Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Syndrome and Carcinoid Tumors With/Without NECH

Maria Cecilia Mengoli, Giulio Rossi, Alberto Cavazza, Renato Franco, Federica Zito Marino, Mario Migaldi, Letizia Gnetti, Enrico Maria Silini, Luca Ampollini, Marcello Tiseo, Filippo Lococo, Ludovic Fournel, Paolo Spagnolo, Vincent Cottin, Thomas V. Colby

Research output: Contribution to journalArticle

Abstract

The diagnostic criteria of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) are not well defined, and DIPNECH can be mistaken for carcinoids associated with neuroendocrine cell hyperplasia (NECH). In this study, we compared clinical, radiologic, histologic, immunohistochemical, and molecular features of DIPNECH and isolated carcinoids with/without NECH. The study population included 151 cases (77 female patients and 74 male patients), 19 with DIPNECH and 132 with carcinoids with/without NECH. None of the cases displayed molecular alterations or anaplastic lymphoma kinase expression. Compared with individuals with carcinoids with/without NECH, patients with DIPNECH were more likely to be female individuals (P<0.0001), nonsmokers (P=0.021), and symptomatic, and to have an obstructive/mixed respiratory defect, peripheral location of the lesions, and air trapping (P<0.0001) on chest computed tomography, and constrictive bronchiolitis on histology (P<0.0001). Among immunohistochemical markers, DIPNECH was associated with higher expression of thyroid transcription factor-1, CD10, and gastrin-releasing peptide/bombesin-like peptide (P<0.0001). Yet, when a purely histopathologic definition of DIPNECH was applied, 40% of isolated carcinoids also met the diagnostic criteria for DIPNECH, even in the absence of symptoms and/or radiologic abnormalities. Therefore, as DIPNECH represents a distinct clinical syndrome, we suggest the term DIPNECH be limited to cases presenting with respiratory symptoms, functional and/or radiologic abnormalities, and constrictive bronchiolitis on histology.

Original languageEnglish
Pages (from-to)646-655
Number of pages10
JournalAmerican Journal of Surgical Pathology
Volume42
Issue number5
DOIs
Publication statusPublished - Jan 1 2018

    Fingerprint

Keywords

  • carcinoid
  • DIPNECH
  • immunohistochemistry
  • mTOR
  • neuroendocrine cell hyperplasia

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

Mengoli, M. C., Rossi, G., Cavazza, A., Franco, R., Marino, F. Z., Migaldi, M., Gnetti, L., Silini, E. M., Ampollini, L., Tiseo, M., Lococo, F., Fournel, L., Spagnolo, P., Cottin, V., & Colby, T. V. (2018). Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) Syndrome and Carcinoid Tumors With/Without NECH. American Journal of Surgical Pathology, 42(5), 646-655. https://doi.org/10.1097/PAS.0000000000001033