Carcinoid syndrome surgery: Difference between revisions

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==Surgery==
==Surgery==
[[Surgery]], if feasible, is the only curative therapy. If the [[tumor]] has [[metastasized]] (most commonly, to the [[liver]]), the [[tumor]] may be ineligible for [[surgery]] (unresectable), though there are many promising treatment modalities, such as [[radiolabeled]] [[octreotide]], that are very effective in arresting the [[growth]] of the [[tumors]] and prolonging [[Survival rate|survival]] in patients with [[Liver (2)|liver]] [[metastases]].  
[[Surgery]], if feasible, is the only curative therapy. If the [[tumor]] has [[metastasized]] (most commonly, to the [[liver]]), the [[tumor]] may be ineligible for [[surgery]] (unresectable), though there are many promising treatment modalities, such as radiolabeled [[octreotide]], that are very effective in arresting the [[growth]] of the [[tumors]] and prolonging [[Survival rate|survival]] in patients with [[Liver (2)|liver]] [[metastases]].  


===Gastric Carcinoids===
===Gastric Carcinoids===
Line 17: Line 17:
* Type II [[Carcinoid tumor|carcinoids tumor]] can usually be managed with [[endoscopic]] resection (tumors <1 cm) followed by close endoscopic surveillance.
* Type II [[Carcinoid tumor|carcinoids tumor]] can usually be managed with [[endoscopic]] resection (tumors <1 cm) followed by close endoscopic surveillance.
* For larger or multifocal tumors liberal surgical excision or [[Gastric|gastric resection]] with regional [[lymphadenectomy]] is performed.
* For larger or multifocal tumors liberal surgical excision or [[Gastric|gastric resection]] with regional [[lymphadenectomy]] is performed.
* For type III [[Carcinoid tumor|gastric carcinoids]] larger than 2 cm or those with atypical histology or [[gastric wall]] invasion are most appropriately dealt with by [[gastrectomy]] or radical [[gastrectomy]].
* For type III [[Carcinoid tumor|gastric carcinoids]] larger than 2 cm or those with atypical histology or [[Gastric|gastric wall]] invasion are most appropriately dealt with by [[gastrectomy]] or radical [[gastrectomy]].


=== Duodenal carcinoids ===
=== Duodenal carcinoids ===
Line 33: Line 33:


===Jejunal and Ileal Carcinoids===
===Jejunal and Ileal Carcinoids===
*[[Carcinoid tumor|Carcinoids]] of the [[small intestine]] have metastatized in the regional [[lymph nodes]] or the [[liver]] in 50%-60% of cases<ref name="surgery">Treatment Option Overview for gastrointestinal Carcinoid Tumors
*[[Carcinoid tumors|Carcinoids]] of the [[small intestine]] have metastatized in the regional [[lymph nodes]] or the [[liver]] in 50%-60% of cases<ref name="surgery">Treatment Option Overview for gastrointestinal Carcinoid Tumors
. NATIONAL CANCER INSTITUTE . http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_97_toc Accessed on September 22, 2015</ref>
. NATIONAL CANCER INSTITUTE . http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_97_toc Accessed on September 22, 2015</ref>
*Early surgical treatment should include removal of the [[mesentery]] by wedge resection and resection of lymph node metastases surrounding the [[mesenteric artery]] and vein to preserve intestinal vascular supply and to limit the intestinal resection.<ref name="pmid20664473">{{cite journal |vauthors=Boudreaux JP, Klimstra DS, Hassan MM, Woltering EA, Jensen RT, Goldsmith SJ, Nutting C, Bushnell DL, Caplin ME, Yao JC |title=The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the Jejunum, Ileum, Appendix, and Cecum |journal=Pancreas |volume=39 |issue=6 |pages=753–66 |date=August 2010 |pmid=20664473 |doi=10.1097/MPA.0b013e3181ebb2a5 |url=}}</ref>
*Early [[Surgery|surgical treatment]] should include removal of the [[mesentery]] by wedge [[resection]] and resection of [[Lymph nodes|lymph node]] [[metastases]] surrounding the [[mesenteric artery]] and [[Veins|vein]] to preserve [[Intestine|intestinal]] [[Blood|vascular supply]] and to limit the [[intestinal]] resection.<ref name="pmid20664473">{{cite journal |vauthors=Boudreaux JP, Klimstra DS, Hassan MM, Woltering EA, Jensen RT, Goldsmith SJ, Nutting C, Bushnell DL, Caplin ME, Yao JC |title=The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the Jejunum, Ileum, Appendix, and Cecum |journal=Pancreas |volume=39 |issue=6 |pages=753–66 |date=August 2010 |pmid=20664473 |doi=10.1097/MPA.0b013e3181ebb2a5 |url=}}</ref>
*Surgical treatment for advanced carcinoids involves prophylactic removal of [[mesenteric]] metastases early on because later the disease may become impossible to manage surgically.
*Surgical treatment for advanced carcinoids involves prophylactic removal of [[mesenteric]] [[metastases]] early on because later the [[disease]] may become impossible to manage surgically.
*Due to fibrosis between regions of the intestine,surgery may result in fistulation, intestinal devascularization, or creation of a short bowel.
*Due to [[fibrosis]] between regions of the [[intestine]],surgery may result in [[fistulae]], [[intestinal]] devascularization, or creation of a short [[bowel]].


