![]() WHO classification of tumours of the lung, pleura, thymus and heart. In: Travis WD, Brambilla E, Burke AP, et al., editors. Gosney J, Austin JHM, Jett J, et al (2015) Diffuse pulmonary neuroendocrine cell hyperplasia. Rossi G, Cavazza A, Spagnolo P, Sverzellati N, Longo L, Jukna A, Montanari G, Carbonelli C, Vincenzi G, Bogina G, Franco R, Tiseo M (2016) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome. Written informed patient consent for publication has been obtained.Īguayo S, Miller Y, Waldron J, Bogin R, Sunday M, Staton G, Beam W, King T (1992) Idiopathic Diffuse Hyperplasia of Pulmonary Neuroendocrine Cells and Airways Disease. Giving intravenous contrast is a simple step to differentiate carcinoid on background DIPNECH from bronchoceles on background small airways disease/asthma or allergic bronchopulmonary aspergillosis. Ultimately histological sampling of larger nodules/tumours is required to confirm carcinoid. The radiologist may be first to suggest the diagnosis. When carcinoid is present, surgical resection is the treatment of choice for localised disease.Īlthough rare, DIPNECH is being increasingly described with typical clinical and radiological findings, especially when mosaicism and either random nodules or endobronchial tumours are present. CT follow up is generally accepted given risk of developing carcinoid tumours. Somatostatin analogues and inhaled corticosteroids have been used with varying success to treat small airways disease. No consensus treatment or follow-up protocols exist due to rarity. S omatostatin receptor-PET demonstrates higher sensitivity and specificity Gallium-68 DOTATATE Octreotide-PET/CT being the evolving modality that out-performs traditional Octreotide scintigraphy, although is limited by centre availability. Fluorodeoxyglucose-positron emission tomography (FDG-PET) is of limited utility (low-moderate uptake, ~25% false-negative rate). If present, calcification is coarse and eccentric. Carcinoid tumours are smoothly defined, rounded/lobulated, <5cm and homogenously enhance due to increased vascularity. When present, pulmonary carcinoid tumours are usually central and may be multiple. Bronchial wall thickening, mucus plugging/impaction and bronchiectasis are also reported. HRCT findings include mosaic attenuation due to constrictive bronchiolitis causing air-trapping and small (<5mm) random pulmonary nodules (absence of nodularity does not exclude DIPNECH). When present, pulmonary carcinoid tumours are divided by location into central or peripheral the former more symptomatic due to the sequelae of airway obstruction. Imaging plays an important role due to non-specific clinical features, although often cases are initially misdiagnosed. ![]() Pulmonary function tests demonstrate an obstructive or mixed obstructive/restrictive pattern, although can be normal. Middle-aged females with a history of chronic cough or asthma. Neuroendocrine aggregates are defined by size 5mm (carcinoid). Neuroendocrine cells confined to airway mucosa proliferate into aggregates that may extend through the basement membrane to cause constrictive bronchiolitis. The World Health Organisation defines DIPNECH as a preinvasive condition on a spectrum of neuroendocrine cells proliferation and a precursor to carcinoid tumour. No lymphadenopathy or metastases below the diaphragm.ġ8-gauge CT-guided coaxial biopsy of the largest mass yielded carcinoid tumour.ĭiffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH ) is a rare pulmonary condition first described in 1992 since confined to case reports and small case series. No extra-thoracic primary tumour identified. Nodules in the middle lobe, left upper and lower lobes also enhance, some avidly. Post-contrast CT thorax/abdomen/pelvis: The right upper lobe pulmonary mass demonstrates heterogeneous enhancement (68 HU) with some patchy areas of less marked enhancement anteriorly (figure 5). Multiple other airways are also distally plugged. The mass described is airway-centric, with thickening of the proximal airway (figure 3) and plugging of the distal airway (figure 4). There are multiple peribronchovascular and endobronchial smoothly lobulated solid nodules and masses throughout both mid and lower zones, the largest in the anterior segment of the right upper lobe measures 33mm with a density of 32 Hounsfield Units (HU) (figures 2 & 3). High Resolution Computed Tomography (HRCT): There is widespread mosaicism with geographic air-trapping, exacerbated in expiration (figure 1).
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