Diffuse pulmonary neuroendocrine cell hyperplasia with tumorlets and elevated serum CEA

Abstract

iffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a generalized proliferation of scattering single cells, small nodules (neuroendocrine bodies) or linear proliferations of neuroendocrine cells of lung. They may be confined to the bronchial and bronchiolar epithelium, include local extraluminal proliferation (tumorlets), or progress to the development of carcinoid tumor. Sometimes DIPNECH is accompanied by intra-and extraluminal fibrosis of the involved airways. 1,2 It is rare and presents typically in the fifth or sixth decades and is more common in woman. The great majority of pulmonary tumorlets are found incidentally and show no clinical consequence. Only rare occasions bronchopulmonary lymph node metastasis were reported. 3,4,5 DIPNECH and tumorlets may associated with Cushing syndrome, 6,7 elevated serum CEA. 8 Case report A 60-year-old asymptomatic Thai female with history of mild hyperglycemia, hyperlipidemia and hyperuricemia had high and rising serum CEA levels (Figure 1). The chest x-ray and tomosynthesis (Figure 2a-b) showed subsegmental atelectasis and reticular infiltration at the right middle lobe. CT Chest (Figure 3) showed small pulmonary atelectasis at right middle lobe.

Diffuse Intrapulmonary Neuroendocrine Cell Hyperplasia

“…One series reported DIPNECH with multiple endocrine neoplasia type 1 (7), and another with adenocarcinoma of mixed subtype and partial neuroendocrine differentiation (12). Very rarely, it is reported with ectopic adrenocorticotropic hormone production (13) and elevated serum carcinoembryonic antigen levels in the absence of pulmonary or primary gastrointestinal adenocarcinoma (14). DIPNECH has also been found on surgical lung biopsy of patients with respiratory failure of unclear etiology requiring intubation and mechanical ventilation (15).…”