Abstract
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is considered a rare syndrome and knowledge of its pathophysiology is scarce. Classified as pre-neoplastic lesion of carcinoid tumours currently there are many questions regarding the best approach to follow-up and treatment this patients. Mainly affects females, there is no apparent relationship to smoking habit and is often confused with obstructive airway disease. The diagnosis is histological and sometimes can be observed simultaneously a triple pattern consisting of DIPNECH, tumourlets and carcinoid tumour. We report the case of a 35-years-old woman, non-smoker with DIPNECH in association with typical carcinoid tumour and tumourlets.
Highlights
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is considered a rare syndrome that affects the bronchial tree and is often confused with chronic obstructive pulmonary disease [1,2]
We report the case of a 35-years-old woman, non-smoker with DIPNECH in association with typical carcinoid tumour and tumourlets
The spectrum of disease ranges between the foci of hyperplasia less than 5 mm without involvement of the basal membrane that are designate tumourlets and those who have more than 5 mm longer and invade the basement membrane designate carcinoid tumours [1]
Summary
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is considered a rare syndrome that affects the bronchial tree and is often confused with chronic obstructive pulmonary disease [1,2]. The spectrum of disease ranges between the foci of hyperplasia less than 5 mm without involvement of the basal membrane that are designate tumourlets and those who have more than 5 mm longer and invade the basement membrane designate carcinoid tumours [1]. In followup visit after 2 years there was a history of weight loss about 10 kg in 8 months and it was decided to repeat chest CT that revealed in the right lower lobe two small nodular hyperdense solid formations, one with 5 mm and another with 2 mm and in the lower left lobe a solid hyperdense nodular lesion with 18 mm and well-defined contours. A left lower lobectomy to get histological and surgical treatment was performed and the pathology report revealed a neuroendocrine tumour, morphologically without necrosis or mitosis and histological. Corresponding to a typical carcinoid tumour with multiple foci of endocrine cell hyperplasia and tumourlets
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