===Appendiceal Carcinoids===
===Appendiceal Carcinoids===
*Appendiceal carcinoids smaller than 1 are cured by [[appendectomy]].<ref name="pmid20664473">{{cite journal |vauthors=Boudreaux JP, Klimstra DS, Hassan MM, Woltering EA, Jensen RT, Goldsmith SJ, Nutting C, Bushnell DL, Caplin ME, Yao JC |title=The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the Jejunum, Ileum, Appendix, and Cecum |journal=Pancreas |volume=39 |issue=6 |pages=753–66 |date=August 2010 |pmid=20664473 |doi=10.1097/MPA.0b013e3181ebb2a5 |url=}}</ref>
*[[Appendix|Appendiceal]] [[Carcinoid tumor|carcinoids]] smaller than 1 are cured by [[appendectomy]].<ref name="pmid20664473">{{cite journal |vauthors=Boudreaux JP, Klimstra DS, Hassan MM, Woltering EA, Jensen RT, Goldsmith SJ, Nutting C, Bushnell DL, Caplin ME, Yao JC |title=The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the Jejunum, Ileum, Appendix, and Cecum |journal=Pancreas |volume=39 |issue=6 |pages=753–66 |date=August 2010 |pmid=20664473 |doi=10.1097/MPA.0b013e3181ebb2a5 |url=}}</ref>
*Appendiceal carcinoids larger than 2 cm require right-sided hemicolectomy and ileocecal lymphadenectomy because of the significant risk of metastasis.<ref name="surgery">Treatment Option Overview for gastrointestinal Carcinoid Tumors
*[[Appendix|Appendiceal]] [[Carcinoid tumor|carcinoids]] larger than 2 cm require right-sided [[hemicolectomy]] and [[ileocecal]] [[lymphadenectomy]] because of the significant risk of [[Metastasis|metastasis.]]<ref name="surgery">Treatment Option Overview for gastrointestinal Carcinoid Tumors
. NATIONAL CANCER INSTITUTE . http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_97_toc Accessed on September 22, 2015</ref>
. NATIONAL CANCER INSTITUTE . http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_97_toc Accessed on September 22, 2015</ref>
*For tumors between 1 to 2 cm, treatment is controversial, but hemicolectomy may be appropriate if there is invasion in the [[mesoappendix]], if there is residual tumor in the resection margins, or in the presence of [[lymph node]] metastases.
*For [[tumors]] between 1 to 2 cm, treatment is controversial, but [[hemicolectomy]] may be appropriate if there is [[invasion]] in the [[mesoappendix]], if there is residual [[tumor]] in the resection margins, or in the presence of [[lymph node]] [[metastases]].


===Colonic Carcinoids===
===Colonic Carcinoids===
*Colonic carcinoid tumors of the colon are treated similarly to adenocarcinoma of the colon.
*[[Colon|Colonic]] [[carcinoid tumors]] of the [[colon]] are treated similarly to [[adenocarcinoma]] of the [[colon]].
*Radical resection by [[hemicolectomy]] or subtotal colectomy with [[lymphadenectomy]] should be done for colonic carcinoids.
*Radical resection by [[hemicolectomy]] or subtotal [[colectomy]] with [[lymphadenectomy]] should be done for [[Colon|colonic]] [[Carcinoid tumors|carcinoids tumors]].


