胸腺原发神经内分泌癌(primary neuroendocrine carcinoma of thymus, pNECT)为一种罕见的恶性病变,约占胸腺上皮肿瘤2%-5%[1-5]。自1972年Rosai等[6]首次报道至今,文献报道总病例数仅有数百例。约10%-30%的pNECT可以分泌促肾上腺皮质激素(adrenocorticotropin hormone, ACTH),从而导致库欣综合征(Cushing syndrome, CS),这种由非垂体肿瘤异位分泌ACTH导致CS也称为异位ACTH综合征(ectopic adrenocorticotropic hormone syndrome, EAS)。. The gene discussed is POMC; the disease is thymic neuroendocrine carcinoma.