Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) in a 50-Year-Old Woman
,
Abstract
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disorder characterized by hyperplasia of pulmonary neuroendocrine cells. It is frequently asymptomatic and can be challenging to differentiate from other forms of reactive pulmonary neuroendocrine cell hyperplasia (NECH). Presented is a case report of DIPNECH along with a review of the diagnosis and management. A 50-year-old
female patient with a history of airway disease presented to the pulmonary ward with complaints of Functional Class III dyspnea and productive cough. The HRCT revealed the presence of multiple bilateral nodules in both lungs. The histopathology report from the nodule resection confirmed the presence of a carcinoid tumor, specifically identified as DIPNECH. As many similar cases have favorable treatment responses and satisfactory prognoses due to multidisciplinary treatment methods, the necessity of evidence-based management guidelines for DIPNECH and the accuracy of the disease definition is emphasized.
[1] Hendifar AE, Marchevsky AM, Tuli R. Neuroendocrine tumors of the lung: current challenges and advances in the
diagnosis and management of well-differentiated disease. J Thorac Oncol. 2017;12(3):425-36. https://doi.org/10.1016/j.
jtho.2016.11.2222
[2] Lázaro S, et al. Differential development of large-cell neuroendocrine or small-cell lung carcinoma upon
inactivation of 4 tumor suppressor genes. Proc Natl Acad Sci U S A. 2019;116(44):22300-6. https://doi.org/10.1073/
pnas.1821745116
[3] Caplin ME, et al. Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids. Ann Oncol. 2015;26(8):1604-20. https://doi.org/10.1093/annonc/mdv041
[4] Pelosi G, et al. Classification of pulmonary neuroendocrine tumors: new insights. Transl Lung Cancer Res. 2017;6(5):513- 29. https://doi.org/10.21037/tlcr.2017.09.04
[5] Filosso PL, Falcoz PE, Solidoro P, Pellicano D, Passani S, Guerrera F, Ruffini E, & ESTS Lung Neuroendocrine WorkingGroup Participating Centers. The European Society of Thoracic Surgeons (ESTS) lung neuroendocrine tumors (NETs) database. J Thorac Dis. 2018;10(Suppl 29):S3528-S3532 . https://doi.org/10.21037/jtd.2018.04.104
[6] Benzerdjeb N, Berna P, Sevestre H. GLUT1: A novel tool reflecting proliferative activity of lung neuroendocrine tumors? Pathol Int. 2017;67(1):32-6. https://doi.org/10.1111/pin.12486
[7] Peinado P, Arco C, Fernández-Aceñero MJ. Neuroendocrine tumors in rare locations: Description of 27 cases. Med Clin (Barc). 2019;155(10):460-1. https://doi.org/10.1016/j. medcli.2019.05.023
[8] Warren WH, et al. Neuroendocrine neoplasms of the bronchopulmonary tract: a classification of the spectrum of
carcinoid to small cell carcinoma and intervening variants. J Thorac Cardiovasc Surg. 1985;89(6):819-25. https://doi.
org/10.1016/S0022-5223(19)38687-8
[9] Hauso O, et al. Neuroendocrine tumor epidemiology: contrasting Norway and North America. Cancer. 2008;113(10):2655-64.https://doi.org/10.1002/cncr.23883
[10] Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003;97(4):934-59. https://doi.
org/10.1002/cncr.11105
[11] Travis WD. Pathology and genetics of tumours of the lung pleura, thymus, and heart. Lyon: IARC Press; 2004.
[12] Travis WD. WHO classification of tumors. Pathology and Genetics of Tumors of the Lung, Pleura, Thymus and Heart.
Lyon: IARC Press; 2004:128-36.
[13] Travis WD, et al. Introduction to the 2015 World Health Organization classification of tumors of the lung, pleura,
thymus, and heart. J Thorac Oncol. 2015;10(9):1240-2. https://doi.org/10.1097/JTO.0000000000000663
[14] Samhouri BF, et al. Is the combination of bilateral pulmonary nodules and mosaic attenuation on chest CT specific for DIPNECH? Orphanet J Rare Dis. 2021;16:1-11. https://doi.org/10.1186/s13023-021-02103-w
[15] Rossi G, et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome. Eur Respir J. 2016;47(6):1829-41. https://doi.org/10.1183/13993003.01954-2015
[16] Barbareschi M, Mengoli MC, Cavazza A. Practical Pulmonary Pathology: A Diagnostic Approach, E-Book: A Volume in the Pattern Recognition Series. 2022:303.
