMID 32858945.
  9. ^ Rosai, Juan (2004) [1953]. "11. Gastrointestinal tract". Rosai and Ackerman's surgical pathology (9th ed.). Mosby. pp. 761–769.
  10. ^ Sabiston Principles of Surgery (20th ed.). p. 1308.
  11. ^ a b Marmor, Schelomo; Portschy, Pamela R.; Tuttle, Todd M.; Virnig, Beth A. (6 January 2015). "The Rise in Appendiceal Cancer Incidence: 2000–2009". Journal of Gastrointestinal Surgery. 19 (4): 743–750. doi:10.1007/s11605-014-2726-7. PMID 25560182. S2CID 24206562.
  12. ^ "Carcinoid Tumors: Symptoms, Diagnosis & Treatment". Cleveland Clinic. Retrieved 2023-02-08.
  13. ^ Teixeira, Frederico José Ribeiro; Couto Netto, Sérgio Dias do; Akaishi, Eduardo Hiroshi; Utiyama, Edivaldo Massazo; Menegozzo, Carlos Augusto Metidieri; Rocha, Marcelo Cristiano (9 March 2017). "Acute appendicitis, inflammatory appendiceal mass and the risk of a hidden malignant tumor: a systematic review of the literature". World Journal of Emergency Surgery. 12 (1): 12. doi:10.1186/s13017-017-0122-9. PMC 5343298. PMID 28286544.
  14. ^ Hajjar, Roy; Dubé, Pierre; Mitchell, Andrew; Sidéris, Lucas (2019). "Combined Mucinous and Neuroendocrine Tumours of the Appendix Managed with Surgical Cytoreduction and Oxaliplatin-based Hyperthermic Intraperitoneal Chemotherapy". Cureus. 11 (1): e3894. doi:10.7759/cureus.3894. ISSN 2168-8184. PMC 6424473. PMID 30911451.
  15. ^ Michael Feely, Raul S. Gonzalez. "Appendix - Other tumors - Mucinous neoplasms (LAMN and HAMN)". Topic Completed: 1 October 2017. Revised: 11 December 2019
  16. ^ Griniatsos, J; Michail, O (2010). "Appendiceal neuroendocrine tumors: recent insights and clinical implications". World Journal of Gastrointestinal Oncology. 2 (4): 192–196. doi:10.4251/wjgo.v2.i4.192. PMC 2999180. PMID 21160597.
  17. ^ M. Townsend, Courtney (2012) [1969]. "51. The appendix". Sabiston (18th ed.). Elsevier. p. 1289.
  18. ^ McCusker, M. E.; Coté, T. R.; Clegg, L. X.; Sobin, L. H. (2002). "Primary malignant neoplasms of the appendix". Cancer. 94 (12): 3307–3312. doi:10.1002/cncr.10589. PMID 12115365. S2CID 40814989.
  19. ^ Bailey and Love's Short Practice of Surgery (27th ed.). p. 1315.
  20. ^ Selim, Jocelyn (Fall 2009). . CR. 4 (4). Philadelphia: American Association for Cancer Research. Archived from the original on 19 April 2010. Retrieved 26 May 2023.
  21. ^ "Stuart Scott Says, 'F U, Cancer!'". Men's Health. Retrieved 7 January 2015.
  22. ^ Sandomir, Richard (4 January 2015). "Stuart Scott, ESPN's Voice of Exuberance, Dies at 49". New York Times. Retrieved 26 May 2023.
  23. ^ Nikolić, Aleksandar (30 January 2015). "Vlada Divljan poručio: Nisam životno ugrožen, osećam se vrlo dobro" [Vlada Divljan said: I am not in danger, I feel very well]. Blic (in Serbian). Retrieved 26 May 2023.
  24. ^ Weiss, Haley (10 January 2024). "What to Know About Appendiceal Cancer". TIME. Retrieved 11 March 2024.

Further reading edit

  • Hamilton, S.R.; Aaltonen, L.A., eds. (2000). "Ch. 5 Tumours of the Appendix". (PDF). Lyon: International Agency for Research on Cancer. Archived from the original (PDF) on 2014-05-18. Retrieved 2014-05-18.
  • McGory, Marcia L.; Maggard, Melinda A.; Kang, Hakjung; O'Connell, Jessica B.; et al. (2005). "Malignancies of the appendix: Beyond case series reports". Diseases of the Colon & Rectum. 48 (12): 2264–71. doi:10.1007/s10350-005-0196-4. PMID 16258711. S2CID 23839281.
  • O'Donnell, M.E.; Carson, J.; Garstin, W.I.H. (2006). "Surgical treatment of malignant carcinoid tumours of the appendix". International Journal of Clinical Practice. 61 (3): 431–7. doi:10.1111/j.1742-1241.2006.00875.x. PMID 16911574. S2CID 24142127 – via Medscape.
  • Goede, A.C.; Caplin, M.E.; Winslet, M.C. (2003). "Carcinoid tumour of the appendix". British Journal of Surgery. 90 (11): 1317–22. doi:10.1002/bjs.4375. PMID 14598408. S2CID 36569061.
  • Stinner, B.; Rothmund, M. (2005). "Neuroendocrine tumours (carcinoids) of the appendix". Best Practice & Research: Clinical Gastroenterology. 19 (5): 729–38. doi:10.1016/j.bpg.2005.06.003. PMID 16253897.

