Imaging: Pulmonary Nodules |

An Under Recognized Cause of Chronic Cough: Diffuse Idiopathic Pulmonary Neuroendocine Cell Hyperplasia (DIPNECH) FREE TO VIEW

Whittney Warren, DO; William Kelly, MD
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Walter Reed National Naval Medical Center, Bethesda, MD

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A255. doi:10.1016/j.chest.2016.02.266
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SESSION TITLE: Pulmonary Nodules

SESSION TYPE: Case Report Slide

PRESENTED ON: Saturday, April 16, 2016 at 01:00 PM - 02:00 PM

INTRODUCTION: DIPNECH is a rare cause of chronic cough that should be considered in patients with wheezing, dyspnea and multiple small pulmonary nodules.

CASE PRESENTATION: A 64-year-old male former smoker presented to pulmonary clinic complaining of non productive cough for ten years. Prior evaluation included treatment for upper airway cough syndrome and gastroesophageal reflux, stopping ACE-I, inhaled beta-agonists, and a negative bronchoscopy. Physical examination was unremarkable. Serial PFTs evolved from normal to mixed pattern with airway hyperactivity. Chest CT showed a 1.7 cm lingua nodule (1.2 cm 10 years prior), with numerous bilateral 3-4 mm pulmonary nodules. Scattered areas of mosaicism and ground glass opacity were appreciated on the updated higher resolution CT. Surgical resection demonstrated typical carcinoid with associated tumorlets and no lymph node metastasis. Postoperative octreoscan was negative but chromogranin and HIAA levels were elevated. Management included inhaled corticosteroids and serial imaging with somatostatin analogue to be considered in the future.

DISCUSSION: Pulmonary neuroendocrine, or Kulchitsky-type cells, are rare airway epithelial cells that release bioactive compounds. Neuroendocrine hyperplasia is a premalignant finding limited to the basement membrane or airway wall and occurs spontaneously or secondary to cigarette smoke and fibrotic lung diseases. Cellular proliferation of < 5 mm beyond these boundaries is a carcinoid tumorlet. Carcinoid tumors are tumorlets > 5 mm. Coexistence of all three often occurs in DIPNECH, a rare condition defined by WHO as a carcinoid precursor absent in other neuroendocrine cancers. DPINECH is found in 5% of patients undergoing carcinoid resection. Patients are usually misclassified as difficult to control asthma or chronic obstructive pulmonary disease. Preoperative diagnosis is rare, but should be considered in females age 45-67, with cough, dyspnea and wheezing. Radiographic clues include small pulmonary nodules, ground-glass opacities, mosaic attenuation, air trapping, and bronchiectasis. Minimum pathologic criteria for surgical specimens have been proposed, but transbronchial biopsy may suffice. Octreotide, chemotherapy, steroids, and transplant have been tried for symptomatic treatment.

CONCLUSIONS: DIPNECH should be considered in patients with refractory cough and obstructive pulmonary physiology, even with small pulmonary nodules that are stable over many years. The majority of patients have an indolent course with 83% 5 year survival. Prognosis depends more on the obstructive pulmonary physiology than the carcinoid tumors.

Reference #1: Carr LL, Chung JH, Achcar RD et al. The Clinical Course of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia. CHEST. 2015;147(2):415-422.

Reference #2: Schnabel PA, Junker K. Pulmonary neuroendocrine tumors in the new WHO 2015 classification: Start of breaking new grounds? Pathologe. 2015;36(3):283-92.

DISCLOSURE: The following authors have nothing to disclose: Whittney Warren, William Kelly

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