Skip to main content
Erschienen in: Lung 5/2015

01.10.2015

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence

verfasst von: Eric Wirtschafter, Ann E. Walts, Sandy T. Liu, Alberto M. Marchevsky

Erschienen in: Lung | Ausgabe 5/2015

Einloggen, um Zugang zu erhalten

Abstract

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is recognized as a preneoplastic condition by the World Health Organization. We reviewed our experience with 30 patients and performed a systematic review of the English literature to collect best evidence on the clinical features and disease course in 169 additional patients. Some patients presented with one or more carcinoid tumors associated with multiple small pulmonary nodules on imaging studies and showed DIPNECH as a somewhat unexpected pathologic finding. Others presented with multiple small pulmonary nodules that raised suspicion of metastatic disease on imaging. A third subset was presented with previously unexplained respiratory symptoms. In most patients, DIPNECH was associated with a good prognosis, with chronological progression into a subsequent carcinoid tumor noted in only one patient and death attributed directly to DIPNECH in only two patients. There is no best evidence to support the use of octreotide, steroids, or bronchodilators in DIPNECH patients.
Literatur
1.
Zurück zum Zitat Champaneria MC, Modlin IM, Kidd M et al (2006) Friedrich Feyrter: a precise intellect in a diffuse system. Neuroendocrinology 83(5–6):394–404CrossRefPubMed Champaneria MC, Modlin IM, Kidd M et al (2006) Friedrich Feyrter: a precise intellect in a diffuse system. Neuroendocrinology 83(5–6):394–404CrossRefPubMed
2.
3.
Zurück zum Zitat Sunday ME, Hua J, Dai HB et al (1990) Bombesin increases fetal lung growth and maturation in utero and in organ culture. Am J Respir Cell Mol Biol 3(3):199–205CrossRefPubMed Sunday ME, Hua J, Dai HB et al (1990) Bombesin increases fetal lung growth and maturation in utero and in organ culture. Am J Respir Cell Mol Biol 3(3):199–205CrossRefPubMed
4.
Zurück zum Zitat Wharton J, Polak JM, Bloom SR et al (1978) Bombesin-like immunoreactivity in the lung. Nature 273(5665):769–770CrossRefPubMed Wharton J, Polak JM, Bloom SR et al (1978) Bombesin-like immunoreactivity in the lung. Nature 273(5665):769–770CrossRefPubMed
5.
Zurück zum Zitat Alshehri M, Cutz E, Banzhoff A et al (1997) Hyperplasia of pulmonary neuroendocrine cells in a case of childhood pulmonary emphysema. Chest 112(2):553–556CrossRefPubMed Alshehri M, Cutz E, Banzhoff A et al (1997) Hyperplasia of pulmonary neuroendocrine cells in a case of childhood pulmonary emphysema. Chest 112(2):553–556CrossRefPubMed
6.
Zurück zum Zitat Yeger H, Speirs V, Youngson C et al (1996) Pulmonary neuroendocrine cell in cultures of human infant airway mucosa from postmortem tissues. Am J Respir Cell Mol Biol 15(2):232–236CrossRefPubMed Yeger H, Speirs V, Youngson C et al (1996) Pulmonary neuroendocrine cell in cultures of human infant airway mucosa from postmortem tissues. Am J Respir Cell Mol Biol 15(2):232–236CrossRefPubMed
7.
Zurück zum Zitat Benson REC, Rosado-de-Christenson ML, Martinez-Jimenez S et al (2013) Spectrum of pulmonary neuroendocrine proliferations and neoplasms. Radiographics 33(6):1631–1649CrossRefPubMed Benson REC, Rosado-de-Christenson ML, Martinez-Jimenez S et al (2013) Spectrum of pulmonary neuroendocrine proliferations and neoplasms. Radiographics 33(6):1631–1649CrossRefPubMed
8.
Zurück zum Zitat Gosney JR (2004) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia as a precursor to pulmonary neuroendocrine tumors. Chest 125(5 Suppl):108SCrossRefPubMed Gosney JR (2004) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia as a precursor to pulmonary neuroendocrine tumors. Chest 125(5 Suppl):108SCrossRefPubMed
9.
