Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) and the Role of Somatostatin analogs: A Case Series

Chauhan, Aman; Ramirez, Robert A.

doi:10.1007/s00408-015-9754-2

Cited by 33 publications

(18 citation statements)

References 19 publications

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“…Octreotide, a somatostatin analogue shown to reduce the hormonal hypersecretion of neuroendocrine cells in gastrointestinal and bronchial carcinoids [46], has been used with some success, particularly in the presence of somatostatin receptors [47]. CHAUHAN and RAMIREZ [47] retrospectively evaluated five cases of DIPNECH among 184 cases of primary pulmonary neuroendocrine tumours and observed significant improvement of symptoms (cough) in four patients treated with somatostatin analogues. Similar results have recently been reported by CARR et al [21].…”

Section: Management and Prognosismentioning

confidence: 99%

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome

et al. 2016

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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.

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Section: Management and Prognosismentioning

confidence: 99%

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome

et al. 2016

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“…To date, there were four case series reported regarding the SSAs therapy in patients with DIPNECH syndrome. These studies reported significant improvement or even complete regression of the respiratory symptoms (especially cough) in almost all patients treated with SSAs [8,9,13,15].…”

Section: Discussionmentioning

confidence: 96%

“…The clinical course however, is characterized by slowly progressive, unspecific pulmonary symptoms such as long-lasting dry cough, wheezing and exertional dyspnoea [2,[4][5][6][7][8]. Nonetheless, DIPNECH can also cause severe airflow obstruction and respiratory failure which can prove fatal [2,3,7,9].…”

Section: Abbreviationsmentioning

confidence: 99%

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Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) - A Case Report of Successful SSA Therapy after Failure of Systemic Corticosteroids

Badovinac¹,

Harlander²,

Ranković³

et al. 2018

Clin Med Rev Case Rep

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Treatment of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) has not been established yet. The majority of patients is treated with glucocorticoids. This article presents a case when such treatment was inefficient. When switched to somatostatin analogues treatment, patient's clinical condition and radiologic findings improved. Nonetheless, we were not able to prove the expression of somatostatin receptors in the lung nodules. Somatostatin analogues normally act through these receptors, yet it is possible that indirect mechanisms are involved as well. We suggest somatostatin analogues should be implemented as standard treatment for DIPNECH, regardless the presence of somatostatin receptors on lung nodules.

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“…Lung neuroendocrine tumours (NETs) are classified according to their pathological characteristics (Table 1) [2]. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is considered a pre-invasive lesion with a potential toward the development of lung NETs [3,4]. Lung carcinoids (LC) are rare tumours [5]; however, incidence is increasing due to improvement in diagnostic techniques [6].…”

Section: Introductionmentioning

confidence: 99%

68Gallium DOTANOC-PET Imaging in Lung Carcinoids: Impact on Patients' Management

Lamarca

Pritchard

Westwood³

et al. 2017

Neuroendocrinology

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Background:⁶⁸Gallium DOTA-PET imaging is preferable to standard somatostatin receptor scintigraphy where available; however, its role in the management of lung carcinoid tumours (LC) remains unclear. Methods: All consecutive patients with histologically confirmed LC from two ENETS Centres of Excellence were identified retrospectively. The primary objective was to assess the impact of ⁶⁸Ga-DOTANOC-PET on clinical management in patients with LC. Results: Of 166 patients screened, 46 were eligible: 52% female, median age 57 years (range 21-86); type of LC: diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (4%), typical (44%), atypical (35%), not reported (17%); stage: localised (63%), locally advanced (13%), and metastatic (17%) (7% unknown). A total of 47 ⁶⁸Ga-DOTANOCs were performed with the following rationale: LC diagnosis confirmation (4; 9%), primary tumour identification (2; 4%), post-surgical assessment (19; 40%), staging (patients with known LC present at time of ⁶⁸Ga-DOTANOC) (19; 40%), and consideration of peptide receptor radionuclide therapy (3; 7%). Twenty-seven (57%) scans showed evidence of non-physiological uptake: median maximum standardised uptake value 7.2 (range 1.42-53). ⁶⁸Ga-DOTANOC provided additional information in 37% (95% CI 22-51) of patients and impacted on management in 26% (95% CI 12-41); 9 patients (21%) were identified to have occult sites of metastases. Out of the 19 patients with post-surgical ⁶⁸Ga-DOTANOC, 3 (16%) were identified to have distant metastases. There were no differences in the rate of practice changing ⁶⁸Ga-DOTANOC results by type of LC (p value 0.5). Conclusions: Our results support the role of ⁶⁸Ga-DOTANOC for optimising the management of patients with LC, including post-surgical re-staging due to the potential for identifying occult metastases.

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Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) and the Role of Somatostatin analogs: A Case Series

Cited by 33 publications

References 19 publications

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) - A Case Report of Successful SSA Therapy after Failure of Systemic Corticosteroids

68Gallium DOTANOC-PET Imaging in Lung Carcinoids: Impact on Patients' Management

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