*From the Royal Liverpool University Hospital, Liverpool, UK.
Correspondence to: J.R. Gosney, BSc, MD, Department of Pathology, 5th floor, Duncan Bldg, Royal Liverpool University Hospital, Daulby St, Liverpool L69 3GA, UK; e-mail: email@example.com
In 1992, Aguayo1 et al described six patients with slowly progressive cough and breathlessness, obstructive and/or restrictive respiratory impairment, and diffuse reticulonodular infiltrates seen on thoracic tomographic scanning, in whom a pulmonary biopsy revealed widespread intraluminal and extraluminal proliferation of pulmonary neuroendocrine cells (PNCs) in all patients, accompanied by peripheral carcinoid tumors in three patients. There was submucosal fibrosis of affected airways, but none of the conditions usually associated with the reactive proliferation of PNCs was evident. This and subsequent studies have further defined the clinical and pathologic parameters of this rare condition, now conventionally known as diffuse idiopathic PNC hyperplasia or DIPNECH.
DIPNECH raises interesting questions about the relationship between the proliferation of PNCs, hitherto considered a reactive process, and the genesis of bona fide pulmonary neuroendocrine tumors (PNTs), such that it now appears as a preneoplastic condition in the latest World Health Organization histologic classification of tumors of the lung and pleura. However, the carcinoid tumors that accompany DIPNECH and are presumed to develop from it appear to be invariably peripheral and always of low grade. The disease runs a chronic, indolent course, and the emergence from DIPNECH of PNTs in the central airways or of higher grades of malignancy has not been described.
It is probable, therefore, that DIPNECH is a precursor exclusively of typical carcinoid tumors in a peripheral location. In this regard, it may be related to the more localized and less florid proliferation of PNCs that are commonly seen in the vicinity of such tumors. A role for the condition as a precursor to the predominantly central, high-grade PNTs (ie, the large cell and small cell neuroendocrine carcinomas) seems unlikely, however, any precursor to these more common and dangerous tumors remains unknown.
Abbreviations: DIPNECH = diffuse idiopathic PNC hyperplasia; PNC = pulmonary neuroendocrine cell; PNT = pulmonary neuroendocrine tumor
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