Table 2. National Comprehensive Cancer Network Guidelines for the Management of Symptomatic Patients With NET of the Gastrointestinal Tract.
| Disease stage | Management | Guideline Recommendations |
|---|---|---|
| First line | Resection | • Resection of primary tumor should be considered in locally symptomatic patients |
| • Ablative techniques (radiofrequency, microwave, and cryotherapy) are options for metastases; however, prospective data for these interventions are limited | ||
| SSAs (octreotide or lanreotide) | • Recommended for first-line control of tumor growth | |
| • Octreotide LAR is appropriate for long-term management of patients with carcinoid syndrome | ||
| • Short-acting octreotide can be added to octreotide LAR for rapid relief of symptoms or breakthrough symptoms | ||
| • SSA would better benefit patients who have positive somatostatin receptor expression on imaging (octreoscan/Ga-68 dotatate PET) | ||
| Progressive disease | SSAs (octreotide or lanreotide) | • Treatment should be continued in patients with functional tumors and can be added to other treatment options |
| • May be used in patients with low-grade tumors as first-line therapy to stabilize disease | ||
| Everolimus | • Option for patients with disease progression; however, the safety and effectiveness of everolimus has not been established in patients with carcinoid syndrome | |
| 177Lu-DOTATATE | • Recommended for patients with somatostatin receptor–positive imaging | |
| Hepatic-directed therapy | • Appropriate for hepatic-predominant disease/hepatic progression | |
| • Options include arterial embolization, hepatic chemoembolization, hepatic radioembolization, or cytoreductive surgery/ablative therapy | ||
| INFα | • Usually only initiated after failure of SSA treatment; out of favor now | |
| Cytotoxic chemotherapy | • Should only be considered as a last treatment option | |
| • There are no data to support a specific sequence of regional vs. systemic therapy and no data to guide sequencing of systemic options | ||
| Refractory disease | Telotristat ethyl + SSAs | • Recommended for persistent diarrhea that is not controlled by SSAs alone |
| Hepatic arterial embolization ± cytoreductive surgery | • Appropriate for hepatic predominant disease | |
| Other systemic therapy based on disease site | • Cytotoxic chemotherapy can be used in patients with progressive disease and no other treatment options |
Note. LAR = long-acting release; PET = positron emission tomography; Lu = lutetium; SSA = somatostatin analog; INFα = interferon alpha. Information from NCCN (2018).