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• W3081698207 abstract "Case PresentationA 66-year-old woman with no significant medical history presented initially to her primary care physician’s office with a 2-weeks history of productive cough and associated wheezing after cough paroxysms. Empiric antibiotic was started, with no improvement. Chest radiograph was performed, which showed bilateral nodular opacities (Fig 1). The patient’s cough resolved in 6 weeks after empiric treatment with oral steroids, inhaled steroids, and bronchodilators. A follow-up chest radiograph done at 6 weeks showed persistent abnormalities, and she was referred for a pulmonary evaluation. She denied any fevers, cough, hemoptysis, shortness of breath, wheezing, loss of appetite, or weight loss at that time. She had no significant medical problems and was not on any medications at that time. She was a nonsmoker. She worked in an office setting and denied any occupational or recreational exposures or recent travels. There was no family history of lung diseases or cancer. A 66-year-old woman with no significant medical history presented initially to her primary care physician’s office with a 2-weeks history of productive cough and associated wheezing after cough paroxysms. Empiric antibiotic was started, with no improvement. Chest radiograph was performed, which showed bilateral nodular opacities (Fig 1). The patient’s cough resolved in 6 weeks after empiric treatment with oral steroids, inhaled steroids, and bronchodilators. A follow-up chest radiograph done at 6 weeks showed persistent abnormalities, and she was referred for a pulmonary evaluation. She denied any fevers, cough, hemoptysis, shortness of breath, wheezing, loss of appetite, or weight loss at that time. She had no significant medical problems and was not on any medications at that time. She was a nonsmoker. She worked in an office setting and denied any occupational or recreational exposures or recent travels. There was no family history of lung diseases or cancer. On physical examination, the patient was well-appearing and in no distress. Her vital signs were within normal limits. No pallor, icterus, or cyanosis was present. No jugular vein distention or cervical lymphadenopathy was noted. Lungs were clear to auscultation. No wheezing was noted. Results of the rest of the examination were normal. A chest CT scan was obtained, which showed multiple nodules bilaterally, with the largest measuring approximately 1 cm in diameter. In addition, there were scattered areas of mosaic attenuation, consistent with air trapping (Fig 2). Pulmonary function tests (PFTs) revealed mild obstruction without bronchodilator responsiveness, with no restriction and normal diffusion capacity. A thoracoscopic lung biopsy was performed. Pathology revealed multiple foci of proliferation of neuroendocrine cells and nests of neuroendocrine cells of varying sizes, without lymphovascular invasion (Fig 3A). Immunostaining was positive for chromogranin A (Fig 3B), cytokeratin (Fig 3C), thyroid transcription factor-1 (Fig 3D), and synaptophysin.Figure 3Multiple foci of proliferation of neuroendocrine cells and nests of neuroendocrine cells without lymphovascular invasion (A). Immunostaining shows positivity for chromogranin A (B), cytokeratin (C), TTF-1 (D). TTF-1 = Thyroid transcription factor -1.View Large Image Figure ViewerDownload Hi-res image Download (PPT) What is the diagnosis? What other diagnostic testing is necessary for further evaluation of this condition? Diagnosis: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, carcinoid tumorlets, carcinoid tumors, occurring concurrently Other diagnostic tests: Other diagnostic tests that should be performed as part of evaluation of this condition include serum levels of chromogranin A, adrenocorticotrophic hormone, serotonin, and urinary 5-hydroxyindole acetic acid, all of which may be secreted by the neuroendocrine cells. These levels were normal in this patient. Octreotide scintigraphy should be performed for localization of metastases; this was negative in this case as well. Pulmonary neuroendocrine cells (PNC) are specialized epithelial cells in the respiratory tract. Neuroendocrine hyperplasia as a primary pathologic process, independent of other lung abnormalities, has been identified in the last 2 decades. Proliferation of neuroendocrine cells in the bronchial or bronchiolar mucosa, without extension beyond the basement membrane, is referred to as diffuse idiopathic neuroendocrine cell hyperplasia (DIPNECH). When these neuroendocrine cells extend beyond the basement membrane to form collections smaller than 5 mm, they are referred to as carcinoid tumorlets. Once the proliferating neuroendocrine cells form nodules larger than 5 mm, they constitute carcinoid tumors. The World Health Organization classified DIPNECH as a preneoplastic condition for carcinoid tumors of the lung, based on the spectrum of multiple neuroendocrine lesions occurring in the same patients. DIPNECH is reported to occur with variable clinical, imaging, and histological features. There are no standardized clinicopathologic criteria for diagnosis. There are distinguishing histologic features between DIPNECH and carcinoid tumors. DIPNECH is believed to represent the precursor lesion with continued progression to carcinoid tumorlets and tumors. Of note, carcinoids can occur without any precursor lesions