Aim: We highlight the occurrence of an unusual neuroendocrine tumour,
a large cell neuroendocrine carcinoma, arising from the thymus. Case d
etails: A 68-year-old man with a history of cigarette smoking had a la
rge mediastinal tumour arising from the thymus removed, Two years late
r the tumour recurred; it was debulked surgically but the patient died
2 months later, Histological examination of both tumour specimens rev
ealed a tumour with an endocrine pattern, composed of large pleomorphi
c cells with large nuclei and prominent nucleoli, The mitotic count ra
nged from 19 to 26 per 10 high-power fields and large tracks of coagul
ative tumour necrosis were present, The tumour cells were strongly pos
itive for neuron-specific enolase (NSE), chromogranin, CAM5.2 and AE1/
3, with cytoplasmic dot-like accentuation for the latter three markers
, The tumour fulfilled the criteria for a diagnosis of large cell neur
oendocrine carcinoma. Conclusions: Large cell neuroendocrine carcinoma
should be distinguished from atypical carcinoid and small cell carcin
oma, It is a distinctive neuroendocrine malignancy with a prognosis be
tween that of atypical carcinoid and small cell carcinoma, and needs t
o be treated aggressively.