Zurück zum Zitat Miller MA, Mark GJ, Kanarek D (1978) Multiple peripheral pulmonary carcinoids and tumorlets of carcinoid type, with restrictive and obstructive lung disease. Am J Med 65(2):373–378CrossRefPubMed Miller MA, Mark GJ, Kanarek D (1978) Multiple peripheral pulmonary carcinoids and tumorlets of carcinoid type, with restrictive and obstructive lung disease. Am J Med 65(2):373–378CrossRefPubMed
10.
Zurück zum Zitat Aguayo SM, Miller YE, Waldron JA Jr et al (1992) Brief report: idiopathic diffuse hyperplasia of pulmonary neuroendocrine cells and airways disease. New Engl J Med 327(18):1285–1288CrossRefPubMed Aguayo SM, Miller YE, Waldron JA Jr et al (1992) Brief report: idiopathic diffuse hyperplasia of pulmonary neuroendocrine cells and airways disease. New Engl J Med 327(18):1285–1288CrossRefPubMed
11.
Zurück zum Zitat Travis W, Nicholson A, Marx A (eds) (2015) Tumors of the lung, pleura thymus and heart. IARC Press, Lyon Travis W, Nicholson A, Marx A (eds) (2015) Tumors of the lung, pleura thymus and heart. IARC Press, Lyon
12.
Zurück zum Zitat Marchevsky AM, Wirtschafter E, Walts AE (2015) The spectrum of changes in adults with multifocal pulmonary neuroendocrine proliferations: what is the minimum set of pathologic criteria to diagnose DIPNECH? Hum Pathol 46(2):176–181CrossRefPubMed Marchevsky AM, Wirtschafter E, Walts AE (2015) The spectrum of changes in adults with multifocal pulmonary neuroendocrine proliferations: what is the minimum set of pathologic criteria to diagnose DIPNECH? Hum Pathol 46(2):176–181CrossRefPubMed
13.
14.
Zurück zum Zitat Davies SJ, Gosney JR, Hansell DM et al (2007) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax 62(3):248–252PubMedCentralCrossRefPubMed Davies SJ, Gosney JR, Hansell DM et al (2007) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax 62(3):248–252PubMedCentralCrossRefPubMed
15.
Zurück zum Zitat Al-Ayoubi AM, Ralston JS, Richardson SR et al (2014) Diffuse pulmonary neuroendocrine cell hyperplasia involving the chest wall. Ann Thorac Surg 97(1):333–335CrossRefPubMed Al-Ayoubi AM, Ralston JS, Richardson SR et al (2014) Diffuse pulmonary neuroendocrine cell hyperplasia involving the chest wall. Ann Thorac Surg 97(1):333–335CrossRefPubMed
16.
Zurück zum Zitat Aubry MC, Thomas CF Jr, Jett JR et al (2007) Significance of multiple carcinoid tumors and tumorlets in surgical lung specimens: analysis of 28 patients. Chest 131(6):1635–1643CrossRefPubMed Aubry MC, Thomas CF Jr, Jett JR et al (2007) Significance of multiple carcinoid tumors and tumorlets in surgical lung specimens: analysis of 28 patients. Chest 131(6):1635–1643CrossRefPubMed
17.
Zurück zum Zitat Carmichael MG, Zacher LL (2005) The demonstration of pulmonary neuroendocrine cell hyperplasia with tumorlets in a patient with chronic cough and a history of multiple medical problems. Mil Med 170(5):439–441CrossRefPubMed Carmichael MG, Zacher LL (2005) The demonstration of pulmonary neuroendocrine cell hyperplasia with tumorlets in a patient with chronic cough and a history of multiple medical problems. Mil Med 170(5):439–441CrossRefPubMed
18.
Zurück zum Zitat Carr LL, Chung JH, Achcar RD et al (2015) The clinical course of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. Chest 147(2):415–422CrossRefPubMed Carr LL, Chung JH, Achcar RD et al (2015) The clinical course of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. Chest 147(2):415–422CrossRefPubMed
19.
Zurück zum Zitat Cohen AJ, King TE Jr, Gilman LB et al (1998) High expression neutral endopeptidase in idiopathic diffuse hyperplasia of pulmonary neuroendocrine cells. Am J Respir Crit Care Med 158(5.1):1593–1599CrossRefPubMed Cohen AJ, King TE Jr, Gilman LB et al (1998) High expression neutral endopeptidase in idiopathic diffuse hyperplasia of pulmonary neuroendocrine cells. Am J Respir Crit Care Med 158(5.1):1593–1599CrossRefPubMed
20.
