Pulmonary Function Tests as a Biomarker in Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Patients Treated With Somatostatin Analogues.

Abstract

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) describes an indolent, under-recognised and poorly understood pulmonary condition with fewer than 200 reported cases across the literature. Currently, cases are diagnosed following a biopsy of the primary lesion, with treatment options centring on symptomatic benefit as opposed to targeting the underlying aetiology. Classically, DIPNECH lesions have been described as slow growing and benign, but with growing awareness of the condition, reports of metastatic disease with significant symptomatic burden have been reported. However, effectively addressing the subset of DIPNECH patients with greater metastatic potential remains an unmet clinical need. Due to the similarities between DIPNECH and carcinoid patients, several centres have considered using somatostatin analogues to not only help symptomatically but also to initiate tumour regression. However, to date, there are limited biomarkers to help evaluate the benefit of such options. In this review, we consider the use of pulmonary function tests (PFTs) to help quantify the benefit of somatostatin analogues. Although much of the evidence stems from small single-centre studies, the use of PFTs within the treatment pathway for both localised and metastatic DIPNECH represents a meaningful improvement from subjective monitoring of disease.