Pulmonary neuroendocrine tumor
ICD-10 C34.9 · ICD-11 2C25.4

Treatment of advanced or metastatic atypical lung carcinoid, including significantly progressive and post-somatostatin analogue disease

This protocol addresses the management of pulmonary neuroendocrine tumors in patients presenting with advanced or metastatic atypical lung carcinoid — including high-grade cases — as well as those with significantly progressive disease or carcinoid that has progressed following somatostatin analogue therapy.

Clinical scenario

Advanced or metastatic atypical lung carcinoid (including high-grade), significantly progressive carcinoid, or disease that has progressed after somatostatin analogue therapy. These situations call for a structured, evidence-based treatment approach.

Treatment approach

For this scenario, targeted therapy plays a central role as a first-line option in atypical carcinoid, with cytotoxic chemotherapy representing an alternative strategy in select patients.

Complete regimen details, sequencing, and eligibility criteria are in the full protocol.

References

DOI: 10.1016/j.annonc.2021.01.003

Everolimus is considered as first-line therapy in the majority of ACs or following progression to SSA for both TC and AC patients [II, B].

Everolimus is recommended either as first line in case of AC or, second-line post-SSA, in patients with TC and or progressive advanced LCs and ThCs [II, B].

Dacarbazine/temozolomide-based ChT as first line, and platinum-based ChT as second line, are recommended in advanced LC patients refractory or intolerant to everolimus therapy [IV, C].

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