Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)

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Introduction. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a poorly understood disease that, until recently, was largely underdiagnosed. It most often develops in non-smoking women aged 50 years and older without preexisting pulmonary pathology and is extremely rare in men. Aim. To raise awareness of the diagnosis of DIPNECH using the presented clinical case, and familiarize physicians with the clinical and morphological features of this lung pathology. Mat erials and methods. This clinical case of DIPNECH was diagnosed in a 71-year-old female patient with severe concomitant cardiac pathology. Chest CT revealed multiple dense, well-defined lesions measuring 2–12 mm in size in the lung tissue on both sides. These lesions were considered metastatic lesions to both lungs. Due to deteriorating cardiac status, the patient underwent stenting of the anterior interventricular septum vessels, and in 2 months an atypical resection of the middle and lower lobes of the right lung was performed. Morphological examination revealed changes in the tissue of the middle and lower lobes of the right lung consistent with DIPECH, and TTF-1 expression in the cells of the lesions indicated a primary lung lesion. The patient was discharged for follow-up at her place of residence. After 4.5 years, she noted a deterioration in her condition. A follow-up PET/CT scan revealed multiple lesions measuring 3‒12 mm in the upper lobe of the right lung, and the patient underwent an atypical resection of the upper lobe of the right lung. The morphological features and immunophenotype of the lesions in the upper lobe of the right lung were also consistent with DIPNECH. C on cl u si on s . This case demonstrates that the diagnosis of DIPNECH should be included in the mandatory differential diagnosis algorithm for unclear lung pathology, especially in women at risk for developing such pathology.

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