Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Syndrome Treated With Sirolimus

Russier, M.; Plantier, Laurent; Derot, Gaelle; Muret, A. De; Marchand-Adam, S.

doi:10.7326/l17-0634

Cited by 10 publications

(8 citation statements)

References 5 publications

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“…Two patients had radiological improvement, 1 patient had radiological progression, and all 3 patients demonstrated improvement in FEV1 over time. [39] In our study, one patient received everolimus for metastatic pulmonary carcinoid. This patient reported stable symptoms, but manifested radiological progression on follow-up.…”

Section: Discussionmentioning

confidence: 89%

Constrictive bronchiolitis in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

et al. 2020

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BackgroundDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is characterised by multifocal proliferation of neuroendocrine cells and belongs in the spectrum of pulmonary neuroendocrine tumors. Some patients with DIPNECH develop airflow obstruction but the relationship between the two entities remains unclear.MethodsWe performed a computer-assisted search of the Mayo Clinic's electronic medical records for biopsy-proven cases of DIPNECH. We extracted clinical, pulmonary function, imaging and histopathologic data along with treatments and outcomes.ResultsAmong 44 patients with DIPNECH 91% were female and median age was 65 years (IQR: 56–69 years); 73% were never smokers. 38 patients (86%) had respiratory symptoms including cough (68%) and dyspnea (30%); 45% were previously diagnosed to have asthma or COPD. Pulmonary function testing showed an obstructive pattern in 52%, restrictive pattern 11%, mixed pattern 9%, nonspecific pattern 23%, and normal 5%. On chest CT scan, 95% manifested diffuse nodules and 77% manifested mosaic attenuation. For management, 25% of patients were observed without pharmacologic therapy, 55% received an inhaled bronchodilator, 41% received an inhaled corticosteroid, 32% received octreotide; systemic steroids, azithromycin, or combination chemotherapy was employed in 4 patients (9%). Of 24 patients with follow-up PFT available, 50% remained stable, 33% worsened and 17% improved over a median interval of 21.3 months (IQR: 9.7–46.9 months).ConclusionDIPNECH occurs mostly in women and manifests diffuse pulmonary nodules and mosaic attenuation on imaging. It is commonly associated with airflow obstruction due to constrictive bronchiolitis, which manifests limited response to current pharmacologic therapy.

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Section: Discussionmentioning

confidence: 89%

Constrictive bronchiolitis in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

et al. 2020

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“…Similar to lung NETs, 20 activation of the mechanistic target of rapamycin (i.e., mTOR) pathway has been demonstrated in DIPNECH 5,21 and this may be another avenue for investigation in patients with respiratory symptoms. In a small case series of three women with DIPNECH and exertional dyspnoea treated with sirolimus, two patients demonstrated radiological improvement, one demonstrated radiological progression and all three showed improvement of FEV 1 during follow‐up 22 …”

Section: Discussionmentioning

confidence: 97%

“…In a small case series of three women with DIPNECH and exertional dyspnoea treated with sirolimus, two patients demonstrated radiological improvement, one demonstrated radiological progression and all three showed improvement of FEV 1 during follow-up. 22 Thirty-six patients had a BMI available on first clinical review, and a high proportion of patients were obese (42%) or morbidly obese (22%). This is difficult to interpret given the small sample size; however, it appears higher than the prevalence of obesity (30%) and morbid obesity (5%) in women aged 45-64 years in England.…”

Section: Discussionmentioning

confidence: 99%

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH): Prevalence, clinicopathological characteristics and survival outcome in a cohort of 311 patients with well‐differentiated lung neuroendocrine tumours

Hayes

Luong

Banks

et al. 2022

J Neuroendocrinology

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Introduction: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is considered to be a rare condition associated with lung neuroendocrine tumours (NET), and its natural history is poorly described. We aimed to assess the prevalence and clinicopathologic characteristics of DIPNECH in the lung NET population, and to investigate predictors of time-to-progression (TTP) and overall survival (OS). Methods:We retrospectively identified patients diagnosed with DIPNECH between April 2005 and December 2020. Clinical data were collected from medical records.The relationship between baseline characteristics and TTP and OS was analysed using the Kaplan-Meier method. Univariate analysis was performed using the Cox proportional hazards model. Results:Of 311 patients with well-differentiated lung NETs, 61 (20%) had DIPNECH and were included in the study. Baseline demographics described 95% female, 59% never smokers and mean body mass index 34.4 kg m -2 ; 77% were typical carcinoids (TC), 13% atypical carcinoids (AC), and 10% both TC and AC (multicentric). At presentation, 54% of patients were asymptomatic. Multicentric NETs were demonstrated in 16 (26%) on histopathology, and a further 32 (52%) had synchronous NETs suggested on imaging (multiple nodules ≥ 5 mm). Seven (11%) patients developed metastases and the median OS from time of first metastasis was 37 months. AC histopathology and NET TNM stage ≥ IIA were associated with poorer TTP and OS. Of the DIP-NECH cohort, the 15-year survival rate was 86%.Conclusions: DIPNECH may be more prevalent in the lung NET population than previously appreciated, especially in women. Although our results confirm that DIP-NECH is predominantly an indolent disease associated with TC, 23% developed AC and these patients may warrant closer observation.

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“…Another potential treatment is targeted therapy using an mTOR inhibitor such as everolimus or sirolimus, as DIPNECH has been found to exhibit activation of the mTOR pathway ( 125 ). There is no substantial data on the efficacy of mTOR inhibitors to advocate its use with DIPNECH as studies have not demonstrated symptomatic improvement or changes of FEV1 other than improved radiographic findings ( 117 , 126 ). Lastly, there are case reports utilizing lung transplantation as primary treatment for DIPNECHs ( 116 , 127 ).…”

Section: Dipnechmentioning

confidence: 99%

A multidisciplinary approach to the work up and management of pulmonary carcinoid tumors and DIPNECH: a narrative review

Ramirez

Cass

Das

et al. 2022

Transl Lung Cancer Res

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Background and Objective: Low and intermediate grade neuroendocrine tumors of the lung are uncommon malignancies representing 2% of all lung cancers. These are termed typical and atypical pulmonary carcinoid tumors. These can arise in the setting of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). The presentation, workup, management and outcomes of patients with these tumors can overlap with more common lung cancers but differ in that many of these patients have a prolonged clinical course. The objective of this narrative review is to summarize the literature and provide evidence and expert-based algorithms for work up and treatment of pulmonary carcinoids and DIPNECH.

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Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Syndrome Treated With Sirolimus

Cited by 10 publications

References 5 publications

Constrictive bronchiolitis in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

Constrictive bronchiolitis in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH): Prevalence, clinicopathological characteristics and survival outcome in a cohort of 311 patients with well‐differentiated lung neuroendocrine tumours

A multidisciplinary approach to the work up and management of pulmonary carcinoid tumors and DIPNECH: a narrative review

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