External links edit

  • Cancer.Net: Appendix Cancer

appendix, cancer, also, known, appendiceal, cancer, very, rare, malignant, tumor, that, forms, vermiform, appendix, other, namesappendiceal, canceran, excised, biopsy, appendiceal, carcinoid, tumorspecialtyoncology, general, surgerysymptomsbloating, discomfort. Appendix cancer also known as appendiceal cancer is a very rare malignant tumor that forms in the vermiform appendix 7 Appendix cancerOther namesAppendiceal cancerAn excised biopsy of an appendiceal carcinoid tumorSpecialtyOncology general surgerySymptomsBloating discomfort in lower right abdomen shortness of breath loss of appetite 1 Usual onset 50 55 years old 2 TypesColonic Type Adenocarcinoma Non carcinoid Appendix Tumors Signet Ring Cell Adenocarcinoma 1 Risk factorsSmoking family history Multiple endocrine neoplasia type 1 3 Diagnostic methodBiopsy CT Scan MRI 1 Differential diagnosisAcid reflux Irritable bowel syndrome Lactose intolerance Stomach cancer 1 TreatmentAppendectomy chemotherapy radiation therapy 4 PrognosisFive year survival rate 25 88 U S 5 Frequency 1 000 cases per year U S 6 DeathsUnknown Gastrointestinal stromal tumors are rare tumors with malignant potential 8 Primary lymphomas can occur in the appendix Breast cancer colon cancer and tumors of the female genital tract may metastasize to the appendix 9 Contents 1 Diagnosis 1 1 Carcinoid 1 2 Mucinous neoplasm 2 Treatment 3 Epidemiology 4 Notable cases 5 References 6 Further reading 7 External linksDiagnosis edit nbsp Appendix neoplasms by incidence and prognosis Carcinoid tumors are the most common tumors of the appendix 10 Other common forms are mucinous adenocarcinomas adenocarcinoma not otherwise specified NOS and signet ring cell adenocarcinoma listed from highest to lowest incidence 11 Carcinoid edit nbsp Histopathology of an appendiceal carcinoid The arrow points out a cluster of neuroendocrine cells There are also inflammatory cells consistent with acute appendicitis A carcinoid is a neuroendocrine tumor NET of the intestines 12 Incidence rates among carcinoids occur at about 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 13 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 edit nbsp Low grade appendiceal mucinous neoplasm Minimal cytological atypia of the epithelial cells 14 Mucinous cystadenoma is an obsolete term for appendiceal mucinous neoplasm 15 Treatment editSmall neuroendocrine tumors 16 lt 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 17 Epidemiology editA 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 18 Carcinoid is found in roughly 1 in 300 400 appendectomies for acute appendicitis 19 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 11 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 Notable cases editActress Audrey Hepburn was diagnosed with appendiceal cancer and died of the disease in 1993 20 In 2007 ESPN sportscast anchor Stuart Scott was diagnosed with appendiceal cancer and died of the disease in 2015 21 22 Serbian musician Vlada Divljan was diagnosed in 2012 and died of subsequent complications in 2015 23 Mexican actor Adan Canto died of appendiceal cancer at the age of 42 in 2024 24 References edit a b c d MedStar Georgetown Cancer Institute Rare Diseases article Moffitt Cancer Center University of Chicago Medicine cancer net MD Anderson What is the Appendix News Medical net 2016 10 18 Retrieved 2023 02 08 Park Jin Hwa Kang Bo kyeong Lee Hang Lak Yoon Jai Hoon Lee Kang Nyeong Jun Dae Won Lee Oh Young Han Dong Soo Yoon Byung Chul Choi Ho Soon September 2020 Correlation between Three Dimensional Volume and Malignant Potential of Gastrointestinal Stromal Tumors GISTs Journal of Clinical Medicine 9 9 2763 doi 10 3390 jcm9092763 ISSN 2077 0383 PMC 7564070 PMID 32858945 Rosai Juan 2004 1953 11 Gastrointestinal tract Rosai and