A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia.

Godwin Ofikwu,Ajai Rajabalan,Dennis Vega,Leaque Ahmed,Vishnu R Mani,Albert Adu

doi:10.1155/2015/318175

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.

Highlights

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a relatively new and rare disease that has recently received attention, and it is commonly misdiagnosed
We present our experience in the management of a unique case of a 45-year-old, nonsmoking woman who presented with bilateral pulmonary nodules and left hilar adenopathy in association with papillary carcinoma of the left breast, oncocytoma, and angiomyolipoma of the left kidney
This recent surge in frequency of the diagnosis of DIPNECH is due to increased use of high resolution CT (HRCT) scans combined with increased awareness of this entity as previously suggested by Davis et al [3]

Summary

A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare clinical condition with only about 100 cases reported in the literature. 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. 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

Introduction

Case Summary

Findings

Discussion