Intestinal Carcinoid Tumor Treatment & Management

  • Author: Hemant Singhal, MD, MBBS, FRCSE, FRCS(C); Chief Editor: Jules E Harris, MD   more...
 
Updated: Jan 27, 2012
 

Medical Care

Medical care is usually only for symptomatic relief.

  • Chemotherapy
    • Response rates are variable but rarely exceed 30%. Results are usually short-lived (ie, < 1-y duration). 5-Fluorouracil and streptozocin (Zanosar)–based regimens are commonly used in patients with metastatic carcinoid tumors. The value of using newer agents (eg, taxanes, gemcitabine [Gemzar], irinotecan [Camptosar]) remains unproven.
    • Chemotherapeutic regimens are only for palliative purposes; if eligible, patients should be put on a clinical trial of investigational therapy.
  • Radiation: This has only a palliative role, particularly for painful bony metastasis.
  • Other modalities
    • High doses of sodium iodine-131–labeled metaiodobenzylguanidine, low-dose interferon alfa, and octreotide have all been used, with some reduction in symptoms; however, tumor reduction is rarely observed. When reductions occur, they are only transient.[10] The somatostatin analog octreotide may provide control of carcinoid symptoms if findings on OctreoScan are positive or if somatostatin receptors are found in tumor tissue.
    • A randomized, placebo-controlled phase 3 trial found that everolimus plus octreotide long-acting repeatable (LAR) improved progression-free survival in patients with advanced neuroendocrine tumors associated with intestinal carcinoid syndrome.[11]
    • Patients with problematic diarrhea usually find benefit from antidiarrheal medication. Cyproheptadine and histamine-2 receptor blockers may be of benefit because they suppress the production of vasoactive amines or block their peripheral effects.
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Surgical Care

Surgical resection is the standard therapeutic modality.

  • Appendiceal carcinoids
    • For tumors smaller than 1.5 cm in greatest diameter that are confined to mucosa, appendicectomy is adequate, with no need for follow-up care. Cure rates are 100%.
    • Tumors 2 cm or larger in diameter, those at the base of the appendix, or those with mesenteric lymphadenopathy are not common but are considered potentially malignant. Consider more aggressive surgery in the form of a right hemicolectomy and lymphadenectomy, similar to that performed for colonic adenocarcinoma.
    • Invasion of only the mesoappendix does not alter the long-term prognosis, but cecal involvement necessitates further surgery.
    • A study by Cashin et al investigated the use of cytoreductive surgery and intraperitoneal chemotherapy in patients with appendiceal adenocarcinoids. The study found that the completeness of cytoreduction score (CC) correlated with overall survival. Candidates for this treatment should be referred early in order to attain a better CC score. The study also confirmed that drugs used for colorectal caner also seemed adequate for appendicial adenocarcinoid, although other drugs may be more active.[12]
  • Small bowel carcinoids
    • At laparotomy, perform a thorough examination of the small bowel because multiple lesions are fairly common.
    • Macroscopic tumor size is a fairly good indictor of malignant potential. For tumors smaller than 1 cm in diameter, local resection is usually adequate. Tumors larger than 1.5 cm have a risk of recurrence, hence the need for a segmental bowel resection with lymphadenectomy.
  • Rectal carcinoids
    • Tumor size is of essence with regard to the extent of resection.
    • Tumors of up to 1 cm in diameter require only local excision or fulguration, with cure rates close to 100%.[13] Consider large tumors (≥2 cm) malignant, and manage them similarly to adenocarcinoma of the rectum, with extensive resection. Tumors of 1-2 cm can be treated either by limited or more aggressive resection, with each case is guided according to the size, invasive nature, and anatomic location.
    • All patients except for those with lesions smaller than 1 cm require conscientious follow-up care.
  • Metastatic intestinal carcinoid - Possible options
    • Surgery is considered worthwhile in most cases because this is the best form of palliation, regardless of whether the tumor is of the secretory type. Tailor the procedure accordingly, and avoid attempts at major debulking procedures.
    • All patients with advanced-stage carcinoid tumors should be evaluated for possible multimodal surgical therapy. Primary tumors should be resected, even in the presence of distant metastases to prevent future intestinal obstruction. The "wait and see" method of management of this slow-growing cancer no longer has merit.
    • Obstructive small bowel lesions could be resected (if possible) or bypassed.
    • Multiple liver metastases in patients with carcinoid syndrome are resected, cauterized, or ablated with percutaneous alcohol injections because this usually results in a dramatic relief of symptoms.
    • Hepatic artery ligation or embolization (eg, collagen fibers, gel foam, alcohol) can result in significant tumor necrosis and is of value in patients with bulky, inoperable, or symptomatic liver metastasis, with up to a 60% reduction of tumor bulk in some cases. This form of treatment can be combined with intrahepatic chemotherapeutic infusion. However, it can result in toxic effects, particularly fever, nausea, vomiting, and abdominal pain. Occasionally, the carcinoid symptoms may worsen.[14]

