婴儿色素性神经外胚瘤临床病理观察

MELANOTIC NEUROECTODERMAL TUMOR OF INFANCY: A CLINICOPATHOLOGICAL ANALYSIS OF 5 CASES

目的:探讨色素性神经外胚瘤的病理形态、免疫组化及诊断和鉴别要点。方法:对5例色素性神经外胚瘤病例进行临床病理、免疫组化及电镜观察。结果:色素性神经外胚瘤好发于1岁以内的婴儿,3例肿瘤发生在上颌骨,2例发生在附睾。镜下由两种细胞构成,即上皮样瘤细胞和小圆形似成神经细胞样瘤细胞。免疫组化示上皮样瘤细胞CK、HMB45阳性,小圆形瘤细胞NSE阳性,超微结构显示肿瘤细胞内分别可见前黑色素小体、黑色素小体和神经内分泌颗粒。结论:色素性神经外胚瘤是一种少见的原始性神经外胚层肿瘤,生物学行为属于潜在恶性或低度恶性肿瘤。

Objective: To Study the Clinicopathological and immunohistochemical characteristics and differential diagnosis of melanotic neuroectodermal tumor of infancy(MNTI). Methods: Five cases of MNTI were analyzed using HE, immunohistochemical stains and electron microscope. Results: MNTI occurred predominantly in infancy, three tumor sat in the upper jaws and two sat in epididymis. Histologically, the tumor was composed of epithelial cells and small cells neuroblastoma-like cells. Immunohistochemically, epithelial cells expressed CK, HMB45 and small cells expressed NSE. Ultrastructural observation showed the epithelial cells had premelanotic-bodies and melanotic-bodies, small cells had neuroendocrine granules. Conclusions: MNTI is a rare tumor origins from primitive neuroectodermal, studies indicated that the tumor is malignant potential or low malignant.