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| {{Infobox medical condition (new) | | {{Infobox medical condition |
| | name = Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia | | | name = Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia |
| | synonyms = '''DIPNECH''' | | | synonyms = DIPNECH |
| | image = | | | image = |
| | alt =
| | | caption = |
| | caption = | | | field = [[Pulmonology]] |
| | pronounce =
| | | symptoms = [[Cough]], [[dyspnea]], [[wheezing]] |
| | field = [[Respirology]] | | | complications = [[Obstructive lung disease]], [[carcinoid tumors]] |
| | symptoms = | | | onset = Middle-aged adults |
| | complications = | | | duration = Chronic |
| | onset = | | | causes = Unknown |
| | duration = | | | risks = |
| | types =
| | | diagnosis = [[High-resolution computed tomography]], [[lung biopsy]] |
| | causes = | | | differential = [[Asthma]], [[chronic obstructive pulmonary disease]], [[bronchitis]] |
| | risks = | | | treatment = [[Corticosteroids]], [[bronchodilators]], [[surgery]] |
| | diagnosis = | | | medication = |
| | differential = | | | prognosis = Variable, can be progressive |
| | prevention =
| | | frequency = Rare |
| | treatment = | |
| | medication = | |
| | prognosis = | |
| | frequency = | |
| | deaths =
| |
| }} | | }} |
| | | {{Short description|A rare lung condition involving neuroendocrine cell proliferation}} |
| '''Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia''' ('''DIPNECH''') is a diffuse [[Parenchyma|parenchymal]] lung disease which often presents with symptoms of cough and shortness of breath. The pathological definition published by the [[World Health Organization]] is “a generalized [[Cell growth|proliferation]] of scattered single cells, small [[Nodule (medicine)|nodules]] (neuroendocrine bodies), or linear proliferations of [[Pulmonary neuroendocrine cell|pulmonary neuroendocrine]] (PNE) cells that may be confined to the [[Bronchus|bronchial]] and bronchiolar epithelium.”<ref name="Travis 2004 Book">{{cite book|last1=Travis|first1=WD|title=World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart.|date=2004|publisher=IARC Press|location=Lyon, France}}</ref> The true prevalence of this disease is not known. To date, just under 200 cases have been reported in the literature.<ref name="Wirstschafter 26104490">{{cite journal|last1=Wirstschafter|first1=E|last2=Walts|first2=A|last3=Marchevsky|first3=A|title=Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia of the lung (DIPNECH): current best evidence|journal=Lung|date=2012|volume=193|issue=5|pages=659–67|pmid=26104490|doi=10.1007/s00408-015-9755-1}}</ref> However, with an increase in recognition of this disease by radiologists and pulmonologists, the number of cases has been increasing. DIPNECH predominantly affects middle-aged women<ref name="Gorshtein 21751183">{{cite journal|last1=Gorshtein|first1=A|last2=Gross|first2=DJ|last3=Barak|first3=D|last4=Strenov|first4=Y|last5=Refaeli|first5=Y|last6=Shimon|first6=I|last7=Grozinsky-Glasberg|first7=S|title=Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and the associated lung neuroendocrine tumors: clinical experience with a rare entity|journal=Cancer|date=2012|volume=118|issue=3|pages=612–619|pmid=21751183|doi=10.1002/cncr.26200}}</ref> with slowly progressive lung obstruction. DIPNECH is usually discovered in one of two ways: 1) as an unexpected finding following a lung surgery; or 2) by evaluation of a patient in a pulmonary clinic with longstanding, unexplained symptoms.<ref name="Davies 17099078">{{cite journal|last1=Davies|first1=SJ|last2=Gosney|first2=JR|last3=Hansell|first3=DM|last4=Wells|first4=AU|last5=du Bois|first5=RM|last6=Burke|first6=MM|last7=Sheppard|first7=MN|last8=Nicholson|first8=AG|title=Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognized spectrum of disease|journal=Thorax|date=2007|volume=62|issue=3|pages=248–252|pmid=17099078|doi=10.1136/thx.2006.063065|pmc=2117154}}</ref> | | '''Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia''' (DIPNECH) is a rare pulmonary disorder characterized by the proliferation of [[neuroendocrine cells]] within the [[lungs]]. This condition is considered a precursor to [[neuroendocrine tumors]] and is often associated with [[obstructive lung disease]]. |
| | | ==Pathophysiology== |
| ==Signs and symptoms== | | DIPNECH involves the diffuse proliferation of neuroendocrine cells in the [[bronchiolar epithelium]]. These cells can form small nodules known as [[tumorlets]], which are less than 5 mm in diameter. The condition is thought to be a precursor to [[carcinoid tumors]], which are a type of [[neuroendocrine tumor]]. The exact cause of DIPNECH is unknown, but it is considered idiopathic, meaning it arises spontaneously without a known cause. |
