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Case Reports in Surgery
Volume 2015, Article ID 318175, 5 pages
http://dx.doi.org/10.1155/2015/318175
Case Report

A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia

1Department of Surgery, Columbia University College of Physicians and Surgeons at Harlem Hospital, New York, NY 10037, USA
2Department of Surgery, New York University School of Medicine, New York, NY 10016, USA
3Department of Cardiothoracic Surgery, Harlem Hospital Center, New York, NY 10037, USA

Received 4 September 2015; Accepted 8 October 2015

Academic Editor: Francesco Petrella

Copyright © 2015 Godwin Ofikwu et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare clinical condition with only about 100 cases reported in the literature. It is characterized by primary hyperplasia of pulmonary neuroendocrine cells (PNECs) which are specialized epithelial cells located throughout the entire respiratory tract, from the trachea to the terminal airways. DIPNECH appears in various forms that include diffuse proliferation of scattered neuroendocrine cells, small nodules, or a linear proliferation. It is usually seen in middle-aged, nonsmoking women with symptoms of cough, dyspnea, and wheezing. We present a 45-year-old, nonsmoking woman who presented with symptoms of DIPNECH associated with bilateral pulmonary nodules and left hilar adenopathy. Of interest, DIPNECH in our patient was associated with metastatic pulmonary carcinoids, papillary carcinoma of the left breast, oncocytoma and angiomyolipoma of her left kidney, and cortical nodules suggestive of tuberous sclerosis. She had video assisted thoracoscopic surgery (VATS), modified radical mastectomy with reconstruction, and radical nephrectomy. She is currently symptom-free most of the time with over two years of follow-up.