[17] Nassar AA, et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a systematic overview. Am J Respir Crit Care Med. 2011;184(1):8-16. https://doi.org/10.1164/rccm.201010-1685PP
[18] Purdy A, Ido F, Stahlnecker D. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): A Case of
Indolent Pulmonary Nodules Diagnosed with RoboticAssisted Navigational Bronchoscopy. Case Rep Pulmonol.
2021;2021:6312296. https://doi.org/10.1155/2021/6312296
[19] Davies SJ, et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax. 2007;62(3):248-52. https://doi.org/10.1136/thx.2006.063065
[20] Babalola O, Muskrat J, Kanchustambham V. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Progressing to Carcinoid Tumor: A Case of Chronic Cough. Cureus. 2023;15(10):e46689 . https://doi.org/10.7759/cureus.46659
[21] Tigges S. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). Radiopaedia.org. 2024. Available from: https://doi.org/10.53347/rID-95994. https://doi.org/10.53347/rID-95994
[22] Gutierrez M, et al. Radiological-pathological correlation in diffuse idiopathic pulmonary neuroendocrine cell
hyperplasia (DIPNECH): imaging and histopathology. Clin Radiol. 2024;79(2):133-41. https://doi.org/10.1016/j.crad.
2023.10.013
[23] Hurabielle C, et al. De-labelling severe asthma diagnosis: the challenge of DIPNECH. ERJ Open Res. 2022;8(1):00631-2021. https://doi.org/10.1183/23120541.00485-2021
[24] Heaney LG, et al. Eosinophilic and noneosinophilic asthma: an expert consensus framework to characterize phenotypes in a global real-life severe asthma cohort. Chest. 2021;160(3):814-30. https://doi.org/10.1016/j.chest.2021.04.013
[25] Sazonova O, et al. Development and validation of diffuse idiopathic pulmonary neuroendocrine hyperplasia diagnostic criteria. JTO Clin Res Rep. 2020;1(4):100078. https://doi.org/10.1016/j.jtocrr.2020.100078
[26] Carr LL, et al. The clinical course of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. Chest. 2015;147(2):415-22. https://doi.org/10.1378/chest.14-0711
[27] Hayes AR, et al. 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. J Neuroendocrinol. 2022;34(10):e13184 . https://doi. org/10.1111/jne.13184
diagnosis and management of well-differentiated disease. J Thorac Oncol. 2017;12(3):425-36. https://doi.org/10.1016/j.
jtho.2016.11.2222
[2] Lázaro S, et al. Differential development of large-cell neuroendocrine or small-cell lung carcinoma upon
inactivation of 4 tumor suppressor genes. Proc Natl Acad Sci U S A. 2019;116(44):22300-6. https://doi.org/10.1073/
pnas.1821745116
[3] Caplin ME, et al. Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids. Ann Oncol. 2015;26(8):1604-20. https://doi.org/10.1093/annonc/mdv041
[4] Pelosi G, et al. Classification of pulmonary neuroendocrine tumors: new insights. Transl Lung Cancer Res. 2017;6(5):513- 29. https://doi.org/10.21037/tlcr.2017.09.04
[5] Filosso PL, Falcoz PE, Solidoro P, Pellicano D, Passani S, Guerrera F, Ruffini E, & ESTS Lung Neuroendocrine WorkingGroup Participating Centers. The European Society of Thoracic Surgeons (ESTS) lung neuroendocrine tumors (NETs) database. J Thorac Dis. 2018;10(Suppl 29):S3528-S3532 . https://doi.org/10.21037/jtd.2018.04.104
[6] Benzerdjeb N, Berna P, Sevestre H. GLUT1: A novel tool reflecting proliferative activity of lung neuroendocrine tumors? Pathol Int. 2017;67(1):32-6. https://doi.org/10.1111/pin.12486
[7] Peinado P, Arco C, Fernández-Aceñero MJ. Neuroendocrine tumors in rare locations: Description of 27 cases. Med Clin (Barc). 2019;155(10):460-1. https://doi.org/10.1016/j. medcli.2019.05.023
[8] Warren WH, et al. Neuroendocrine neoplasms of the bronchopulmonary tract: a classification of the spectrum of
carcinoid to small cell carcinoma and intervening variants. J Thorac Cardiovasc Surg. 1985;89(6):819-25. https://doi.
org/10.1016/S0022-5223(19)38687-8
[9] Hauso O, et al. Neuroendocrine tumor epidemiology: contrasting Norway and North America. Cancer. 2008;113(10):2655-64.https://doi.org/10.1002/cncr.23883
[10] Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003;97(4):934-59. https://doi.
org/10.1002/cncr.11105
[11] Travis WD. Pathology and genetics of tumours of the lung pleura, thymus, and heart. Lyon: IARC Press; 2004.