| About 20% of DIPNECH patients are symptom free at the time they first present. The most common symptoms include:<ref name="Adams 16131792">{{cite journal|last1=Adams|first1=H|last2=Brack|first2=T|last3=Kestenholz|first3=P|last4=Vogt|first4=P|last5=Steinert|first5=HC|last6=Russi|first6=EW|title=Diffuse idiopathic neuroendocrine cell hyperplasia causing severe airway obstruction in a patient with a carcinoid tumor|journal=Respiration|date=2006|volume=73|issue=5|pages=690–693|pmid=16131792|doi=10.1159/000088007}}</ref><ref name="Armas 7611544">{{cite journal|last1=Armas|first1=OA|last2=White|first2=DA|last3=Erlandson|first3=RA|last4=Rosai|first4=J|title=Diffuse idiopathic pulmonary neuroendocrine cell proliferation presenting as interstitial lung disease|journal=Am J Surg Pathol|date=1995|volume=19|issue=8|pages=963–970|pmid=7611544|doi=10.1097/00000478-199508000-00013}}</ref><ref name="Aubry 17400673">{{cite journal|last1=Aubry|first1=MC|last2=Thomas|first2=CF Jr|last3=Jett|first3=JR|last4=Swensen|first4=SJ|last5=Myers|first5=JL|title=Significance of multiple carcinoid tumors and tumorlets in surgical lung specimens: analysis of 28 patients|journal=Chest|date=2007|volume=131|issue=6|pages=1635–1643|pmid=17400673|doi=10.1378/chest.06-2788}}</ref>
| | ==Clinical Presentation== |
| | | Patients with DIPNECH often present with symptoms of [[chronic cough]], [[dyspnea]] (shortness of breath), and sometimes [[wheezing]]. These symptoms are due to the obstruction of airways by the proliferating neuroendocrine cells and tumorlets. In some cases, patients may be asymptomatic, and the condition is discovered incidentally during imaging studies for other reasons. |
| * Chronic cough
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| * Shortness of breath or [[dyspnea]] when exercising or exerting one's self
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| *[[Wheeze|Wheezing]] (less frequent)
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| *[[Hemoptysis]] (Infrequent)
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| | |
| Symptoms may be present for many years prior to diagnosis and are often ascribed to other lung conditions. Erroneous initial diagnoses of [[asthma]] or [[chronic obstructive pulmonary disease]] often are made in patients with DIPNECH.<ref name="Carr 25275948">{{cite journal|last1=Carr|first1=LL|last2=Chung|first2=JH|last3=Duarte Achcar|first3=R|last4=Lesic|first4=Z|last5=Rho|first5=JY|last6=Yagihashi|first6=K|last7=Tate|first7=RM|last8=Swigris|first8=JJ|last9=Kern|first9=JA|title=The clinical course of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia|journal=Chest|date=2015|volume=147|issue=2|pages=415–422|pmid=25275948|doi=10.1378/chest.14-0711}}</ref>
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| ==Diagnosis== | | ==Diagnosis== |
| The major criterion for diagnosis is typically a confirmed surgical [[biopsy]]. Minor diagnostic criteria have been proposed for DIPNECH.<ref name="Carr 25275948" /> | | The diagnosis of DIPNECH is typically made through a combination of clinical evaluation, imaging studies, and histopathological examination. [[High-resolution computed tomography]] (HRCT) of the chest may reveal multiple small nodules and areas of air trapping. A [[lung biopsy]] is often required to confirm the diagnosis, showing the characteristic proliferation of neuroendocrine cells and the presence of tumorlets. |
| | |
| * Clinical presentation: woman, between the age of 45 and 67 with cough and/or shortness of breath for 5–10 years
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| * Pulmonary function: increased [[Lung volumes|residual volume]], increased total lung capacity, fixed obstruction, low [[Diffusing capacity for carbon monoxide|diffusing capacity]] of the lung for carbon monoxide that corrects with alveolar volume
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| * High-resolution CT scan: diffuse pulmonary nodules 4–10 mm, greater than 20 nodules, mosaic [[attenuation]] or [[air trapping]] in greater than 50% of the lung
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| * Transbronchial biopsy: proliferation of pulmonary neuroendocrine cells
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| *[[Serum (blood)|Serum]] markers: elevated serum [[chromogranin A]] levels
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| | |
| ===Pathology===
| |
| In most DIPNECH cases, upon examination of the lung tissue, the overgrowth of pulmonary neuroendocrine cells is seen along the small airways, with extension through the [[basement membrane]] of the bronchiolar epithelium leading to formation of carcinoid tumorlets. When the tumorlets become greater than 5mm in size they are considered bronchial carcinoids. Upon microscopic examination, the PNE cells have round, oval, or spindle nuclei with [[salt-and-pepper chromatin]] and clear or eosinophilic cytoplasm.<ref name="Travis 2004 Book" />
