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Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Imaging and Clinical Features of a Frequently Delayed Diagnosis.

Publication ,  Journal Article
Little, BP; Junn, JC; Zheng, KS; Sanchez, FW; Henry, TS; Veeraraghavan, S; Berkowitz, EA
Published in: AJR Am J Roentgenol
December 2020

OBJECTIVE. The purpose of this study was to assess features of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) on CT, clinical presentation, and delays in radiologic and clinical diagnosis in a series of 32 patients. MATERIALS AND METHODS. Medical records of patients with DIPNECH from the years 2000-2017 were obtained from an institutional data warehouse. Inclusion criteria were an available CT examination and either a pathologic diagnosis of DIPNECH or pathologic findings of multiple carcinoid tumorlets or carcinoid tumor with CT features suggesting DIPNECH. Two thoracic radiologists with 10 and 14 years of experience reviewed CT examinations and scored cases in consensus. RESULTS. All 32 patients were women, and most had never smoked (69%). The mean age at presentation was 61 years. Symptoms included chronic cough (59%) or dyspnea (28%), and the initial clinical diagnosis was asthma in 41%. DIPNECH was clinically suspected at presentation in only one case and was mentioned by the interpreting radiologist in only 31% of cases. CT characteristics included numerous nodules with a lower zone and peribronchiolar predominance, mosaic attenuation, and nodular bronchial wall thickening. Number of nodules at least 5 mm in diameter showed strong inverse correlations with the percentage predicted for both forced vital capacity and forced expiratory volume in 1 second and a moderate inverse correlation with total lung capacity percentage predicted. In cases with a follow-up CT interval of 3 years or longer, 85% of patients showed an increase in size of the largest nodule, and 70% had an increase in size in multiple nodules. CONCLUSION. Many cases of DIPNECH are originally missed or misdiagnosed by radiologists and clinicians. Awareness of the typical clinical and imaging features of DIPNECH may prompt earlier diagnosis of this condition.

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Published In

AJR Am J Roentgenol

DOI

EISSN

1546-3141

Publication Date

December 2020

Volume

215

Issue

6

Start / End Page

1312 / 1320

Location

United States

Related Subject Headings

  • Tomography, X-Ray Computed
  • Precancerous Conditions
  • Nuclear Medicine & Medical Imaging
  • Neuroendocrine Tumors
  • Neuroendocrine Cells
  • Multiple Pulmonary Nodules
  • Middle Aged
  • Lung Neoplasms
  • Hyperplasia
  • Humans
 

Citation

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Little, B. P., Junn, J. C., Zheng, K. S., Sanchez, F. W., Henry, T. S., Veeraraghavan, S., & Berkowitz, E. A. (2020). Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Imaging and Clinical Features of a Frequently Delayed Diagnosis. AJR Am J Roentgenol, 215(6), 1312–1320. https://doi.org/10.2214/AJR.19.22628
Little, Brent P., Jacqueline C. Junn, Karen S. Zheng, Frank W. Sanchez, Travis S. Henry, Srihari Veeraraghavan, and Eugene A. Berkowitz. “Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Imaging and Clinical Features of a Frequently Delayed Diagnosis.AJR Am J Roentgenol 215, no. 6 (December 2020): 1312–20. https://doi.org/10.2214/AJR.19.22628.
Little BP, Junn JC, Zheng KS, Sanchez FW, Henry TS, Veeraraghavan S, et al. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Imaging and Clinical Features of a Frequently Delayed Diagnosis. AJR Am J Roentgenol. 2020 Dec;215(6):1312–20.
Little, Brent P., et al. “Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Imaging and Clinical Features of a Frequently Delayed Diagnosis.AJR Am J Roentgenol, vol. 215, no. 6, Dec. 2020, pp. 1312–20. Pubmed, doi:10.2214/AJR.19.22628.
Little BP, Junn JC, Zheng KS, Sanchez FW, Henry TS, Veeraraghavan S, Berkowitz EA. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Imaging and Clinical Features of a Frequently Delayed Diagnosis. AJR Am J Roentgenol. 2020 Dec;215(6):1312–1320.

Published In

AJR Am J Roentgenol

DOI

EISSN

1546-3141

Publication Date

December 2020

Volume

215

Issue

6

Start / End Page

1312 / 1320

Location

United States

Related Subject Headings

  • Tomography, X-Ray Computed
  • Precancerous Conditions
  • Nuclear Medicine & Medical Imaging
  • Neuroendocrine Tumors
  • Neuroendocrine Cells
  • Multiple Pulmonary Nodules
  • Middle Aged
  • Lung Neoplasms
  • Hyperplasia
  • Humans