Carcinoid Tumor Treatment & Management

  • Author: Cameron K Tebbi, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Dec 20, 2011
 

Medical Care

If metastasis of carcinoid tumor has occurred and in cases where surgical excision is not suitable, consider treatment with currently recommended chemotherapy.

  • Chemotherapeutic agents currently used in clinical trials to palliate metastatic carcinoid disease include the following:
    • Alkylating agents
    • Doxorubicin
    • 5-Fluorouracil
    • Dacarbazine
    • Actinomycin D
    • Cisplatin
    • Etoposide
    • Streptozotocin
    • Interferon alfa
    • Somatostatin analogs with a radioactive load
  • A combination of the agents listed above is typically used.
  • Chemoembolization of hepatic artery for treatment of metastatic carcinoid tumor has been widely used in adults.[52]
  • In one study, 8 adults with carcinoid tumor metastatic to liver were treated with intra-arterial 5-fluorouracil and embolization of hepatic tumors with bovine collagen fiber admixed with iohexol, cisplatin, mitomycin C, and doxorubicin. This treatment resulted in symptomatic relief and tumor regression in 4 patients and stabilized the disease in the rest of the patients.
  • Octreotide, a somatostatin analog, is highly effective in reducing symptoms; however, in the pediatric age group, stunted linear growth is of concern. Survival advantage with the use of this drug has not yet been proven.
    • Octreotide reduces the amount of the growth factor produced and, thus, theoretically impairs growth.
    • Intermittent and continuous infusions of octreotide have been reported. Superior results obtained with the latter modality. Such treatment can result in near-normalization of the plasma insulinlike growth factor I and partial suppression of plasma growth hormone–releasing hormone (GHRH).[53]
    • The availability of a long-acting somatostatin analog that can be given once a month has eliminated the need for injections 2-3 times per day, with equal efficacy.[54]
    • In metastatic carcinoid tumors, long-term use of octreotide is reported. However, receptor alteration induced during the use of this agent requires consecutive drug dosage increase to control the symptoms.[55] At present, no formal, well-designed study has been performed to systematically measure the effects of this modality of therapy. Although experience is limited, adverse effects in children have been similar to those in adults. Adverse effects include gallstones and steatorrhea, which may sometimes require pancreatic enzyme replacement. Local irritation at the injection site is a common complaint. These adverse effects must be weighed against the potential benefits.
    • A randomized, double-blind, placebo-controlled, phase 3 study showed that using everolimus in conjunction with octreotide improved progression-free survival in patients with low-grade and intermediate-grade advanced neuroendocrine tumors.[56]
  • In situ targeted therapy with somatostatin analogs (eg, octreotide attached to a radioactive load using yttrium-90 or111 indium-labeling agents) provides promise for patients with unresectable tumors. This therapy is currently used on an experimental basis in adults and children.
  • A case of a 16-year-old female with recurrent disseminated, ganglioside positive bronchial carcinoid, successfully treated with recombinant alpha interferon with a third relapse treated with NGcGM3/VSSP Montanide ISA 51 vaccination in conjunction with rec-h lFNalpha is available.[57] The disease was stable 56 months posttherapy.
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Surgical Care

If feasible, the treatment of choice is surgical excision. Surgical technique may vary according to the type or location of the tumor.

  • In most appendiceal tumors, simple appendectomy is sufficient for treatment. In intestinal carcinoids, block resection of the tumor with adjacent lymph nodes must be attempted. In the bronchial location, aggressive surgical resection, and not bronchoscopic removal, is recommended.[20, 19]
  • In localized tumors, surgical resection can result in cure, with 70-90% survival rate.
  • In rectal tumors, endoscopic resection in adults is sufficient for small tumors measuring less than 1 cm, for tumors limited to the mucosa, and in tumors resected with adequate margin at presentation. Nevertheless, resection does not guarantee prevention of metastasis at a later date.[58] In adults, surgical resection of the primary tumor is shown to provide survival advantage.[59]
  • When total resection is not possible, debulking may provide symptomatic relief.
  • For hepatic tumors, surgical ligation of the hepatic artery can potentially deprive blood supply to the tumor cells and cause necrosis while preserving most of the normal live cells. However, over time new blood vessels develop and restore circulation.
  • Intra-arterial infusion of chemotherapeutic agents with chemoembolization of the hepatic artery may also provide effective, albeit short term, relief of symptoms due to hepatic metastasis. If hepatic metastasis is present but resectable, surgical resection is preferred.
  • In selected cases, cryotherapy can be effective. Bronchoscopic cryotherapy has been successfully applied in treatment of isolated endoluminal carcinoid tumor in an adult patient.[60]
  • In patients with tumors less than 1 cm located in the appendix, appendectomy is the treatment of choice. More extensive surgery is indicated for tumors larger than 2 cm, lymphatic invasion, lymph node involvement, mesoappendix infiltration, positive resection margins, and cellular pleomorphism with a high mitotic index. For tumors larger than 2 cm, accepted treatment has been hemicolectomy; however, a survival advantage over simple appendectomy has not been demonstrated.[61] Given the relatively low malignant potential of appendiceal carcinoids, some have suggested simple appendectomy for tumors more than 2 cm diameter without affecting overall survival.[62]
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Contributor Information and Disclosures
Author

Cameron K Tebbi, MD  Professor of Pediatrics, Chief, Division of Pediatric Hematology-Oncology, University of South Florida College of Medicine; Director, Children's Medical Services, Pediatric Hematology/Oncology, Tampa Division, State of Florida Department of Health

Cameron K Tebbi, MD is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, Histiocyte Society, and International Society of Paediatric Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Kathleen M Sakamoto, MD, PhD  Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Co-Associate Program Director of the Signal Transduction Program Area, Jonsson Comprehensive Cancer Center, California Nanosystems Institute and Molecular Biology Institute, University of California, Los Angeles, David Geffen School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Cincinnati Children's Hospital Medical Center; Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, University of Cincinnati College of Medicine

Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

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Distribution of carcinoid tumors.
 
 
 
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