Zurück zum Zitat Gorshtein A, Gross DJ, Barak D et al (2012) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and the associated lung neuroendocrine tumors: clinical experience with a rare entity. Cancer 118(3):612–619CrossRefPubMed Gorshtein A, Gross DJ, Barak D et al (2012) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and the associated lung neuroendocrine tumors: clinical experience with a rare entity. Cancer 118(3):612–619CrossRefPubMed
21.
Zurück zum Zitat Lim C, Stanford D, Young I et al (2010) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a report of two cases. Pathol Int 60(7):538–541CrossRefPubMed Lim C, Stanford D, Young I et al (2010) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a report of two cases. Pathol Int 60(7):538–541CrossRefPubMed
22.
Zurück zum Zitat Miller RR, Muller NL (1995) Neuroendocrine cell hyperplasia and obliterative bronchiolitis in patients with peripheral carcinoid tumors. Am J Surg Pathol 19(6):653–658CrossRefPubMed Miller RR, Muller NL (1995) Neuroendocrine cell hyperplasia and obliterative bronchiolitis in patients with peripheral carcinoid tumors. Am J Surg Pathol 19(6):653–658CrossRefPubMed
23.
24.
Zurück zum Zitat Warth A, Herpel E, Schmahl A et al (2008) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) in association with an adenocarcinoma: a case report. J Med Case Rep 2:21PubMedCentralCrossRefPubMed Warth A, Herpel E, Schmahl A et al (2008) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) in association with an adenocarcinoma: a case report. J Med Case Rep 2:21PubMedCentralCrossRefPubMed
25.
Zurück zum Zitat Canessa PA, Santini D, Zanelli M et al (1997) Pulmonary tumorlets and microcarcinoids in bronchiectasis. Monaldi Arch Chest Dis 52(2):138–139PubMed Canessa PA, Santini D, Zanelli M et al (1997) Pulmonary tumorlets and microcarcinoids in bronchiectasis. Monaldi Arch Chest Dis 52(2):138–139PubMed
26.
Zurück zum Zitat Dwarakanath A, Kumar A (2013) A puzzling lady with persistent wheezing and pulmonary nodules. N Z Med J 126(1378):82–85PubMed Dwarakanath A, Kumar A (2013) A puzzling lady with persistent wheezing and pulmonary nodules. N Z Med J 126(1378):82–85PubMed
27.
Zurück zum Zitat McGuire AL, Maziak DE, Sekhon HS (2013) Diffuse intrapulmonary neuroendocrine cell hyperplasia. Can Respir J 20(6):406–408PubMedCentralPubMed McGuire AL, Maziak DE, Sekhon HS (2013) Diffuse intrapulmonary neuroendocrine cell hyperplasia. Can Respir J 20(6):406–408PubMedCentralPubMed
28.
Zurück zum Zitat Stenzinger A, Weichert W, Hensel M et al (2010) Incidental postmortem diagnosis of DIPNECH in a patient with previously unexplained ‘asthma bronchiale’. Pathol Res Pract 206(11):785–787CrossRefPubMed Stenzinger A, Weichert W, Hensel M et al (2010) Incidental postmortem diagnosis of DIPNECH in a patient with previously unexplained ‘asthma bronchiale’. Pathol Res Pract 206(11):785–787CrossRefPubMed
30.
Zurück zum Zitat Zhou H, Ge Y, Janssen B, 4 et al (2014) Double lung transplantation for diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. J Bronchology Interv Pulmonol 21:342–345CrossRefPubMed Zhou H, Ge Y, Janssen B, 4 et al (2014) Double lung transplantation for diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. J Bronchology Interv Pulmonol 21:342–345CrossRefPubMed
31.
Zurück zum Zitat Falkenstern-Ge RF, Kimmich M, Friedel G et al (2011) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: 7-year follow-up of a rare clinicopathologic syndrome. J Cancer Res Clin Oncol 137(10):1495–1498CrossRefPubMed Falkenstern-Ge RF, Kimmich M, Friedel G et al (2011) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: 7-year follow-up of a rare clinicopathologic syndrome. J Cancer Res Clin Oncol 137(10):1495–1498CrossRefPubMed
32.
Zurück zum Zitat Nassar AA, Jaroszewski DE, Helmers RA et al (2011) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a systematic overview. Am J Respir Crit Care Med 184(1):8–16CrossRefPubMed Nassar AA, Jaroszewski DE, Helmers RA et al (2011) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a systematic overview. Am J Respir Crit Care Med 184(1):8–16CrossRefPubMed
33.
