Endocrine Abstracts

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH), which was formally recognised in 1992, has been described as an idiopathic pre-invasive condition that may co-exist with carcinoid tumours. Classically it has been described in women with predominantly obstructive lung function and not in association with smoking. Although many have stable disease, in some documented cases there has been progressive lung disease leading to the need for lung transplant. There are a few case reports, with the largest including a systematic review identifying 24 cases. We present three further cases of DIPNECH which have been seen by the respiratory and neuroendocrine team, compare these to the previously documented cases and discuss some of the difficulties in diagnosing and managing these patients. Case 1 is a male patient who was noted to have focal vascular invasion and also lymph node involvement with his DIPNECH, this highlights the classification as a pre-invasive lesion. Case 2 is a female who underwent two lung resections for carcinoid nodules on the background of DIPNECH to exclude malignancy. In this case there may have been scope to avoid the second procedure in light of the initial findings. Case 3 is a further female case who was known to have Cushing’s disease, after presenting with shortness of breath she had chest imaging which confirmed multiple nodules which were noted on biopsy to be carcinoid tumours. In this case steroid treatment would not have been appropriate to manage her breathlessness and she was instead managed with somatostatin analogues after seeing the neuroendocrine team.