A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia

Ofikwu, Godwin; Mani, Vishnu R.; Rajabalan, Ajai; Adu, Albert; Ahmed, Leaque; Vega, David

doi:10.1155/2015/318175

Cited by 6 publications

(9 citation statements)

References 5 publications

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“…Other potential therapies are somatostatin analogues, however more studies are needed to determine their precise role. (27,29,43,46) Conclusion DIPNECH is a rare clinical entity that requires a high clinical suspicion. Because of clinical, spirometry, and imaging similarity to other obstructive lung diseases, and the requirement for lung biopsy to make the diagnosis, DIPNECH is probably an underdiagnosed entity, with still limited treatment options.…”

Section: Discussionmentioning

confidence: 97%

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia in a patient with multiple pulmonary nodules: case report and literature review

Yamin¹,

Hawarri²,

Labib³

et al. 2017

Southwest J Pulm Crit Care

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Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disease, where carcinoid tumorlets invade the pulmonary parenchyma and bronchioles. These nests of cells release a variety of mediators including bombesin and gastrin releasing peptide that cause heterogeneous bronchoconstriction, creating a mosaic appearance on chest imaging studies, especially on expiratory scans. Clinically patients usually have long standing symptoms of shortness of breath (SOB) and cough that are difficult to distinguish from asthma. In this article we describe a case of DIPNECH in a patient with several years' history of SOB and cough, and review 179 cases of DIPNECH reported in the literature since 1992. Case PresentationA 72-year-old, non-smoking lady was admitted to the hospital in preparation for bilateral mastectomy. She recently received a diagnosis of bilateral breast invasive ductal carcinoma grade 2, estrogen receptor/progesterone receptor/human epidermal growth factor receptor 2 (HER-2) positive in the left tumor but negative in the right tumor.Her past medical history was significant for hypertension, long standing cough and dyspnea on exertion labeled as asthma poorly responsive to nebulizers. Socially, she was a house wife with no history of occupational exposure.The patient was found to be tachypneic (respiratory rate 22 breaths/minute) and hypoxemic (oxygen saturation 86% on room air). Heart rate and blood pressure were within normal limits. She had bilateral decreased breath sounds and diffuse expiratory wheezes.Chest CT scan revealed diffuse mosaic pattern and multiple pulmonary nodules in both lungs suggestive of metastases (Figure 1).

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Section: Discussionmentioning

confidence: 97%

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia in a patient with multiple pulmonary nodules: case report and literature review

Yamin¹,

Hawarri²,

Labib³

et al. 2017

Southwest J Pulm Crit Care

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“…A specimen must have five or more neuroendocrine cells alone or clustered in the basement membrane of the epithelium of at least three bronchioles, and three or more carcinoid tumorlets 1. These cells typically stain positively for the neuroendocrine markers synaptophysin, chromogranin and CD56 7…”

Section: Discussionmentioning

confidence: 99%

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

Flint

Henry³

2019

BMJ Case Rep

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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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“…The pathological diagnosis criteria in surgical samples are the presence of five or more NE cells, either singly or in clusters, located within the basement membrane of the bronchiolar epithelium of at least three bronchioles, combined with three or more carcinoid tumorlets [ 1 ]. The NE cells in DIPNECH are positive for neuroendocrine markers of differentiation such as synaptophysin, chromogranin, and CD56 [ 2 ]. CD10 is stained consistently in neuroendocrine cells.…”

Section: Discussionmentioning

confidence: 99%

“…DIPNECH is considered a precursor lesion for carcinoid tumors, which can be typical or atypical carcinoids depending on their grade—low and intermediate grade, respectively [ 2 ]. However, the risk of developing a clinically significant disease from these lesions appears to be low.…”

Section: Introductionmentioning

confidence: 99%