DIPNECH: Association Between Histopathology and Clinical Presentation

Trisolini, Rocco; Valentini, Ilaria; Tinelli, Carmine; Ferrari, Marco; Guiducci, Gian Marco; Parri, Sergio Nicola Forti; Dalpiaz, Giorgia; Cancellieri, Alessandra

doi:10.1007/s00408-016-9854-7

Cited by 14 publications

(9 citation statements)

References 16 publications

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“…To the best of our knowledge, this multicenter study is the first to focus on long-term CT follow-up in DIPNECH, and our cohort is one of the largest reported to date [5,6,[15][16][17][18][19][20]. As in other reported series, we found a strong female predominance with 93% of women in our cohort compared to 79 to 100% in the literature [6,15].…”

Section: Discussionsupporting

confidence: 63%

Long-Term Imaging Follow-Up in DIPNECH: Multicenter Experience

Chung

Bommart

Marchand-Adam

et al. 2021

JCM

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Diffuse pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pre-invasive disease whose pathophysiology remains unclear. We aimed to assess long-term evolution in imaging of DIPNECH, in order to propose follow-up recommendations. Patients with histologically confirmed DIPNECH from four centers, evaluated between 2001 and 2020, were enrolled if they had at least two available chest computed tomography (CT) exams performed at least 24 months apart. CT exams were analyzed for the presence and the evolution of DIPNECH-related CT findings. Twenty-seven patients, mostly of female gender (n = 25/27; 93%) were included. Longitudinal follow-up over a median 63-month duration (IQR: 31–80 months) demonstrated an increase in the size of lung nodules in 19 patients (19/27, 70%) and the occurrence of metastatic spread in three patients (3/27, 11%). The metastatic spread was limited to mediastinal lymph nodes in one patient, whereas the other two patients had both lymph node and distant metastases. The mean time interval between baseline CT scan and metastatic spread was 70 months (14, 74 and 123 months). Therefore, long-term annual imaging follow-up of DIPNECH might be appropriate to encompass the heterogeneous longitudinal behavior of this disease.

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Section: Discussionsupporting

confidence: 63%

Long-Term Imaging Follow-Up in DIPNECH: Multicenter Experience

Chung

Bommart

Marchand-Adam

et al. 2021

JCM

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“…A previous report noted that chronic obstructive pulmonary disease had low-level increases in circulating NETest gene expression [17]. We postulate that this increase in gene expression reflects the well-described increase in histological foci of pulmonary neuroendocrine cell proliferation and tumorlets that occur in this disease [41]. A similar process has been described in chronic gastritis with increased ECL cell proliferation and concomitant increases in neuroendocrine gene expression [42].…”

Section: Discussionmentioning

confidence: 64%

NETest Liquid Biopsy Is Diagnostic of Lung Neuroendocrine Tumors and Identifies Progressive Disease

et al. 2019

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Background: There are no effective biomarkers for the management of bronchopulmonary carcinoids (BPC). We examined the utility of a neuroendocrine multigene transcript “liquid biopsy” (NETest) in BPC for diagnosis and monitoring of the disease status. Aim: To independently validate the utility of the NETest in diagnosis and management of BPC in a multicenter, multinational, blinded study. Material and Methods: The study cohorts assessed were BPC (n = 99), healthy controls (n = 102), other lung neoplasia (n = 101) including adenocarcinomas (ACC) (n = 41), squamous cell carcinomas (SCC) (n = 37), small-cell lung cancer (SCLC) (n = 16), large-cell neuroendocrine carcinoma (LCNEC) (n = 7), and idiopathic pulmonary fibrosis (IPF) (n = 50). BPC were histologically classified as typical (TC) (n = 62) and atypical carcinoids (AC) (n = 37). BPC disease status determination was based on imaging and RECIST 1.1. NETest diagnostic metrics and disease status accuracy were evaluated. The upper limit of normal (NETest) was 20. Twenty matched tissue-blood pairs were also evaluated. Data are means ± SD. Results: NETest levels were significantly increased in BPC (45 ± 25) versus controls (9 ± 8; p < 0.0001). The area under the ROC curve was 0.96 ± 0.01. Accuracy, sensitivity, and specificity were: 92, 84, and 100%. NETest was also elevated in SCLC (42 ± 32) and LCNEC (28 ± 7). NETest accurately distinguished progressive (61 ± 26) from stable disease (35.5 ± 18; p < 0.0001). In BPC, NETest levels were elevated in metastatic disease irrespective of histology (AC: p < 0.02; TC: p = 0.0006). In nonendocrine lung cancers, ACC (18 ± 21) and SCC (12 ± 11) and benign disease (IPF) (18 ± 25) levels were significantly lower compared to BPC level (p < 0.001). Significant correlations were evident between paired tumor and blood samples for BPC (R: 0.83, p < 0.0001) and SCLC (R: 0.68) but not for SCC and ACC (R: 0.25–0.31). Conclusions: Elevated NETest levels are indicative of lung neuroendocrine neoplasia. NETest levels correlate with tumor tissue and imaging and accurately define clinical progression.

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“…In a subgroup of patients, the disease is accidentally diagnosed in the course of the study of another entity, usually a neoplasm. In another, patients have a clinical history characterized by cough and dyspnea that have worsened slowly over years (3). A minority may also present with hemoptysis or wheezing (2).…”

Section: Discussionmentioning

confidence: 99%

Coexistence of DIPNECH and carotid body paraganglioma: is it just a coincidence?

Pedro

Cunha

Neto

et al. 2020

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary We describe the case of a 56 year-old woman with the almost simultaneous appearance of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) and a carotid body paraganglioma. Of interest, 6 years earlier, the patient underwent total thyroidectomy due to papillary thyroid carcinoma and, in the meantime, she was submitted to mastectomy to treat an invasive ductal carcinoma of the breast. In order to explain these lesions, an extensive genetic study was performed. Results showed positivity for the presence of the tumor suppressor gene PALB2, whose presence had already been detected in a niece with breast cancer. The patient underwent different procedures to treat the lesions and currently she is symptom-free over 2 years of follow-up. Learning points: The presence of two rare neoplasms in a single person should raise the suspicion of a common etiology. To the best of our knowledge, this is the first case that shows the coexistence of DIPNECH and paraganglioma. The contribution of the PALB2 gene in the etiology of these rare neoplasms is a possibility.

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DIPNECH: Association Between Histopathology and Clinical Presentation

Cited by 14 publications

References 16 publications

Long-Term Imaging Follow-Up in DIPNECH: Multicenter Experience

Long-Term Imaging Follow-Up in DIPNECH: Multicenter Experience

NETest Liquid Biopsy Is Diagnostic of Lung Neuroendocrine Tumors and Identifies Progressive Disease

Coexistence of DIPNECH and carotid body paraganglioma: is it just a coincidence?

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