DIPNECH predominantly occurs in the elderly (median age 66 years) and in women (93%) and is not associated with smoking, the demographic characteristics of our cases were consistent with the literature.
The definitive diagnosis of DIPNECH is impossible in conventional chest radiography.
To our knowledge, there has not been familial case of DIPNECH
, although there has been familial case of neuroendocrine cell hyperplasia of infancy (NEHI), which was related to mutation of NKX2 encoding TTF-1.
originates in an uncontrolled linear growth of pulmonary neuroendocrine (NE) cells.
What are the practical issues with the diagnosis of DIPNECH
? In a classic clinical setting of a patient presenting with respiratory symptoms and pathologic findings of diffuse NE hyperplasia and tumorlets in the absence of other pathologic findings, the diagnosis of DIPNECH
The relationship between DIPNECH
to more central, highgrade neuroendocrine tumors is unclear.