Early-stage (cT1N0) pulmonary neuroendocrine tumors arising in specific clinical settings — including diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH), multiple endocrine neoplasia type 1 (MEN-1) syndrome, or in patients with significant comorbid conditions — present a distinct management challenge where standard anatomic resection may not be the first or only option.
This protocol applies to patients with a cT1N0 lung carcinoid in the context of DIPNECH, in MEN-1 syndrome, or where comorbid conditions alter the risk-benefit balance of surgery. The characteristically indolent course of tumors in these subgroups supports a more conservative initial strategy, with escalation guided by clinical or morphological change.
Depending on the clinical picture, the approach may begin with watchful radiological follow-up. Should progression occur, options involving limited surgical or nonsurgical locoregional interventions — chosen with lung function preservation as a central goal — are among the alternatives to standard anatomic resection.
The complete evidence-based regimen, including specific criteria for escalation, intervention selection, and sequencing, is available in the full structured protocol.
DOI: 10.1016/j.annonc.2021.01.003
Watchful radiological follow-up, as an alternative to surgery, may be considered in case of cT1N0 carcinoid within the setting of DIPNECH, but also in MEN-1 patients and patients with comorbid conditions, due to the indolent course of most tumours [IV, C].
In these subgroups of patients, watchful follow-up or, in case of clinical (functioning syndrome) and or morphological progression, sublobar surgical resections including wedge resection, but also nonsurgical locoregional therapeutic procedures or medical treatments (SSAs) to preserve lung function constitute alternatives to anatomic pulmonary resection [V, C].
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