Chetty R, Batitang S, Govender D
Department of Anatomical Pathology, University of Natal School of Medicine, Durban, South Africa.
Histopathology. 1997 Sep;31(3):274-6. doi: 10.1046/j.1365-2559.1997.2380849.x.
We highlight the occurrence of an unusual neuroendocrine tumour, a large cell neuroendocrine carcinoma, arising from the thymus.
A 68-year-old man with a history of cigarette smoking had a large mediastinal tumour arising from the thymus removed. Two years later the tumour recurred; it was debulked surgically but the patient died 2 months later: Histological examination of both tumour specimens revealed a tumour with an endocrine pattern, composed of large pleomorphic cells with large nuclei and prominent nucleoli. The mitotic count ranged from 19 to 26 per 10 high-power fields and large tracks of coagulative tumour necrosis were present. The tumour cells were strongly positive 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 neuroendocrine carcinoma.
Large cell neuroendocrine carcinoma should be distinguished from atypical carcinoid and small cell carcinoma. It is a distinctive neuroendocrine malignancy with a prognosis between that of atypical carcinoid and small cell carcinoma, and needs to be treated aggressively.
我们着重介绍了一种罕见的神经内分泌肿瘤，即起源于胸腺的大细胞神经内分泌癌的发生情况。
一名有吸烟史的68岁男性接受了胸腺来源的巨大纵隔肿瘤切除术。两年后肿瘤复发；进行了手术减瘤，但患者在2个月后死亡。对两份肿瘤标本进行组织学检查，发现肿瘤具有内分泌模式，由具有大核和明显核仁的大的多形性细胞组成。每10个高倍视野的有丝分裂计数为19至26个，并且存在大片凝固性肿瘤坏死。肿瘤细胞对神经元特异性烯醇化酶（NSE）、嗜铬粒蛋白、CAM5.2和AE1/3呈强阳性，后三种标志物呈胞质点状增强。该肿瘤符合大细胞神经内分泌癌的诊断标准。
大细胞神经内分泌癌应与非典型类癌和小细胞癌相鉴别。它是一种独特的神经内分泌恶性肿瘤，预后介于非典型类癌和小细胞癌之间，需要积极治疗。