===Rectal Carcinoids===
===Rectal Carcinoids===
*Tumors smaller than 1 cm can be removed by endoscopic excision but excised specimens should be examined histologically to exclude muscularis invasion.<ref name="pmid8044398">{{cite journal |vauthors=Mani S, Modlin IM, Ballantyne G, Ahlman H, West B |title=Carcinoids of the rectum |journal=J. Am. Coll. Surg. |volume=179 |issue=2 |pages=231–48 |date=August 1994 |pmid=8044398 |doi= |url=}}</ref>
*[[Tumors]] smaller than 1 cm can be removed by [[endoscopic]] excision but excised specimens should be examined [[histologically]] to exclude [[Muscularis mucosa|muscularis]] [[invasion]].<ref name="pmid8044398">{{cite journal |vauthors=Mani S, Modlin IM, Ballantyne G, Ahlman H, West B |title=Carcinoids of the rectum |journal=J. Am. Coll. Surg. |volume=179 |issue=2 |pages=231–48 |date=August 1994 |pmid=8044398 |doi= |url=}}</ref>
*Patients with tumors that are greater than 2 cm or that have invasion of the muscularis as seen by [[endoscopic ultrasound]] or MRI, surgical resection with abdominoperineal resection (APR) or low anterior resection (LAR) is recommended because of the high rate of nodal metastases and risk of distant metastatic disease.
*Patients with [[tumors]] that are greater than 2 cm or that have [[invasion]] of the [[muscularis]] as seen by [[endoscopic ultrasound]] or [[MRI]], surgical resection with [[abdominoperineal resection]] (APR) or low anterior resection (LAR) is recommended because of the high rate of [[Lymph nodes|nodal]] [[metastases]] and risk of distant [[metastatic]] [[disease]].
*There is considerable debate about whether local excision or rectal resection (i.e., APR or LAR) is needed for tumors that are 1 cm to 2 cm.  
*There is considerable debate about whether local excision or [[rectal]] resection (i.e., APR or LAR) is needed for [[tumors]] that are 1 cm to 2 cm.  
*Although it may be possible to recognize tumors with particular atypia and high mitotic index before embarking on the more radical surgery, the presence of muscularis invasion or regional metastases generally supports rectal resection.
*Although it may be possible to recognize [[tumors]] with particular atypia and high [[mitotic index]] before embarking on the more radical [[surgery]], the presence of [[muscularis]] [[invasion]] or regional [[metastases]] generally supports [[rectal]] resection.


===Metastatic Gastrointestinal Carcinoid Tumors===
===Metastatic Gastrointestinal Carcinoid Tumors===
Definitive role of surgery in metastatic disease has not been established, conservative resections of the [[intestine]] mesenteric tumors, and fibrotic areas may improve symptoms and quality of life substantially in patients with metastatic [[hepatic]], [[mesenteric]], and [[peritoneal]] carcinoids.<ref name="surgery">Treatment Option Overview for gastrointestinal Carcinoid Tumors
Definitive role of [[surgery]] in [[metastatic]] disease has not been established, conservative [[resections]] of the [[intestine]] [[mesenteric]] [[tumors]], and [[Fibrosis|fibrotic]] areas may improve [[symptoms]] and quality of life substantially in patients with [[metastatic]] [[hepatic]], [[mesenteric]], and [[peritoneal]] carcinoids.<ref name="surgery">Treatment Option Overview for gastrointestinal Carcinoid Tumors
. NATIONAL CANCER INSTITUTE . http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_97_toc Accessed on September 22, 2015</ref><ref name="ModlinLye2003">{{cite journal|last1=Modlin|first1=Irvin M.|last2=Lye|first2=Kevin D.|last3=Kidd|first3=Mark|title=A 5-decade analysis of 13,715 carcinoid tumors|journal=Cancer|volume=97|issue=4|year=2003|pages=934–959|issn=0008-543X|doi=10.1002/cncr.11105}}</ref>
. NATIONAL CANCER INSTITUTE . http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_97_toc Accessed on September 22, 2015</ref><ref name="ModlinLye2003">{{cite journal|last1=Modlin|first1=Irvin M.|last2=Lye|first2=Kevin D.|last3=Kidd|first3=Mark|title=A 5-decade analysis of 13,715 carcinoid tumors|journal=Cancer|volume=97|issue=4|year=2003|pages=934–959|issn=0008-543X|doi=10.1002/cncr.11105}}</ref>


