Moran C A, Suster S
Department of Pathology, University of Alabama at Birmingham, 35233-7331, USA.
Mod Pathol. 1999 Jun;12(6):587-91.
Seven cases of spindle-cell neuroendocrine carcinomas (carcinoid tumors) of the thymus are presented. The patients were three women and four men between the ages of 26 and 74 years (median age, 50 yr). The lesions presented as large anterior mediastinal masses on radiographic examination and were treated by surgical excision. Grossly, the tumors were tan-brown and well circumscribed but encapsulated, and they measured from 2 to 15 cm in greatest diameter. Histologically, they were characterized by a dense proliferation of spindle cells that focally adopted a vaguely organoid pattern, with discrete nests of tumor cells separated by thin fibrovascular septa. Mitotic figures were present in all of our cases and ranged from 2 to 8 per 10 high power fields. Focal areas of necrosis were also present in all of the cases. Immunohistochemical studies performed in six cases showed positive staining for chromogranin in five cases, synaptophysin and keratin in four, and Leu 7 in three. Clinical follow-up showed that two patients died of their tumors 6 and 11 years after diagnosis; one was alive 8 years after diagnosis. Spindle-cell neuroendocrine carcinomas of the thymus (spindle-cell thymic carcinoids) should be considered in the differential diagnosis of spindle-cell neoplasms of the anterior mediastinum. Because of their aggressive clinical behavior, it is important to separate them from the other benign or low-grade spindle-cell tumors that are more common at this location.
本文报告7例胸腺梭形细胞神经内分泌癌(类癌肿瘤)。患者年龄在26至74岁之间,其中3例为女性,4例为男性(中位年龄50岁)。影像学检查显示病变为前纵隔巨大肿块,均接受了手术切除。大体上,肿瘤呈棕褐色,边界清楚但有包膜,最大直径2至15厘米。组织学上,其特征为梭形细胞密集增生,局部呈模糊的器官样结构,肿瘤细胞巢离散,由薄的纤维血管间隔分隔。所有病例均可见核分裂象,每10个高倍视野中核分裂象为2至8个。所有病例均有局灶性坏死区域。6例进行了免疫组化研究,5例嗜铬粒蛋白染色阳性,4例突触素和细胞角蛋白染色阳性,3例Leu 7染色阳性。临床随访显示,2例患者在诊断后6年和11年死于肿瘤;1例在诊断后8年仍存活。胸腺梭形细胞神经内分泌癌(梭形细胞胸腺类癌)应列入前纵隔梭形细胞肿瘤的鉴别诊断。由于其侵袭性临床行为,将其与该部位更常见的其他良性或低级别梭形细胞肿瘤区分开来很重要。