Pulmonary Peripheral Carcinoids after Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Tumorlets: Report of 3 Cases

Abstract

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) and tumorlets are neuroendocrine cells proliferations smaller than 5 mm. The former confines to bronchial/bronchiolar wall, while the latter broke through epithelial basement membrane. The authors present 3 cases of DIPNECH and tumorlets associated with a typical peripheral carcinoid tumor without underlying lung disease. The patients presented with nonspecific pulmonary symptoms: 3 females, 60, 72, and 84 years old, whose CT-scans showed well-defined pulmonary nodules, 2.2, 1.6, and 1.4 cm, respectively; first patient was submitted to lobectomy and the others underwent surgical biopsy. Whitish/brownish lobulated tumors corresponded to typical carcinoids (less than 2 mitoses/2 mm2 and without necrosis); polygonal/elongated cells under lobular pattern expressed CD56, chromogranin A, synaptophysin, and CK7; Ki-67 positivity was between 1 and 3%. Bronchial/bronchiolar wall neuroendocrine cell hyperplasia and several neuroendocrine nodules under 5 mm, with identical morphologic and immunoexpression, were observed, without lung disease. Typical carcinoid associated with DIPNECH and tumorlets without other pulmonary diseases is rare. Sporadic cases may recall embryonal neuroendocrine differentiation potentiality to develop peripheral hyperplasia, most commonly in underoxygenated parenchyma. The described cases are elucidative of peripheral spectrum of neuroendocrine cell tumour evolution, reinforcing higher female incidence as in central carcinoids, still without a clear preneoplastic lesion.

Orthopedia Homeobox (OTP) in Pulmonary Neuroendocrine Tumors: The Diagnostic Value and Possible Molecular Interactions

“…Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) was first described by Aguayo et al, as an unusual clinical entity that may cause airway fibrosis [24]. DIPNECH is confined to the respiratory epithelium layer without penetration of the basement membrane and is not related to any known predisposing condition [25]. Nowadays, it is known that pulmonary neuroendocrine cell hyperplasia (PNECH) can emerge, not only as a reaction to inflammation, but also in the context of carcinoid or adenocarcinoma development (Figure 1D) [26].…”

Rare diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: one case report and literature review

“…It is misdiagnosed for asthma or chronic bronchitis usually. Most patients are nonsmoker female between the ages of fifty and seventy with a dry cough and/or dyspnea, the clinical symptoms lowly worsens or keeps stable evolution over several years ( 8 , 9 ). This patient is 68 years old at the first visit and regular follow-ups 4 years preoperative, dry cough symptom gradually worsens.…”

“…Firm the diagnosis of DIPNECH relied on pathology and IHC staining, endocrine markers Syn, CA-g and CD56 are all positive and CD10 is stained consistently in neuroendocrine cells. Otherwise bombesin, BCL-2, retinoblastoma protein, p27, and calcitonin staining is variable in diagnosis ( 8 ). In this case, IHC staining CD56, CA-g, TTF and Syn are positive and ki-67 is only about 5%, which is same to the literature reported.…”