Zurück zum Zitat Armas OA, White DA, Erlandson RA et al (1995) Diffuse idiopathic pulmonary neuroendocrine cell proliferation presenting as interstitial lung disease. Am J Surg Pathol 19(18):963–970CrossRefPubMed Armas OA, White DA, Erlandson RA et al (1995) Diffuse idiopathic pulmonary neuroendocrine cell proliferation presenting as interstitial lung disease. Am J Surg Pathol 19(18):963–970CrossRefPubMed
34.
Zurück zum Zitat Irshad S, McLean E, Rankin S et al (2010) Unilateral diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and multiple carcinoids treated with surgical resection. J Thorac Oncol 5(6):921–923CrossRefPubMed Irshad S, McLean E, Rankin S et al (2010) Unilateral diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and multiple carcinoids treated with surgical resection. J Thorac Oncol 5(6):921–923CrossRefPubMed
35.
Zurück zum Zitat Montoro Zulueta FJ, Martinez Prieto M, Verdugo Cartas MI et al (2012) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with multiple synchronous carcinoid tumors. Arch Bronchoneumol 48(12):472–475 Montoro Zulueta FJ, Martinez Prieto M, Verdugo Cartas MI et al (2012) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with multiple synchronous carcinoid tumors. Arch Bronchoneumol 48(12):472–475
36.
Zurück zum Zitat Oba H, Nishida K, Takeuchi S et al (2013) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with a central and peripheral carcinoid and multiple tumorlets: a case report emphasizing the role of neuropeptide hormones and human gonadotropin-alpha. Endocr Pathol 24(4):220–228CrossRefPubMed Oba H, Nishida K, Takeuchi S et al (2013) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with a central and peripheral carcinoid and multiple tumorlets: a case report emphasizing the role of neuropeptide hormones and human gonadotropin-alpha. Endocr Pathol 24(4):220–228CrossRefPubMed
37.
Zurück zum Zitat Adams H, Brack T, Kestenholz P et al (2006) Diffuse idiopathic neuroendocrine cell hyperplasia causing severe airway obstruction in a patient with a carcinoid tumor. Respiration 73(5):690–693CrossRefPubMed Adams H, Brack T, Kestenholz P et al (2006) Diffuse idiopathic neuroendocrine cell hyperplasia causing severe airway obstruction in a patient with a carcinoid tumor. Respiration 73(5):690–693CrossRefPubMed
38.
Zurück zum Zitat Cameron CM, Roberts F, Connell J et al (2011) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an unusual case of cyclical ectopic adrenocorticotrophic syndrome. Br J Radiol 84(997):e14–e17CrossRefPubMed Cameron CM, Roberts F, Connell J et al (2011) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an unusual case of cyclical ectopic adrenocorticotrophic syndrome. Br J Radiol 84(997):e14–e17CrossRefPubMed
39.
Zurück zum Zitat Coletta EN, Voss LR, Lima MS et al (2009) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia accompanied by airflow obstruction. J Bras Pneumol 35(5):489–494CrossRefPubMed Coletta EN, Voss LR, Lima MS et al (2009) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia accompanied by airflow obstruction. J Bras Pneumol 35(5):489–494CrossRefPubMed
40.
Zurück zum Zitat Johney EC, Pfannschmidt J, Rieker RJ et al (2006) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and a typical carcinoid tumor. J Thorac Cardiovasc Surg 131(5):1207–1208CrossRefPubMed Johney EC, Pfannschmidt J, Rieker RJ et al (2006) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and a typical carcinoid tumor. J Thorac Cardiovasc Surg 131(5):1207–1208CrossRefPubMed
41.
Zurück zum Zitat Ruffini E, Bongiovanni M, Cavallo A et al (2004) The significance of associated pre-invasive lesions in patients resected for primary lung neoplasms. Eur J Cardiothorac Surg 26(1):165–172CrossRefPubMed Ruffini E, Bongiovanni M, Cavallo A et al (2004) The significance of associated pre-invasive lesions in patients resected for primary lung neoplasms. Eur J Cardiothorac Surg 26(1):165–172CrossRefPubMed
42.
