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目的:探讨儿童颅内幕上原始神经胚层肿瘤的临床表现、影像学特点、病理特点及手术治疗方法。方法:回顾性分析于2000年1月~2012年1月在我院经手术与病理证实为儿童颅内原始神经胚层肿瘤的50例患儿的临床资料。结果:(1)临床表现,本组患儿临床表现均以癫痫发作、慢性颅内高压及神经功能损害为主;(2)影像学特点,CT及MRI显示肿瘤体积大,血供丰富,边界清晰,呈囊性变、瘤内出血,病灶周围水肿较轻或无水肿;(3)病理特点,原始神经胚层由小圆细胞组成,可见菊花团生成,免疫组化染色呈多向分化倾向;(4)手术治疗,本组患儿均实施显微外科术,全切除39例,次全切除11例,术后放疗40例,好转出院46例,死亡4例,平均生存12.5±6.7个月。结论:应结合临床表现、影像学特点、病理特点对儿童颅内幕上原始神经胚层肿瘤进行诊断,治疗首选手术切除,术后需进行放射治疗。
Objective: To investigate the clinical manifestations, imaging features, pathological features and surgical treatment of primitive neuroectodermal tumors in the skull. Methods: The clinical data of 50 children with intracranial primitive neuroectodermal tumors confirmed by operation and pathology in our hospital from January 2000 to January 2012 were retrospectively analyzed. Results: (1) Clinical manifestations, clinical manifestations in this group of children with seizures, chronic intracranial hypertension and neurological dysfunction; (2) imaging features, CT and MRI showed tumor volume, rich in blood supply, the border Clear, cystic degeneration, intratumoral hemorrhage, lesser edema around the lesion or no edema; (3) Pathological features, primitive neuroectoderm composed of small round cells, the formation of chrysanthemum can be seen, immunohistochemical staining showed a multi-directional tendency; ( 4) Surgical treatment, the group of patients were performed microsurgery, total resection in 39 cases, subtotal resection in 11 cases, 40 cases of postoperative radiotherapy, discharge improvement in 46 cases, 4 patients died, the average survival of 12.5 ± 6.7 months. Conclusion: The diagnosis of primary neuroectodermal tumors on the intracranial suprascapus should be performed in combination with clinical manifestations, imaging features and pathological features. The patients should be treated with the first choice of surgical resection and radiotherapy after operation.