TT OF CARCINOID TUMOR : If metastasis occurs and surgical excision is not suitable, consider treatment with currently recommended adjunctive 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.
In 1 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 in tumoral regression in 4 patients, and it 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, stunt linear growth is of concern.
Octreotide reduces the amount of the growth factor produced and thus theoretically impairs growth.
Intermittent and continuous infusion of octreotide administration are reported, with superior results obtained with the latter.
Such treatment can result in near-normalization of the plasma insulin-like growth factor I and partial suppression of plasma growth hormone-releasing hormone (GHRH) (Lefebvre, 1995).
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 injections, with equal efficacy (Rubin, 1999).
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 with the disease. 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 weighted against the potential benefits.
In situ targeted therapy with somatostatin analogs (eg, octreotide attached to a radioactive load using yttrium-90 or 111In-labeling agents) can provide promising therapy for patients with unresectable tumors.
This therapy is currently used on an experimental basis in adults and children.
Surgical Care: The treatment of choice is surgical excision, if feasible. The 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 (Fink, 2001; Fauroux, 2005).
In localized tumors, surgical resection can result in a cure with 70-90% survival rate.
When total resection is not possible, debulking may provide symptomatic relief.
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, new blood vessels develop over time 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 in cases of carcinoid tumors.
If hepatic metastasis is present but resectable, surgical resection is preferred.