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Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): What Is It and What Should You Know?
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A7348 - Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): What Is It and What Should You Know?
Author Block: A. Schertz1, K. C. Alonso2, A. B. Chatterjee3; 1Internal Medicine, Wake Forest University Baptist Medical Center, Winston-Salem, NC, United States, 2Pulmonary and Critical Care Medicine, Winston Salem, NC, United States, 3WFUHS Pulm/CCM, Wake Forest Univ Sch of Med, Winston Salem, NC, United States.
INTRODUCTION: Within the spectrum of pulmonary carcinoid tumor is the distinct entity: diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). It is defined as a proliferation of scattered pulmonary neuroendocrine cells, small nodules of neuroendocrine bodies or a linear proliferation of neuroendocrine cells. The earliest descriptions of DIPNECH were thought to represent in situ carcinoma, however, it became apparent that these lesions infrequently progressed to carcinoid tumor. There is a general paucity of data, with approximately 100 patients described in medical literature as of 2016. Our goal is to further characterize DIPNECH as a distinct entity from carcinoid tumor, identify common findings seen in DIPNECH patients, and discuss the clinical course of the disease. METHODS: Retrospective review of consecutive patients seen at Wake Forest Baptist University Medical Center through 2016. RESULTS: Seven patients in our sample met diagnostic criteria for DIPNECH, 100% were women. The mean age at diagnosis was 52 ± 11 years. Five were white (71%) and 2 were black (29%). Six of the patients were non-smokers (86%). The mean time since diagnosis was 8.71 ± 4.61 years. Six patients met criteria for obesity (86%), mean BMI was 36.40 (IQR 33.90 to 39.20). The most common presenting symptoms were cough and subjective dyspnea (86%). CT imaging of the chest showed mosaic attenuation (100%), air trapping (71%), and multiple nodules (86%). Bilateral nodules were seen in 5 patients (71%). Six patients (86%) were diagnosed with carcinoid tumor concurrently with DIPNECH and/or carcinoid tumorlets on biopsy. Histology for all 7 patients (100%) had features of typical carcinoid. Four (57%) underwent resection, 2 (29%) received only surveillance, 2 (29%) were treated with octreotide, and 1 (14%) was treated with systemic chemotherapy. There was no progression of disease on imaging following diagnosis. No extra-pulmonary metastases were found. CONCLUSIONS: Distinguishing DIPNECH from pulmonary carcinoid is important because it can mean the difference between a diagnosis of stage IV lung cancer, and the distinct, pre-malignant entity of DIPNECH. Further recognition and study of the DIPNECH population is still required to elucidate whether a true risk of conversion from DIPNECH to pulmonary carcinoid exists.
Author Block: A. Schertz1, K. C. Alonso2, A. B. Chatterjee3; 1Internal Medicine, Wake Forest University Baptist Medical Center, Winston-Salem, NC, United States, 2Pulmonary and Critical Care Medicine, Winston Salem, NC, United States, 3WFUHS Pulm/CCM, Wake Forest Univ Sch of Med, Winston Salem, NC, United States.
INTRODUCTION: Within the spectrum of pulmonary carcinoid tumor is the distinct entity: diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). It is defined as a proliferation of scattered pulmonary neuroendocrine cells, small nodules of neuroendocrine bodies or a linear proliferation of neuroendocrine cells. The earliest descriptions of DIPNECH were thought to represent in situ carcinoma, however, it became apparent that these lesions infrequently progressed to carcinoid tumor. There is a general paucity of data, with approximately 100 patients described in medical literature as of 2016. Our goal is to further characterize DIPNECH as a distinct entity from carcinoid tumor, identify common findings seen in DIPNECH patients, and discuss the clinical course of the disease. METHODS: Retrospective review of consecutive patients seen at Wake Forest Baptist University Medical Center through 2016. RESULTS: Seven patients in our sample met diagnostic criteria for DIPNECH, 100% were women. The mean age at diagnosis was 52 ± 11 years. Five were white (71%) and 2 were black (29%). Six of the patients were non-smokers (86%). The mean time since diagnosis was 8.71 ± 4.61 years. Six patients met criteria for obesity (86%), mean BMI was 36.40 (IQR 33.90 to 39.20). The most common presenting symptoms were cough and subjective dyspnea (86%). CT imaging of the chest showed mosaic attenuation (100%), air trapping (71%), and multiple nodules (86%). Bilateral nodules were seen in 5 patients (71%). Six patients (86%) were diagnosed with carcinoid tumor concurrently with DIPNECH and/or carcinoid tumorlets on biopsy. Histology for all 7 patients (100%) had features of typical carcinoid. Four (57%) underwent resection, 2 (29%) received only surveillance, 2 (29%) were treated with octreotide, and 1 (14%) was treated with systemic chemotherapy. There was no progression of disease on imaging following diagnosis. No extra-pulmonary metastases were found. CONCLUSIONS: Distinguishing DIPNECH from pulmonary carcinoid is important because it can mean the difference between a diagnosis of stage IV lung cancer, and the distinct, pre-malignant entity of DIPNECH. Further recognition and study of the DIPNECH population is still required to elucidate whether a true risk of conversion from DIPNECH to pulmonary carcinoid exists.
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