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目的:探讨睾丸原始神经外胚层肿瘤的影像学特征、诊断方法和治疗选择。方法:回顾性分析1例罕见的少儿睾丸原始神经外胚层肿瘤的临床资料,包括患儿的病史资料、影像资料、术中所见和病理资料,进行文献复习。结果:原始神经外胚层肿瘤(PNET)是一种较为罕见的高度恶性小圆细胞肿瘤,其诊断主要依靠病理和免疫组化,基因分析有助诊断,复发时间和复发部位及范围与预后密切相关。结论:睾丸原始神经外胚层肿瘤十分罕见,睾丸根治性切除联合化疗治疗对局部肿瘤有效,预后因素包括患者年龄、复发时间和复发范围。
Objective: To explore the imaging features, diagnosis and treatment options of primary testicular neuroectodermal tumors. Methods: A retrospective analysis of 1 case of children with primary testicular primitive neuroectodermal tumor clinical data, including children’s history data, imaging data, intraoperative findings and pathological data for literature review. Results: The primitive neuroectodermal tumor (PNET) is a rare type of highly malignant small round cell tumor. The diagnosis mainly depends on the pathology and immunohistochemistry. The genetic analysis is helpful for the diagnosis. The recurrence time and the site and the range of recurrence are closely related to the prognosis . CONCLUSIONS: Testicular primitive neuroectodermal tumors are rare. Radical resection of the testicle combined with chemotherapy is effective in treating local tumors. Prognostic factors include age, recurrence time and extent of recurrence.