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Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence

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Abstract

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is recognized as a preneoplastic condition by the World Health Organization. We reviewed our experience with 30 patients and performed a systematic review of the English literature to collect best evidence on the clinical features and disease course in 169 additional patients. Some patients presented with one or more carcinoid tumors associated with multiple small pulmonary nodules on imaging studies and showed DIPNECH as a somewhat unexpected pathologic finding. Others presented with multiple small pulmonary nodules that raised suspicion of metastatic disease on imaging. A third subset was presented with previously unexplained respiratory symptoms. In most patients, DIPNECH was associated with a good prognosis, with chronological progression into a subsequent carcinoid tumor noted in only one patient and death attributed directly to DIPNECH in only two patients. There is no best evidence to support the use of octreotide, steroids, or bronchodilators in DIPNECH patients.

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Acknowledgments

The authors would like to acknowledge Dr. Edward Wolin for his assistance in compiling relevant clinical data for this manuscript.

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Authors and Affiliations

  1. Department of Medicine, Cedars-Sinai Medical Center, 8700 Beverly Blvd Room 5512, West Hollywood, CA, 90048-1804, USA

    Eric Wirtschafter

  2. Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA

    Ann E. Walts & Alberto M. Marchevsky

  3. Department of Hematology and Oncology, University of California Los Angeles, David Geffen School of Medicine, Los Angeles, CA, USA

    Sandy T. Liu

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  1. Eric Wirtschafter
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  2. Ann E. Walts
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  3. Sandy T. Liu
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  4. Alberto M. Marchevsky
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Correspondence to Eric Wirtschafter.

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Wirtschafter, E., Walts, A.E., Liu, S.T. et al. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence. Lung 193, 659–667 (2015). https://doi.org/10.1007/s00408-015-9755-1

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  • DOI: https://doi.org/10.1007/s00408-015-9755-1

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