颌下腺外周原始神经外胚层肿瘤1例并文献复习

Peripheral Primitive Neuroectodermal Tumors of Submaxillary Gland: Report of 1 Case and Review of Literature

目的探讨颌下腺外周原始神经外胚层肿瘤(peripheral primitive neuroectodermal tumor,pPNET)的临床、病理组织学特征及诊治方法。方法通过光镜及免疫组织化学染色分析1例颌下腺原发的pPNET,同时复习相关文献。结果肿瘤位于左侧颌下腺内侧,肿瘤细胞呈片状弥漫分布,为单一的小圆形细胞,胞质少,核圆。免疫组织化学示CD99、Syn和Vim均为阳性(+)。结论 pPNET是一种少见的起源于原始神经外胚层的恶性肿瘤,好发于深部软组织,极罕见于颌下腺,具有高度侵袭性,预后差;免疫组织化学有助于pPNET的诊断;目前治疗主要是手术切除加化、放疗。

Objective To investigate the clinicopathological features and the diagnosis and treatment of peripheral primitive neuroectodermal tumor(pPNET) of submandibular gland.Methods A case of submaxillary gland pPNET was analyzed by using light microscopy and immunohistochemical staining.The relevant literature was reviewed. Results The tumor was located in the left medial submandibular gland.Tumor cells showed patchy diffuse distribution,and were identified as single small round cells with less cytoplasm and round nuclei.Immunohistochemistry displayed positive expression of CD99,Syn and Vim.Conclusion The pPNET,a rare malignant peripheral nerve tumor of primitive origin with high invasiveness and poor prognosis,usually occurs in deep soft tissues and is extremely rare in submandibular gland.Immunohistochemical staining is helpful in the diagnosis of pPNET and surgical excision combined with chemotherapy or radiotherapy is the initial treatment for pPNET.