scholarly journals Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia With Progression to Neuroendocrine Tumor

Cureus ◽  
2021 ◽  
Author(s):  
Débora Sousa ◽  
Filipa Rocha ◽  
Bernardo Baptista ◽  
Alexandra Bayão Horta
2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Adam Purdy ◽  
Firas Ido ◽  
Deborah Stahlnecker

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an atypical pulmonary disorder with limited understanding. Given the rare nature of this disease, it is essential to obtain adequate tissue pathology to confirm the diagnosis. This disease is mainly diagnosed in middle-aged, nonsmoking females, and it is now accepted as a precursor lesion to pulmonary carcinoid tumors. DIPNECH presents with characteristic radiographic and histologic findings, but its diagnosis, management, and prognosis are often underrecognized and poorly understood. Those with symptoms may present with shortness of breath, wheezing, and persistent cough and are often misdiagnosed with reactive airway disease. Pulmonary function testing may reveal airflow obstruction and air trapping. Imaging is characterized by multiple lung nodules, typically less than 5 mm in size, with a background mosaic attenuation on computed tomography imaging. Histologically, DIPNECH can be suspected based on the presence of hyperplastic neuroendocrine cells. DIPNECH is considered a precursor to invasive neuroendocrine tumor, and up to 50% of patients may have a well-differentiated neuroendocrine tumor at the time of presentation. Here, we present the case of a 46-year-old female with a history of ulcerative colitis on mesalamine who presented with a 6-month history of ongoing shortness of breath, chest tightness, wheezing, and cough. She was initially diagnosed with asthma before imaging later revealed as multiple pulmonary nodules with a diffuse mosaic pattern. Using robotic-assisted navigational bronchoscopy, she underwent sampling of a dominant 1.8 cm right middle lobe pulmonary nodule and pathology was consistent with low-grade neuroendocrine tumor.


Pneumologie ◽  
2012 ◽  
Vol 66 (S 01) ◽  
Author(s):  
V Kösek ◽  
J Schmidt ◽  
B Redwan ◽  
A Rukosujew ◽  
KM Müller ◽  
...  

2016 ◽  
Vol 47 (6) ◽  
pp. 1829-1841 ◽  
Author(s):  
Giulio Rossi ◽  
Alberto Cavazza ◽  
Paolo Spagnolo ◽  
Nicola Sverzellati ◽  
Lucia Longo ◽  
...  

The term diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) may be used to describe a clinico-pathological syndrome, as well as an incidental finding on histological examination, although there are obvious differences between these two scenarios. According to the World Health Organization, the definition of DIPNECH is purely histological. However, DIPNECH encompasses symptomatic patients with airway disease, as well as asymptomatic patients with neuroendocrine cell hyperplasia associated with multiple tumourlets/carcinoid tumours. DIPNECH is also considered a pre-neoplastic lesion in the spectrum of pulmonary neuroendocrine tumours, because it is commonly found in patients with peripheral carcinoid tumours.In this review, we summarise clinical, physiological, radiological and histological features of DIPNECH and critically discuss recently proposed diagnostic criteria. In addition, we propose that the term “DIPNECH syndrome” be used to indicate a sufficiently distinct patient subgroup characterised by respiratory symptoms, airflow obstruction, mosaic attenuation with air trapping on chest imaging and constrictive obliterative bronchiolitis, often with nodular proliferation of neuroendocrine cells with/without tumourlets/carcinoid tumours on histology. Surgical lung biopsy is the diagnostic gold standard. However, in the appropriate clinical and radiological setting, transbronchial lung biopsy may also allow a confident diagnosis of DIPNECH syndrome.


2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Daniel R. Almquist ◽  
Vinicius Ernani ◽  
Mohamad Bassam Sonbol

2010 ◽  
Vol 5 (6) ◽  
pp. 921-923 ◽  
Author(s):  
Sheeba Irshad ◽  
Emma McLean ◽  
Sheila Rankin ◽  
Sally Barrington ◽  
George Santis ◽  
...  

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