From the Guidelines
Systemic treatment for metastatic pulmonary carcinoid tumors should begin with somatostatin analogs (SSAs) as the first-line treatment, according to the most recent guidelines. This approach is supported by the 2021 ESMO clinical practice guidelines for diagnosis, treatment, and follow-up of lung and thymic carcinoids 1. The guidelines recommend SSAs as the first-line treatment in patients with typical carcinoids (TC) and/or slowly progressing advanced somatostatin receptor-positive lung carcinoids (LC) [IV, C].
Treatment Options
- SSAs, such as octreotide LAR or lanreotide, are effective for controlling symptoms of carcinoid syndrome and stabilizing tumor growth in patients with somatostatin receptor-positive disease.
- For progressive disease despite SSAs, targeted therapies like everolimus may be used, which inhibits the mTOR pathway to slow tumor growth 1.
- Peptide receptor radionuclide therapy (PRRT) with lutetium-177-DOTATATE is another effective option for patients with somatostatin receptor-positive tumors.
- Traditional chemotherapy with temozolomide or capecitabine plus temozolomide may be considered for aggressive, high-grade tumors or those with rapid progression.
- Interferon-alpha can be added to SSAs for additional tumor control.
Treatment Selection
Treatment selection should be guided by tumor characteristics, including grade, proliferation rate, somatostatin receptor status, and the patient's symptom burden, with regular imaging every 3-6 months to assess response and adjust therapy accordingly 1. The guidelines also recommend considering clinical trials, as there is limited evidence of efficacy for all treatment options 1.
High-Grade Tumors
For high-grade atypical carcinoids (ACs) or significantly progressive carcinoids post-SSA therapy, everolimus is recommended as either first-line or second-line treatment 1. Temozolomide-based chemotherapy or platinum-based chemotherapy may also be considered in advanced LC patients refractory or intolerant to everolimus therapy 1.
PRRT and IFN-a
PRRT should be considered as an alternative to chemotherapy in selected patients with refractory carcinoid syndrome and progressing or bulky tumors, based on positive uptake at SRI on all RECIST-evaluable targets 1. IFN-a should be considered as an alternative to chemotherapy, especially in patients with refractory carcinoid syndrome 1.
From the FDA Drug Label
Carcinoid Tumors Octreotide acetate injection is indicated for the symptomatic treatment of patients with metastatic carcinoid tumors where it suppresses or inhibits the severe diarrhea and flushing episodes associated with the disease.
The systemic treatment option for metastatic pulmonary carcinoid (lung) tumors is octreotide acetate injection, which is used for the symptomatic treatment of patients with metastatic carcinoid tumors to suppress or inhibit severe diarrhea and flushing episodes associated with the disease 2.
- Key benefits: suppression or inhibition of severe diarrhea and flushing episodes
- Limitations: does not show an effect on the size, rate of growth, or development of metastases
From the Research
Systemic Treatment Options for Metastatic Pulmonary Carcinoid Tumors
- The treatment options for metastatic pulmonary carcinoid tumors include somatostatin analogs (SSAs), chemotherapy, targeted therapy, and peptide receptor radionuclide therapy 3.
- SSAs, such as octreotide and lanreotide, have shown antitumor activity and are considered a standard of care for patients with metastatic neuroendocrine tumors, including pulmonary carcinoids 4, 5.
- Chemotherapy regimens, including gemcitabine-oxaliplatin and 5-fluorouracil-oxaliplatin, have also been evaluated in patients with metastatic pulmonary carcinoid tumors, with reported response rates and progression-free survival times 6.
- Targeted therapies, such as everolimus, have been approved for use in patients with pulmonary carcinoids, although the evidence is limited and prospective trials are needed to determine their efficacy 3.
- Peptide receptor radionuclide therapy may also be used in patients with metastatic pulmonary carcinoid tumors, with reported benefits in terms of survival and symptom reduction 3.
Efficacy of Somatostatin Analogs
- Studies have reported disease control rates of 86.6% to 90.3% with SSA treatment, with median progression-free survival times ranging from 11.1 to 28.6 months 4, 5.
- SSA treatment has been associated with improved overall survival, although the evidence is limited and further studies are needed to confirm this finding 5.
Chemotherapy Regimens
- The gemcitabine-oxaliplatin and 5-fluorouracil-oxaliplatin regimens have been evaluated in patients with metastatic pulmonary carcinoid tumors, with reported response rates of 20% and median progression-free survival times of 15 months 6.
- These regimens may be considered as options for patients with progressive disease, although further studies are needed to determine their efficacy and optimal sequencing 6.
Prognostic Factors
- The prognostic value of various factors, including histologic subtype, Ki-67 index, and 18FDG-PET/CT status, has been evaluated in patients with metastatic pulmonary carcinoid tumors 4, 5.
- These factors may be useful in predicting treatment response and outcomes, although further studies are needed to confirm their prognostic value 4, 5.