婴儿色素性神经外胚瘤1例报道并文献复习

目的 探讨婴儿色素性神经外胚瘤的临床病理特征、免疫组化、诊断和鉴别诊断要点。方法 对1例婴儿色素性神经外胚瘤进行组织学和免疫组化观察和文献复习。结果 婴儿色素性神经外胚瘤好发于1岁以内的婴儿，肿瘤多见于上颌骨和颅骨，表现为浸润性和溶骨性破坏。组织学上显示大的并含不等量色素颗粒的上皮样细胞和小的神经母细胞样细胞。免疫组化显示CK、HMB-45、S-100蛋白、NSE在上皮样细胞呈阳性表达，小圆形瘤细胞S-100蛋白、NSE阳性或部分阳性。肿瘤彻底切除，随访3年未发现转移和复发。结论 婴儿色素性神经外胚瘤是一种少见的起源于神经嵴细胞的肿瘤，具有特征性的临床病理改变，需要和神经母细胞瘤、恶性黑色素瘤及其它小圆细胞肿瘤鉴别，生物学行为属于潜在恶性或低度恶性肿瘤，彻底切除预后良好。

Melanotic neuroectodermal tumor of infancy a case report and review of the literature

Purpose To investigate the clinicopathologic and immunohistochemical features and differential diagnosis of melanotic neuroectodermal tumor of infancy(MNTI). Methods A case of MNTI was observed by morphological and immunohistochemical techniques and review of the literatures. Results MNTI occurred predominantly in infants under 1 year of age. The tumor sat in the maxilla, mandible and skull. Aggressive behavior and osteolytic destruction were observed. Histologically, MNTI was composed of larger melanin-containing epithelial cells and small neuroblast-like cells. Immunohistochemically, the epithelial cells were positive for CK, HMB-45, NSE and S-100 protein. NSE and S-100 protein were also positive or mild positive in the small neuroblast-like cells. The tumor was resected completely. No recurrences or metastases were found at 3 years of follow-up. Conclusions MNTI is a rare tumor originated from neural crest cells. The diagnosis of MNTI can be determined by the clinicopathological features. Differential diagnosis considerations of MNTI should include neuroblastoma, malignant melanoma and other small round cell tumor. Studies indicate that MNTI has malignant potential or low malignant. Better prognosis might be achieved by wide local excision.