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| | |
| Although no formal definition exists regarding the extent of PNE [[hyperplasia]] necessary for a DIPNECH diagnosis, this process is often seen throughout the small airways. Because the hyperplasia of PNE cells can be seen as a reaction to chronic lung disease, surrounding solitary bronchial carcinoids and [[adenocarcinoma]] of the lung, these causes must be excluded prior to a DIPENCH diagnosis.<ref name="Travis 2004 Book" />
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| Obstructive bronchiolitis has been reported as a characteristic [[Histopathology|histopathologic]] finding in patients with DIPNECH.<ref name="Carr 25275948" /> The bronchiolitis is thought to be a response of the small airways to neuropeptides secreted by the PNE cells.
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| | |
| ===Imaging===
| |
| The findings on chest imaging in DIPNECH patients are bilateral and diffuse. The most frequent findings on a computed tomography (CT) of the chest are multiple primary nodules and/or masses, on a background of mosaic attenuation and airway wall thickening.<ref name="Wirstschafter 26104490" /><ref name="Lee 11884770">{{cite journal|last1=Lee|first1=JS|last2=Brown|first2=KK|last3=Cool|first3=C|title=Diffuse pulmonary neuroendocrine cell hyperplasia: radiologic and clinical features|journal=J Comput Assist Tomogr|date=2002|volume=26|issue=2|pages=180–184|pmid=11884770|doi=10.1097/00004728-200203000-00003}}</ref>
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| The nodules have an indolent pattern of growth and are found throughout the lungs. The nodules are typically rounded and well-defined. Upon surgical resection, histologically the nodules are found to be typical carcinoids or carcinoid tumorlets depending on size.
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| ===Pulmonary function studies===
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| Although some patients present with normal lung function, pulmonary function tests generally demonstrate fixed airway obstruction with a decreased FEV1 and reduced [[FEV1/FVC ratio|FEV1/FVC]] ratio without [[bronchodilator]] response. Air trapping is common and leads to increased residual volumes. As the disease progresses, a mixed pattern of obstruction and restriction may develop. In general the obstructive lung disease is slowly progressive with periods of stability.<ref name="Carr 25275948" />
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| ==Treatment== | | ==Treatment== |
| To date there have been no clinical trials to determine effective treatment for this disease. Some patients have been treated with [[somatostatin]] analogs. Although the cough associated with DIPNECH tends to diminish on this treatment, improvement in pulmonary function has not been clearly demonstrated.<ref name="Wirstschafter 26104490" /><ref name="Carr 25275948" /> There are also reports of symptomatic treatment with long- and short-acting beta agonists. Although [[Steroid|steroids]], both oral and inhaled, have been used in the setting of DIPNECH, there is no clear improvement with this treatment.
| | There is no standard treatment for DIPNECH, and management is often tailored to the individual patient. Options may include observation for asymptomatic patients, [[bronchodilators]] and [[corticosteroids]] for symptomatic relief, and in some cases, surgical resection of larger lesions. The use of [[somatostatin analogs]] has been explored in some cases to control symptoms and slow disease progression. |
| | |
| It is not uncommon for typical carcinoids to arise within DIPNECH. Due to presence of these tumors, DIPNECH is classified as a pre-malignant condition.<ref name="Travis 2004 Book" /> Although there have been reports of atypical carcinoids with local [[lymph node]] involvement, there are no reports of more aggressive neuroendocrine tumors, such as large cell neuroendocrine or small cell lung cancer, associated with DIPNECH.<ref name="Gorshtein 21751183" /> When isolated bronchial carcinoids are diagnosed, oncology guidelines recommend surgical resection with lymph node sampling.<ref name="NCCN">{{cite book|title=National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Small Cell Lung Cancer Version 2.2014}}</ref> However, as multiple carcinoids may develop in the setting of DIPNECH, a more conservative approach is often considered to preserve lung function.
