Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with pulmonary carcinoid tumor

Case contributed by Dr Akos Jaray

Presentation

Non-smoker. On immunosuppressant therapy. Follow-up.

Patient Data

Age: 55 years
Gender: Female

There are numerous, well-delineated intrapulmonary nodules and micronodules present throughout both lungs. 

Diffuse ground glass opacities are noted with lower lobe predominance.

A soft tissue mass of 2.5 cm is detected at the left hilum abutting the main pulmonary artery. 

Restaging CT study following radiotherapy shows focal perihilar radiation fibrosis with bronchiectasis. The diffuse ground-glass mosaic attenuation became even more evident.

FDG-PET CT denotes a focus of activity at the left perihilar soft tissue adjacent to left main pulmonary artery, compatible with malignancy. The multiple bilateral pulmonary nodules demonstrate no or low avidity. 

Case Discussion

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare lung condition characterized by the abnormal overgrowth of pulmonary neuroendocrine cells, of which this patient had histological proof. Usually, aspecific symptoms occur such as a chronic, non-productive cough, shortness of breath with exertion and wheezing.

It is considered to be a precancerous condition for pulmonary carcinoid tumors (well-differentiated neuroendocrine tumors). FDG PET CT can be helpful distinguishing between benign nodules and the ones showing malignant transformation. 

The presence of scattered multiple, rounded and ovoid pulmonary nodules and micronodules with mosaic pattern ground-glass opacities might raise the suspicion of DIPNECH.

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Case information

rID: 72403
Published: 23rd Nov 2019
Last edited: 20th May 2020
System: Oncology, Chest
Inclusion in quiz mode: Included
Institution: University of Pécs