See related CME at An Aggressive Surgical Approach Leads to Long-term Survival in Patients With Pancreatic Endocrine Tumors.

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Contributor Information and Disclosures
Author

Hemant Singhal, MD, MBBS, FRCSE, FRCS(C)  Senior Lecturer, Director of Breast Service, Department of Surgery, Imperial College School of Medicine; Consultant Surgeon, Northwick Park and St Marks Hospitals, UK

Hemant Singhal, MD, MBBS, FRCSE, FRCS(C) is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada and Royal College of Surgeons of Edinburgh

Disclosure: Nothing to disclose.

Coauthor(s)

Kanchan Kaur, MBBS, MS (General Surgery), MRCS (Ed)  Consulting Breast and Oncoplastic Surgeon, Medanta, The Medicity, India

Disclosure: Nothing to disclose.

Alan A Saber, MD, MS, FACS, FASMBS  Associate Professor of Surgery, Case Western Reserve University School of Medicine; Director of Metabolic Surgery, Case Medical Center

Alan A Saber, MD, MS, FACS, FASMBS is a member of the following medical societies: American College of Surgeons, American Society for Gastrointestinal Endoscopy, and American Society for Metabolic and Bariatric Surgery

Disclosure: Nothing to disclose.

Charles Zammit, MD  Senior Specialist Registrar, Department of Surgery, Breast Unit Charing Cross Hospital of London, UK

Disclosure: Nothing to disclose.

Michael K McLeod, MD, FACE, FACS  Professor of Surgery and Program Director, Integrated General Surgery Program, Department of Surgery, Michigan State University College of Human Medicine

Michael K McLeod, MD, FACE, FACS is a member of the following medical societies: American Association of Clinical Endocrinologists, American Association of Endocrine Surgeons, American College of Surgeons, American Medical Association, Association for Academic Surgery, Central Surgical Association, International Association of Endocrine Surgeons, Michigan State Medical Society, Midwest Surgical Association, National Medical Association, Society of American Gastrointestinal and Endoscopic Surgeons, and Western Surgical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Lodovico Balducci, MD  Professor of Oncology and Medicine, University of South Florida College of Medicine; Division Chief, Senior Adult Oncology Program, H Lee Moffitt Cancer Center and Research Institute

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Benjamin Movsas, MD  Vice-Chairman, Department of Radiation Oncology, Fox Chase Cancer Center

Benjamin Movsas, MD is a member of the following medical societies: American College of Radiology, American Radium Society, and American Society for Therapeutic Radiology and Oncology

Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Jules E Harris, MD  Clinical Professor of Medicine, Section of Hematology/Oncology, University of Arizona College of Medicine, Arizona Cancer Center

Jules E Harris, MD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Immunologists, American Society of Hematology, and Central Society for Clinical Research

Disclosure: GlobeImmune Salary Consulting

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Distribution of carcinoid tumors.
Frequency of symptoms in carcinoid syndrome.
Table 1. 5-Year Survival Rates by Stage and Primary Site Between 1973 and 1999
Site Localized,



%



Regional,



%



Distant,



%



Stomach683510
Small intestine576740
Appendix918128
Colon745125
Rectum874125
Table 2. Presentation of Intestinal Carcinoids
LocationNonhormonal SymptomsCarcinoid Syndrome,



%



Metastatic Disease,



%



StomachPain



Pernicious anemia



< 105-10
Small intestinePain



Intestinal obstruction



Up to 905-7
AppendixAppendicitis



Incidental finding



< 5< 5
ColonPain



Bleeding



Weight loss



>66< 5
RectumPain



Constipation



Bleeding



5 (< 1 cm tumors)



>90



< 5
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