Zurück zum Zitat Rowan C, Hansell DM, Renzoni E et al (2012) Diffuse cystic lung disease of unexplained cause with coexistent small airway disease: a possible causal relationship? Am J Surg Pathol 36(2):228–234CrossRefPubMed Rowan C, Hansell DM, Renzoni E et al (2012) Diffuse cystic lung disease of unexplained cause with coexistent small airway disease: a possible causal relationship? Am J Surg Pathol 36(2):228–234CrossRefPubMed
43.
Zurück zum Zitat Anevlavis S, Cancellieri A, Livi V et al (2012) A 66-year-old woman with dry cough and exertional dyspnea. Chest 142(3):802–805CrossRefPubMed Anevlavis S, Cancellieri A, Livi V et al (2012) A 66-year-old woman with dry cough and exertional dyspnea. Chest 142(3):802–805CrossRefPubMed
44.
Zurück zum Zitat Dvorackova J, Macak J, Buzrla P (2013) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: case report and review of literature. Cesk Patol 49(2):99–102PubMed Dvorackova J, Macak J, Buzrla P (2013) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: case report and review of literature. Cesk Patol 49(2):99–102PubMed
45.
Zurück zum Zitat Fessler MB, Cool CD, Miller YE et al (2004) Idiopathic diffuse hyperplasia of pulmonary neuroendocrine cells in a patient with acromegaly. Respirology 9(2):274–277CrossRefPubMed Fessler MB, Cool CD, Miller YE et al (2004) Idiopathic diffuse hyperplasia of pulmonary neuroendocrine cells in a patient with acromegaly. Respirology 9(2):274–277CrossRefPubMed
46.
47.
Zurück zum Zitat Lee JS, Brown KK, Cool C et al (2002) Diffuse pulmonary neuroendocrine cell hyperplasia: radiologic and clinical features. J Comput Assist Tomogr 26(2):180–184CrossRefPubMed Lee JS, Brown KK, Cool C et al (2002) Diffuse pulmonary neuroendocrine cell hyperplasia: radiologic and clinical features. J Comput Assist Tomogr 26(2):180–184CrossRefPubMed
48.
Zurück zum Zitat Patel C, Tirukonda P, Bishop R et al (2012) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) masquerading as metastatic carcinoma with multiple pulmonary deposits. Clin Imaging 36(6):833–836CrossRefPubMed Patel C, Tirukonda P, Bishop R et al (2012) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) masquerading as metastatic carcinoma with multiple pulmonary deposits. Clin Imaging 36(6):833–836CrossRefPubMed
49.
Zurück zum Zitat Reyes LJ, Majó J, Perich D et al (2007) Neuroendocrine cell hyperplasia as an unusual form of interstitial lung disease. Respir Med 101(8):1840–1843CrossRefPubMed Reyes LJ, Majó J, Perich D et al (2007) Neuroendocrine cell hyperplasia as an unusual form of interstitial lung disease. Respir Med 101(8):1840–1843CrossRefPubMed
50.
Zurück zum Zitat Mireskandari M, Abdirad A, Zhang Q et al (2013) Association of small foci of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) with adenocarcinoma of the lung. Pathol Res Prac 209(9):578–584CrossRef Mireskandari M, Abdirad A, Zhang Q et al (2013) Association of small foci of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) with adenocarcinoma of the lung. Pathol Res Prac 209(9):578–584CrossRef
51.
Zurück zum Zitat Gosney JR, Williams IJ, Dodson AR et al (2011) Morphology and antigen expression profile of pulmonary neuroendocrine cells in reactive proliferations and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). Histopathology 59(4):751–762CrossRefPubMed Gosney JR, Williams IJ, Dodson AR et al (2011) Morphology and antigen expression profile of pulmonary neuroendocrine cells in reactive proliferations and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). Histopathology 59(4):751–762CrossRefPubMed
Metadaten
Titel
Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence
verfasst von
Eric Wirtschafter
Ann E. Walts
Sandy T. Liu
Alberto M. Marchevsky
Publikationsdatum
01.10.2015
Verlag
Springer US
Erschienen in
Lung / Ausgabe 5/2015
Print ISSN: 0341-2040
Elektronische ISSN: 1432-1750
DOI
https://doi.org/10.1007/s00408-015-9755-1

Weitere Artikel der Ausgabe 5/2015

Lung 5/2015 Zur Ausgabe

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.