*The primary tumor should be resected to prevent an emergency presentation with [[obstruction]], [[perforation]], or [[bleeding]] if the condition of the patient is such that surgery is not a greater risk than the disease
*The primary tumor should be resected to prevent an emergency presentation with [[obstruction]], [[perforation]], or [[bleeding]] if the condition of the patient is such that surgery is not a greater risk than the disease
*Management of hepatic metastases may include:
*Management of [[hepatic]] [[metastases]] may include:
#Surgical resection
#[[Surgical]] resection
#Hepatic artery [[embolization]]
#[[Hepatic artery]] [[embolization]]
#[[Cryoablation]]
#[[Cryoablation]]
#[[Radiofrequency ablation]]
#[[Radiofrequency ablation]]
#Orthotopic [[liver transplantation]].
#Orthotopic [[liver transplantation]].
*In the case of liver metastases, localization and resection of the primary tumor may be considered, even among patients in whom the primary neoplasm is asymptomatic.
*In the case of [[Liver (2)|liver]] [[metastases]], localization and resection of the [[primary tumor]] may be considered, even among patients in whom the [[Neoplasm|primary neoplasm]] is [[Asymptomatic condition|asymptomatic]].
*Palliative [[radiation therapy]] has some efficacy for bone and brain metastases and in the management of [[spinal cord]] metastases.
*[[Palliative therapy|Palliative]] [[radiation therapy]] has some [[efficacy]] for [[bone]] and [[brain]] [[metastases]] and in the management of [[spinal cord]] [[Metastases|metastase]]<nowiki/>s.


===Recurrent gastrointestinal Carcinoid Tumors===
===Recurrent gastrointestinal Carcinoid Tumors===


Treatment of recurrent gastrointestinal carcinoid tumor depends on many factors:<ref name="surgery">Treatment Option Overview for gastrointestinal Carcinoid Tumors
Treatment of recurrent [[gastrointestinal]] [[carcinoid tumor]] depends on many factors:<ref name="surgery">Treatment Option Overview for gastrointestinal Carcinoid Tumors
. NATIONAL CANCER INSTITUTE . http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_97_toc Accessed on September 22, 2015</ref>
. NATIONAL CANCER INSTITUTE . http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_97_toc Accessed on September 22, 2015</ref>
*Previous treatment
*Previous treatment
*Site of recurrence  
*Site of recurrence  
*Individual patient considerations
*Individual [[patient]] considerations


==Carcinoid heart disease==
==Carcinoid heart disease==
*Valve replacement is the most effective treatment option for advanced carcinoid heart disease.{{cite web |url=https://doi.org/10.1007/s11936-013-0265-2 |title=Carcinoid Valve Disease &#124; SpringerLink |format= |work= |accessdate=}}<ref name="pmid26564596">{{cite journal |vauthors=Connolly HM, Schaff HV, Abel MD, Rubin J, Askew JW, Li Z, Inda JJ, Luis SA, Nishimura RA, Pellikka PA |title=Early and Late Outcomes of Surgical Treatment in Carcinoid Heart Disease |journal=J. Am. Coll. Cardiol. |volume=66 |issue=20 |pages=2189–2196 |date=November 2015 |pmid=26564596 |doi=10.1016/j.jacc.2015.09.014 |url=}}</ref>
*[[Valve replacement]] is the most effective treatment option for advanced [[Carcinoid disease|carcinoid heart disease]].{{cite web |url=https://doi.org/10.1007/s11936-013-0265-2 |title=Carcinoid Valve Disease &#124; SpringerLink |format= |work= |accessdate=}}<ref name="pmid26564596">{{cite journal |vauthors=Connolly HM, Schaff HV, Abel MD, Rubin J, Askew JW, Li Z, Inda JJ, Luis SA, Nishimura RA, Pellikka PA |title=Early and Late Outcomes of Surgical Treatment in Carcinoid Heart Disease |journal=J. Am. Coll. Cardiol. |volume=66 |issue=20 |pages=2189–2196 |date=November 2015 |pmid=26564596 |doi=10.1016/j.jacc.2015.09.014 |url=}}</ref>


==References==
==References==

Latest revision as of 23:03, 6 May 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]

Overview

Surgery is the mainstay of treatment for carcinoid tumor. The feasibility of surgery depends on the stage of carcinoid tumor at diagnosis.

Surgery

Surgery, if feasible, is the only curative therapy. If the tumor has metastasized (most commonly, to the liver), the tumor may be ineligible for surgery (unresectable), though there are many promising treatment modalities, such as radiolabeled octreotide, that are very effective in arresting the growth of the tumors and prolonging survival in patients with liver metastases.

Gastric Carcinoids

Duodenal carcinoids

  • Duodenal carcinoids smaller than 2 cm may be excised locally. Tumors between 1 cm and 2 cm, complete resection is ensured by operative full-thickness excision. Follow-up endoscopy is indicated.

