scholarly journals Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): A Case of Indolent Pulmonary Nodules Diagnosed with Robotic-Assisted Navigational Bronchoscopy

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Adam Purdy ◽  
Firas Ido ◽  
Deborah Stahlnecker
Keyword(s):  
Neuroendocrine Tumor ◽  
Pulmonary Nodules ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Cell ◽  
Low Grade ◽  
Shortness Of Breath ◽  
Cell Hyperplasia ◽  
Robotic Assisted ◽  
History Of ◽  
Pulmonary Neuroendocrine Cell

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.

scholarly journals A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia

2015 ◽  
Vol 2015 ◽  
pp. 1-5
Author(s):  
Godwin Ofikwu ◽  
Vishnu R. Mani ◽  
Ajai Rajabalan ◽  
Albert Adu ◽  
Leaque Ahmed ◽  
...  
Keyword(s):  
Thoracoscopic Surgery ◽  
Left Kidney ◽  
Pulmonary Nodules ◽  
Radical Mastectomy ◽  
Left Breast ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Cell ◽  
Cell Hyperplasia ◽  
Cortical Nodules ◽  
Pulmonary Neuroendocrine Cell

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare clinical condition with only about 100 cases reported in the literature. It is characterized by primary hyperplasia of pulmonary neuroendocrine cells (PNECs) which are specialized epithelial cells located throughout the entire respiratory tract, from the trachea to the terminal airways. DIPNECH appears in various forms that include diffuse proliferation of scattered neuroendocrine cells, small nodules, or a linear proliferation. It is usually seen in middle-aged, nonsmoking women with symptoms of cough, dyspnea, and wheezing. We present a 45-year-old, nonsmoking woman who presented with symptoms of DIPNECH associated with bilateral pulmonary nodules and left hilar adenopathy. Of interest, DIPNECH in our patient was associated with metastatic pulmonary carcinoids, papillary carcinoma of the left breast, oncocytoma and angiomyolipoma of her left kidney, and cortical nodules suggestive of tuberous sclerosis. She had video assisted thoracoscopic surgery (VATS), modified radical mastectomy with reconstruction, and radical nephrectomy. She is currently symptom-free most of the time with over two years of follow-up.

scholarly journals Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome

2016 ◽  
Vol 47 (6) ◽  
pp. 1829-1841 ◽  
Author(s):  
Giulio Rossi ◽  
Alberto Cavazza ◽  
Paolo Spagnolo ◽  
Nicola Sverzellati ◽  
Lucia Longo ◽  
...  
Keyword(s):  
Lung Biopsy ◽  
Airflow Obstruction ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Cell ◽  
World Health ◽  
Cell Hyperplasia ◽  
Carcinoid Tumours ◽  
Neoplastic Lesion ◽  
Asymptomatic Patients ◽  
Pulmonary Neuroendocrine Cell

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.

scholarly journals Pulmonary Peripheral Carcinoids after Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Tumorlets: Report of 3 Cases

2015 ◽  
Vol 2015 ◽  
pp. 1-7 ◽  
Author(s):  
Carlos Abrantes ◽  
Rui Caetano Oliveira ◽  
Joana Saraiva ◽  
João Bernardo ◽  
Lina Carvalho
Keyword(s):  
Lung Disease ◽  
Neuroendocrine Differentiation ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Cell ◽  
Peripheral Spectrum ◽  
Surgical Biopsy ◽  
Cell Hyperplasia ◽  
Ki 67 ◽  
Typical Carcinoid ◽  
Pulmonary Neuroendocrine Cell

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) and tumorlets are neuroendocrine cells proliferations smaller than 5 mm. The former confines to bronchial/bronchiolar wall, while the latter broke through epithelial basement membrane. The authors present 3 cases of DIPNECH and tumorlets associated with a typical peripheral carcinoid tumor without underlying lung disease. The patients presented with nonspecific pulmonary symptoms: 3 females, 60, 72, and 84 years old, whose CT-scans showed well-defined pulmonary nodules, 2.2, 1.6, and 1.4 cm, respectively; first patient was submitted to lobectomy and the others underwent surgical biopsy. Whitish/brownish lobulated tumors corresponded to typical carcinoids (less than 2 mitoses/2 mm2and without necrosis); polygonal/elongated cells under lobular pattern expressed CD56, chromogranin A, synaptophysin, and CK7; Ki-67 positivity was between 1 and 3%. Bronchial/bronchiolar wall neuroendocrine cell hyperplasia and several neuroendocrine nodules under 5 mm, with identical morphologic and immunoexpression, were observed, without lung disease. Typical carcinoid associated with DIPNECH and tumorlets without other pulmonary diseases is rare. Sporadic cases may recall embryonal neuroendocrine differentiation potentiality to develop peripheral hyperplasia, most commonly in underoxygenated parenchyma. The described cases are elucidative of peripheral spectrum of neuroendocrine cell tumour evolution, reinforcing higher female incidence as in central carcinoids, still without a clear preneoplastic lesion.