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| ==Prognosis== | | ==Prognosis== |
| The morbidity associated with DIPNECH is due to the associated obstructive lung disease. The lung disease tends to be slowly progressive, but given enough time can lead to significant disability and require supplemental oxygen therapy.<ref name="Carr 25275948" /> There have been reports of lung transplantation in the setting of end-stage DIPNECH.<ref name="Sheerin 7701466">{{cite journal|last1=Sheerin|first1=N|last2=Harrison|first2=NK|last3=Sheppard|first3=MN|last4=Hansell|first4=DM|last5=Yacoub|first5=M|last6=Clark|first6=TJ|title=Obliterative bronchiolitis caused by multiple tumorlets and microcarcinoids successfully treated by single lung transplantation|journal=Thorax|date=1995|volume=50|issue=2|pages=207–209|pmid=7701466|doi=10.1136/thx.50.2.207|pmc=473927}}</ref> | | The prognosis for patients with DIPNECH varies. Some patients may experience stable disease with minimal symptoms, while others may progress to develop more significant obstructive lung disease or [[neuroendocrine tumors]]. Regular follow-up with pulmonary function tests and imaging studies is often recommended to monitor disease progression. |
| | | ==Related pages== |
| ==References==
| | * [[Neuroendocrine tumor]] |
| {{Reflist}}
| | * [[Carcinoid tumor]] |
| | | * [[Obstructive lung disease]] |
| [[Category:Lung disorders]] | | * [[Chronic cough]] |
| [[Category:Neuroendocrinology]] | | [[Category:Pulmonary diseases]] |
| {{dictionary-stub1}}
| | [[Category:Rare diseases]] |
| {{No image}}
| | [[Category:Neuroendocrine tumors]] |
| {{No image}}
| |
A rare lung condition involving neuroendocrine cell proliferation
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disorder characterized by the proliferation of neuroendocrine cells within the lungs. This condition is considered a precursor to neuroendocrine tumors and is often associated with obstructive lung disease.
Pathophysiology[edit]
DIPNECH involves the diffuse proliferation of neuroendocrine cells in the bronchiolar epithelium. These cells can form small nodules known as tumorlets, which are less than 5 mm in diameter. The condition is thought to be a precursor to carcinoid tumors, which are a type of neuroendocrine tumor. The exact cause of DIPNECH is unknown, but it is considered idiopathic, meaning it arises spontaneously without a known cause.
Clinical Presentation[edit]
Patients with DIPNECH often present with symptoms of chronic cough, dyspnea (shortness of breath), and sometimes wheezing. These symptoms are due to the obstruction of airways by the proliferating neuroendocrine cells and tumorlets. In some cases, patients may be asymptomatic, and the condition is discovered incidentally during imaging studies for other reasons.
Diagnosis[edit]
The diagnosis of DIPNECH is typically made through a combination of clinical evaluation, imaging studies, and histopathological examination. High-resolution computed tomography (HRCT) of the chest may reveal multiple small nodules and areas of air trapping. A lung biopsy is often required to confirm the diagnosis, showing the characteristic proliferation of neuroendocrine cells and the presence of tumorlets.
Treatment[edit]
There is no standard treatment for DIPNECH, and management is often tailored to the individual patient. Options may include observation for asymptomatic patients, bronchodilators and corticosteroids for symptomatic relief, and in some cases, surgical resection of larger lesions. The use of somatostatin analogs has been explored in some cases to control symptoms and slow disease progression.
Prognosis[edit]
The prognosis for patients with DIPNECH varies. Some patients may experience stable disease with minimal symptoms, while others may progress to develop more significant obstructive lung disease or neuroendocrine tumors. Regular follow-up with pulmonary function tests and imaging studies is often recommended to monitor disease progression.
Related pages[edit]