Jejunal and Ileal Carcinoids

Appendiceal Carcinoids

Colonic Carcinoids

Rectal Carcinoids

Metastatic Gastrointestinal Carcinoid Tumors

Definitive role of surgery in metastatic disease has not been established, conservative resections of the intestine mesenteric tumors, and fibrotic areas may improve symptoms and quality of life substantially in patients with metastatic hepatic, mesenteric, and peritoneal carcinoids.[1][8]

  • The primary tumor should be resected to prevent an emergency presentation with obstruction, perforation, or bleeding if the condition of the patient is such that surgery is not a greater risk than the disease
  • Management of hepatic metastases may include:
  1. Surgical resection
  2. Hepatic artery embolization
  3. Cryoablation
  4. Radiofrequency ablation
  5. Orthotopic liver transplantation.

Recurrent gastrointestinal Carcinoid Tumors

Treatment of recurrent gastrointestinal carcinoid tumor depends on many factors:[1]

  • Previous treatment
  • Site of recurrence
  • Individual patient considerations

Carcinoid heart disease

  • Valve replacement is the most effective treatment option for advanced carcinoid heart disease."Carcinoid Valve Disease | SpringerLink".[9]

References

  1. 1.0 1.1 1.2 1.3 1.4 Treatment Option Overview for gastrointestinal Carcinoid Tumors.<ref name="pmid12972355">Kulke MH (October 2003). "Neuroendocrine tumours: clinical presentation and management of localized disease". Cancer Treat. Rev. 29 (5): 363–70. PMID 12972355.
    • Type II carcinoids,the surgery is focused on removing the source of hypergastrinemia, typically by excision of duodenal gastrinomas via duodenotomy with resection of lymph node metastases.<ref name="pmid9298884">Bordi C, Falchetti A, Azzoni C, D'Adda T, Canavese G, Guariglia A, Santini D, Tomassetti P, Brandi ML (September 1997). "Aggressive forms of gastric neuroendocrine tumors in multiple endocrine neoplasia type I". Am. J. Surg. Pathol. 21 (9): 1075–82. PMID 9298884.
  2. Callahan AF, White M, Ituarte P, Gagandeep S, Woo Y, Fong Y, Melstrom L (September 2018). "Surgical Intervention in Gastric Carcinoid is Associated With Improved Survival in Local and Regional Disease". Am. J. Clin. Oncol. 41 (9): 882–887. doi:10.1097/COC.0000000000000392. PMID 28763328.
  3. Dias AR, Azevedo BC, Alban L, Yagi OK, Ramos M, Jacob CE, Barchi LC, Cecconello I, Ribeiro U, Zilberstein B (2017). "GASTRIC NEUROENDOCRINE TUMOR: REVIEW AND UPDATE". Arq Bras Cir Dig. 30 (2): 150–154. doi:10.1590/0102-6720201700020016. PMC 5543797. PMID 29257854. Vancouver style error: initials (help)
  4. Mullen JT, Wang H, Yao JC, Lee JH, Perrier ND, Pisters PW, Lee JE, Evans DB (December 2005). "Carcinoid tumors of the duodenum". Surgery. 138 (6): 971–7, discussion 977–8. doi:10.1016/j.surg.2005.09.016. PMID 16360380.
  5. 5.0 5.1 Zyromski NJ, Kendrick ML, Nagorney DM, Grant CS, Donohue JH, Farnell MB, Thompson GB, Farley DR, Sarr MG (2001). "Duodenal carcinoid tumors: how aggressive should we be?". J. Gastrointest. Surg. 5 (6): 588–93. PMID 12086896.
  6. 6.0 6.1 Boudreaux JP, Klimstra DS, Hassan MM, Woltering EA, Jensen RT, Goldsmith SJ, Nutting C, Bushnell DL, Caplin ME, Yao JC (August 2010). "The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the Jejunum, Ileum, Appendix, and Cecum". Pancreas. 39 (6): 753–66. doi:10.1097/MPA.0b013e3181ebb2a5. PMID 20664473.
  7. Mani S, Modlin IM, Ballantyne G, Ahlman H, West B (August 1994). "Carcinoids of the rectum". J. Am. Coll. Surg. 179 (2): 231–48. PMID 8044398.
  8. Modlin, Irvin M.; Lye, Kevin D.; Kidd, Mark (2003). "A 5-decade analysis of 13,715 carcinoid tumors". Cancer. 97 (4): 934–959. doi:10.1002/cncr.11105. ISSN 0008-543X.
  9. Connolly HM, Schaff HV, Abel MD, Rubin J, Askew JW, Li Z, Inda JJ, Luis SA, Nishimura RA, Pellikka PA (November 2015). "Early and Late Outcomes of Surgical Treatment in Carcinoid Heart Disease". J. Am. Coll. Cardiol. 66 (20): 2189–2196. doi:10.1016/j.jacc.2015.09.014. PMID 26564596.

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