COEXISTENCE OF PULMONARY FIBROSIS AND PROLIFERATION OF PULMONARY NEUROENDOCRINE CELLS FORMING TUMORLET

2021 ◽  
Vol 74 (7) ◽  
pp. 1767-1769
Author(s):  
Iga Panek ◽  
Maciej Liczek ◽  
Igor Rakoczy ◽  
Patrycja Węglarz ◽  
Marcin Braun ◽  
...  
Keyword(s):  
Pulmonary Fibrosis ◽  
Lung Biopsy ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Cell ◽  
High Resolution Computed Tomography ◽  
Cell Hyperplasia ◽  
Pulmonary Neuroendocrine Cells ◽  
Progressive Dyspnea ◽  
Pulmonary Neuroendocrine Cell

Tumorlet is a disease rarely diagnosed in clinical practice. It is characterized by pulmonary neuroendocrine cell (PNEC) proliferation which invades the bronchiolar basement membrane and forms nodules with a diameter smaller than 5 mm. Case report: 72-year-old female patient was suffered for many years from progressive dyspnea and coughing with evidence of pulmonary fibrosis on high resolution computed tomography (HRCT). As a result of a lung biopsy, based on immunohistochemical tests, a 2 mm cluster of neuroendocrine cells (NEC) was found and it was diagnosed as tumorlet. Due to a long-term, insidious progress of the disease, as well as sex and age of the patient, the case emphasizes that differential diagnosis should include tumorlet as well as diffuse idiopathic neuroendocrine cell hyperplasia (DIPNECH) as a more extensive manifestation of neuroendocrine cell proliferation in the respiratory tract.

scholarly journals Diffuse Neuroendocrine Cell Hyperplasia: Report of Two Cases

2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Cevriye Cansız Ersöz ◽  
Ayten Kayı Cangır ◽  
Serpil Dizbay Sak
Keyword(s):  
Carcinoid Tumors ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Cell ◽  
Cell Hyperplasia ◽  
Pulmonary Disorder ◽  
Pulmonary Neuroendocrine Cell

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disorder characterised by a proliferation of neuroendocrine cells within the lung. It is believed that a minority of the patients with DIPNECH can develop carcinoid tumors. Here, we report two new cases of DIPNECH with coexisting carcinoid tumors.

scholarly journals Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Clinical Case

2020 ◽  
Author(s):  
Adelaide Pereira Alves ◽  
Ana Barroso ◽  
Margarida Dias
Keyword(s):  
Clinical Practice ◽  
Therapeutic Approach ◽  
Clinical Case ◽  
Daily Clinical Practice ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Cell ◽  
Cell Hyperplasia ◽  
Bronchial Wall ◽  
Pulmonary Neuroendocrine Cell ◽  
Lung Carcinoid

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary condition characterized by diffuse proliferation of neuroendocrine cells in the epithelium of the bronchial wall. DIPNECH may be easily missed in daily clinical practice and diagnosis is often delayed, which may impair prognosis since this condition is considered a pre-invasive lesion for lung carcinoid tumours. We report a clinical case of DIPNECH in order to discuss the diagnostic and therapeutic approach for this entity, the management of which is not yet well established in the literature.

scholarly journals Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) with liver metastases

2019 ◽  
Vol 12 (6) ◽  
pp. e228536 ◽  
Author(s):  
Kristen Flint ◽  
Chengcheng Ye ◽  
Tracey L Henry
Keyword(s):  
Lung Disease ◽  
Obstructive Lung Disease ◽  
Pulmonary Nodules ◽  
Neuroendocrine Cell ◽  
Radiological Findings ◽  
Cell Hyperplasia ◽  
Metastatic Carcinoid ◽  
Carcinoid Tumours ◽  
Pulmonary Disorder ◽  
Pulmonary Neuroendocrine Cell

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) is a rare pulmonary disorder characterised by classic radiological findings and symptoms of obstructive lung disease. DIPNECH is considered a precursor to carcinoid tumours in the lungs. In this case, we describe a patient with years of unexplained dry cough presenting with 2 weeks of progressive nausea and vomiting, and found to have massive hepatomegaly on examination. By CT-PE, she was diagnosed with DIPNECH, and abdominal MRI revealed metastatic carcinoid tumours. Despite its non-specific presentation, DIPNECH has characteristic radiological findings of mosaic attenuation with numerous pulmonary nodules. DIPNECH requires early identification and close surveillance to prevent progression to carcinoid tumours. Thus, it is critical for frontline providers to consider this diagnosis as part of their differential when other common causes of obstructive lung disease have been ruled out.

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