Li Da, Hao Shu-Yu, Wu Zhen, Zhang Li-Wei, Zhang Jun-Ting
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
J Neurosurg Pediatr. 2014 Sep;14(3):296-300. doi: 10.3171/2014.6.PEDS1423. Epub 2014 Jul 4.
Medulla oblongata teratomas are rare. The authors report 2 new cases of teratomas that occurred exclusively in the medulla oblongata. The first case was in a 9-year-old boy who presented with a 6-month history of neck pain and repeated paroxysmal vomiting. Based on preoperative radiographic findings, the initial diagnosis was of an intraaxial medulla oblongata hemangioblastoma. Intraoperatively, the cystic component of the tumor was gray, gelatinous, and soft in consistency. The solid component was light pink, rubbery, and nodular in appearance, with an identifiable boundary. The lesion was completely removed. Histopathological investigation revealed a mature teratoma. Postoperatively, the patient was supported with ventilator assistance and received a tracheotomy, but died of intracranial infection. The second case was in a 10-year-old boy with intermittent headache for 1 month. Radiographs revealed an exophytic cystic and solid lesion with dorsal involvement of the medulla oblongata. The lesion was predominantly solid, pinkish gray, tenacious, and moderately vascularized, with clearly delineated surgical dissection planes. The histopathological examination confirmed a diagnosis of immature teratoma. Total resection was achieved, followed by postoperative chemotherapy. He was alive without recurrence of the lesion or symptoms at 59 months after surgery. Resection of medulla oblongata teratoma is challenging, with inherent surgical risks that are contingent on the tumor growth pattern. Teratomas should be considered in the differential diagnosis of brainstem lesions. Chemotherapy has been suggested for immature teratomas. Long-term follow-up and larger studies of teratomas in unusual locations are required to improve practitioners' understanding of this disease's treatment and outcomes.
延髓畸胎瘤很罕见。作者报告了2例仅发生于延髓的畸胎瘤新病例。第一例是一名9岁男孩,有6个月的颈部疼痛和反复阵发性呕吐病史。根据术前影像学检查结果,初步诊断为轴内延髓血管母细胞瘤。术中,肿瘤的囊性部分呈灰色、胶冻状,质地柔软。实性部分呈浅粉色、橡胶样,外观呈结节状,边界清晰。病变被完全切除。组织病理学检查显示为成熟畸胎瘤。术后,患者接受呼吸机辅助支持并进行了气管切开术,但死于颅内感染。第二例是一名10岁男孩,有1个月的间歇性头痛。影像学检查显示为一个向外生长的囊实性病变,累及延髓背侧。病变以实性为主,呈粉灰色,质地坚韧,血管中等丰富,手术分离平面清晰。组织病理学检查确诊为未成熟畸胎瘤。实现了肿瘤全切,随后进行了术后化疗。术后59个月时,他存活,病变及症状均未复发。切除延髓畸胎瘤具有挑战性,其固有的手术风险取决于肿瘤的生长方式。在脑干病变的鉴别诊断中应考虑畸胎瘤。对于未成熟畸胎瘤,建议进行化疗。需要对罕见部位的畸胎瘤进行长期随访和更大规模的研究,以提高医生对这种疾病治疗及预后的认识。