Carcinoid Tumor Treatment & Management

Updated: Feb 12, 2019
  • Author: Cameron K Tebbi, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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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

    • Experimental agents such as 177Lu-Dotatate

  • 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. [83]

  • 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). [84]

    • 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. [85]

    • 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. [86] 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. [87, 88]

  • 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. [89] The disease was stable 56 months posttherapy.


Surgical Care

If feasible, the treatment of choice is surgical excision which is associated with an excellent prognosis, especially in appendiceal tumors. [33, 90, 2, 3, 4, 45, 46, 47, 48] Surgical technique may vary according to the type or location of the tumor.

  • For pediatric age group, surgical treatment of appendiceal carcinoids can be challenging. In most appendiceal tumors, simple appendectomy is sufficient for treatment and are universally accepted. [91, 90, 2, 3, 4, 45, 46, 47, 48]  Choosing optimal treatment when lesions are greater than two centimeters, appendix is perforated,  or there is involvement in the appendiceal base are challenging and can be controversial. [47, 48, 49] 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. [44, 43]

  • In localized tumors, surgical resection can result in cure, with 70% to more than 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. [92] In adults, surgical resection of the primary tumor is shown to provide survival advantage. [93]

  • 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. [94]

  • 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. [95] 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. [96]