Ackerman s surgical pathology 9th ed Mosby pp 761 769 Sabiston Principles of Surgery 20th ed p 1308 a b Marmor Schelomo Portschy Pamela R Tuttle Todd M Virnig Beth A 6 January 2015 The Rise in Appendiceal Cancer Incidence 2000 2009 Journal of Gastrointestinal Surgery 19 4 743 750 doi 10 1007 s11605 014 2726 7 PMID 25560182 S2CID 24206562 Carcinoid Tumors Symptoms Diagnosis amp Treatment Cleveland Clinic Retrieved 2023 02 08 Teixeira Frederico Jose Ribeiro Couto Netto Sergio Dias do Akaishi Eduardo Hiroshi Utiyama Edivaldo Massazo Menegozzo Carlos Augusto Metidieri Rocha Marcelo Cristiano 9 March 2017 Acute appendicitis inflammatory appendiceal mass and the risk of a hidden malignant tumor a systematic review of the literature World Journal of Emergency Surgery 12 1 12 doi 10 1186 s13017 017 0122 9 PMC 5343298 PMID 28286544 Hajjar Roy Dube Pierre Mitchell Andrew Sideris Lucas 2019 Combined Mucinous and Neuroendocrine Tumours of the Appendix Managed with Surgical Cytoreduction and Oxaliplatin based Hyperthermic Intraperitoneal Chemotherapy Cureus 11 1 e3894 doi 10 7759 cureus 3894 ISSN 2168 8184 PMC 6424473 PMID 30911451 Michael Feely Raul S Gonzalez Appendix Other tumors Mucinous neoplasms LAMN and HAMN Topic Completed 1 October 2017 Revised 11 December 2019 Griniatsos J Michail O 2010 Appendiceal neuroendocrine tumors recent insights and clinical implications World Journal of Gastrointestinal Oncology 2 4 192 196 doi 10 4251 wjgo v2 i4 192 PMC 2999180 PMID 21160597 M Townsend Courtney 2012 1969 51 The appendix Sabiston 18th ed Elsevier p 1289 McCusker M E Cote T R Clegg L X Sobin L H 2002 Primary malignant neoplasms of the appendix Cancer 94 12 3307 3312 doi 10 1002 cncr 10589 PMID 12115365 S2CID 40814989 Bailey and Love s Short Practice of Surgery 27th ed p 1315 Selim Jocelyn Fall 2009 The Fairest of All CR 4 4 Philadelphia American Association for Cancer Research Archived from the original on 19 April 2010 Retrieved 26 May 2023 Stuart Scott Says F U Cancer Men s Health Retrieved 7 January 2015 Sandomir Richard 4 January 2015 Stuart Scott ESPN s Voice of Exuberance Dies at 49 New York Times Retrieved 26 May 2023 Nikolic Aleksandar 30 January 2015 Vlada Divljan porucio Nisam zivotno ugrozen osecam se vrlo dobro Vlada Divljan said I am not in danger I feel very well Blic in Serbian Retrieved 26 May 2023 Weiss Haley 10 January 2024 What to Know About Appendiceal Cancer TIME Retrieved 11 March 2024 Further reading editHamilton S R Aaltonen L A eds 2000 Ch 5 Tumours of the Appendix World Health Organization Classification of Tumours Pathology and Genetics of Tumours of the Digestive System PDF Lyon International Agency for Research on Cancer Archived from the original PDF on 2014 05 18 Retrieved 2014 05 18 McGory Marcia L Maggard Melinda A Kang Hakjung O Connell Jessica B et al 2005 Malignancies of the appendix Beyond case series reports Diseases of the Colon amp Rectum 48 12 2264 71 doi 10 1007 s10350 005 0196 4 PMID 16258711 S2CID 23839281 O Donnell M E Carson J Garstin W I H 2006 Surgical treatment of malignant carcinoid tumours of the appendix International Journal of Clinical Practice 61 3 431 7 doi 10 1111 j 1742 1241 2006 00875 x PMID 16911574 S2CID 24142127 via Medscape Goede A C Caplin M E Winslet M C 2003 Carcinoid tumour of the appendix British Journal of Surgery 90 11 1317 22 doi 10 1002 bjs 4375 PMID 14598408 S2CID 36569061 Stinner B Rothmund M 2005 Neuroendocrine tumours carcinoids of the appendix Best Practice amp Research Clinical Gastroenterology 19 5 729 38 doi 10 1016 j bpg 2005 06 003 PMID 16253897 External links editCancer Net Appendix Cancer Retrieved from https en wikipedia org w index php title Appendix cancer amp oldid 1213241501 Histopathologic subtypes, wikipedia, wiki, book, books, library,

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