in the same biopsy specimen, and the reverse can be true. In a recent case series of 18 patients, 100% of patients with DIPNECH had carcinoid tumorlets, and 28% had typical carcinoid tumors. DIPNECH typically presents in the fifth or sixth decade of life. Most patients are asymptomatic, but some may present with insidious cough or slowly progressive dyspnea. Women are more commonly affected. Reported findings on chest CT in DIPNECH include pulmonary nodules, ground-glass opacities, mosaic attenuation, bronchial wall thickening, and rarely, bronchiectasis. Air trapping and mosaic attenuation in DIPNECH result from obstruction of distal airways caused by fibrosis resulting from neuropeptides secreted by the PNCs or by PNCs causing physical obstruction. Most studies report an obstruction on PFTs, but normal and restrictive patterns also have been reported. Tissue biopsy is required for diagnosis. Surgical biopsy is preferred because of the need for large tissue sample for identifying neuroendocrine hyperplasia. The histopathological appearance may comprise a generalized or linear proliferation of scattered PNC or small nodules. Although diffuse, DIPNECH affects lung bases more severely. DIPNECH-related carcinoid tumors differ in some characteristics from other pulmonary carcinoid tumors. These are usually peripheral locations, whereas most carcinoids not related to DIPNECH are central. Typical carcinoids have a mitotic rate of two or fewer mitoses per 10 high-power fields with no necrosis, whereas atypical carcinoids, in contrast, have a mitotic rate of two to 10 mitoses per 10 high-power fields or the presence of necrosis. PNC hyperplasia may be associated with constrictive bronchiolitis on histopathology, often with surrounding emphysematous change. Most cases of DIPNECH may show progression to carcinoid tumorlets and tumors. After tissue diagnosis is confirmed, serum levels of peptides secreted by PNCs such as chromogranin A (CgA), serotonin, adrenocorticotrophic hormone, and urinary 5-hydroxyindole acetic acid should be measured. Staging of lung carcinoids with octreotide scintigraphy is required for evaluating distant metastases. Limited data exist regarding disease progression of DIPNECH, and there is no known treatment of DIPNECH. Reported treatments vary from conservative management, oral and inhaled steroids, somatostatin analogs, and chemotherapy to surgical lung resection, and even lung transplantation. Obstruction in DIPNECH is treated with bronchodilators and inhaled steroids. Octreotide, a somatostatin analog, reduces the hormonal hypersecretion of PNCs in carcinoid tumors. Octreotide therapy is reported to improve cough and lower serum CgA levels, but did not improve PFTs. Treatment of DIPNECH-associated carcinoid tumors consists of resection of the tumor associated with mediastinal and hilar lymphadenectomy. Resection of only the dominant lesion also has been reported. Despite these treatments, 24% to 55% of patients have a clinically and radiologically progressive disease in which the use of somatostatin analogs could stem the progression. Close follow-up of patients is essential, but there are currently no specific guidelines. The patient was asymptomatic at the time of evaluation; hence, her condition was managed conservatively. She has been followed up with CT scans every 6 months which have remained stable, so no interventions have been necessary. She developed exertional dyspnea on follow-up 2 years after diagnosis, with PFTs showing obstruction. The patient was started on inhaled steroids and bronchodilator therapy as needed with relief of symptoms. She continues to do well, 4 years after diagnosis. 1.DIPNECH, a condition characterized by proliferation of PNCs in the distal airways, is a precursor to the development of pulmonary carcinoid tumors. It represents an indolent malignant process, with progression occurring in up 50% of cases.2.It can occur as multiple small pulmonary nodules or may occur concurrently with carcinoid tumorlets and tumors, appearing as pulmonary nodules of varying sizes.3.Besides nodules, chest CT may show areas of mosaic attenuation from air trapping because of obstruction of distal airways caused by chemical mediators secreted by PNCs in DIPNECH. PFTs may show obstruction.4.Evaluation of DIPNECH with carcinoid tumors should include serum CgA, adrenocorticotrophic hormone, serotonin, and urinary 5-hydroxyindole acetic acid, all of which may be secreted by the PNCs. Octreotide scintigraphy should be performed for localization of extrathoracic metastases.5.No specific guidelines exist for the treatment of DIPNECH or DIPNECH with multiple pulmonary carcinoid tumors.6.Inhaled/systemic steroids, bronchodilators, somatostatin analogs, chemotherapy, and surgical lung resection have been reported. DIPNECH, with concurrent carcinoid tumors with progressive disease and symptoms, should be treated with somatostatin-analog octreotide. Financial/nonfinancial disclosures: None declared. Other contributions: CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met." @default.
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• W3081698207 title "An Older Woman With Transient Cough, Mild Airway Obstruction, and Lung Nodules" @default.
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• W3081698207 doi "https://doi.org/10.1016/j.chest.2020.05.540" @default.
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