Correct!
4. All of the above
There are areas of normal lung interspersed with proliferation of cells both in small bronchi and bronchioles and outside the airway. For those outside the airway proliferating in small nodules the term tumorlet is appropriate. The proliferating cells stained with synaptophysin, also known as the major synaptic vesicle protein p38, a marker for neuroendocrine cells. The histology is characteristic of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).
DIPNECH was described by Sam Aguayo (3) now at the Phoenix VA while he was at the University of Colorado. Recently Nassar et al. (4) from the Mayo Clinic Arizona described 24 cases in the literature and added an additional case. The majority of patients presenting with DIPNECH are middle-aged females with symptoms of cough and dyspnea; obstructive abnormalities on pulmonary function testing; and radiographic imaging showing pulmonary nodules, ground-glass attenuation, and bronchiectasis. In general, the clinical course remains stable; however, progression to respiratory failure does occur. Long-term follow-up studies and the best treatment remains unknown.
The present patient was declined for heart transplant and returned to Indiana on inhaled corticosteroids.
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