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Is there a role of everolimus or capecitabine and temozolomide in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia in patients already on octreotide?

1 Answer
Mednet MemberInvited Expert
Medical Oncology · Mayo Clinic
Answered on · Updated on

The key question is why there is a reason to change the treatment... Is it for worsening symptoms or progressive disease with overt lung NET development or progression of known lung NET?

In a retrospective study of 55 patients with DIPNECH, only 18% had a progression to a lung NET (typical carcinoid). 49% of patients had either increasing number of nodules, increasing size of tumorlets, or both but not meeting the criteria for progression to a lung NET.

In patients with symptomatic DIPNECH (common symptoms being cough, wheezing, and other obstructive symptoms), somatostatin analogues (SSAs) may help with symptoms and symptoms and radiographic appearance may change over time, but it is not known how they affect the evolution of DIPNECH to a lung NET. 

For someone with stubborn symptoms but no obvious radiographic progression, symptomatic therapy directed by an experienced pulmonologist is recommended and may include SSAs, inhaled steroids, and others.

For radiographic progression, one needs to know if these are minor changes, such as new small nodules or changing CT pattern in which case, I would not recommend chemotherapy with CAPTEM or everolimus. In cases of clear radiographic progression to a lung NET, resection and/or radiotherapy may be considered, and only in the rare occasion of substantial radiographic progression would I consider CAPTEM, everolimus, and PRRT.

Needless to say, the evidence for all interventions for DIPNECH is very limited, the quality of evidence is low and there are no reported prospective trials I am aware of.