[12] Travis WD. WHO classification of tumors. Pathology and Genetics of Tumors of the Lung, Pleura, Thymus and Heart.
Lyon: IARC Press; 2004:128-36.
[13] Travis WD, et al. Introduction to the 2015 World Health Organization classification of tumors of the lung, pleura,
thymus, and heart. J Thorac Oncol. 2015;10(9):1240-2. https://doi.org/10.1097/JTO.0000000000000663
[14] Samhouri BF, et al. Is the combination of bilateral pulmonary nodules and mosaic attenuation on chest CT specific for DIPNECH? Orphanet J Rare Dis. 2021;16:1-11. https://doi.org/10.1186/s13023-021-02103-w
[15] Rossi G, et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome. Eur Respir J. 2016;47(6):1829-41. https://doi.org/10.1183/13993003.01954-2015
[16] Barbareschi M, Mengoli MC, Cavazza A. Practical Pulmonary Pathology: A Diagnostic Approach, E-Book: A Volume in the Pattern Recognition Series. 2022:303.
[17] Nassar AA, et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a systematic overview. Am J Respir Crit Care Med. 2011;184(1):8-16. https://doi.org/10.1164/rccm.201010-1685PP
[18] Purdy A, Ido F, Stahlnecker D. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): A Case of
Indolent Pulmonary Nodules Diagnosed with RoboticAssisted Navigational Bronchoscopy. Case Rep Pulmonol.
2021;2021:6312296. https://doi.org/10.1155/2021/6312296
[19] Davies SJ, et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax. 2007;62(3):248-52. https://doi.org/10.1136/thx.2006.063065
[20] Babalola O, Muskrat J, Kanchustambham V. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Progressing to Carcinoid Tumor: A Case of Chronic Cough. Cureus. 2023;15(10):e46689 . https://doi.org/10.7759/cureus.46659
[21] Tigges S. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). Radiopaedia.org. 2024. Available from: https://doi.org/10.53347/rID-95994. https://doi.org/10.53347/rID-95994
[22] Gutierrez M, et al. Radiological-pathological correlation in diffuse idiopathic pulmonary neuroendocrine cell
hyperplasia (DIPNECH): imaging and histopathology. Clin Radiol. 2024;79(2):133-41. https://doi.org/10.1016/j.crad.
2023.10.013
[23] Hurabielle C, et al. De-labelling severe asthma diagnosis: the challenge of DIPNECH. ERJ Open Res. 2022;8(1):00631-2021. https://doi.org/10.1183/23120541.00485-2021
[24] Heaney LG, et al. Eosinophilic and noneosinophilic asthma: an expert consensus framework to characterize phenotypes in a global real-life severe asthma cohort. Chest. 2021;160(3):814-30. https://doi.org/10.1016/j.chest.2021.04.013
[25] Sazonova O, et al. Development and validation of diffuse idiopathic pulmonary neuroendocrine hyperplasia diagnostic criteria. JTO Clin Res Rep. 2020;1(4):100078. https://doi.org/10.1016/j.jtocrr.2020.100078
[26] Carr LL, et al. The clinical course of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. Chest. 2015;147(2):415-22. https://doi.org/10.1378/chest.14-0711
[27] Hayes AR, et al. 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. J Neuroendocrinol. 2022;34(10):e13184 . https://doi. org/10.1111/jne.13184
| Files | ||
| Issue | Vol 9 No 4 (2024): July-August | |
| Section | Case Report(s) | |
| DOI | https://doi.org/10.18502/crcp.v9i4.17980 | |
| Keywords | ||
| Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) Pulmonary neuroendocrine cell hyperplasia Diagnosis | ||
| Rights and permissions | |
|
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Mohseni Ahangar R, Firuzpour F, Aram C. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) in a 50-Year-Old Woman. CRCP. 2025;9(4):152-158.
