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成功治疗伴有严重脊髓压迫的原发性胸段哑铃型卵黄囊瘤:病例报告

Successful treatment of a primary thoracic dumb-bell yolk sac tumor presenting with severe spinal cord compression: Case report.

作者信息

Li Zhenfeng, Lv Zhaorui, Yang Qiang, Li Jianmin

机构信息

Department of Orthopedics, Qilu Hospital of Shandong University, Jinan, Shandong Province, China.

出版信息

Medicine (Baltimore). 2019 Oct;98(43):e17610. doi: 10.1097/MD.0000000000017610.

Abstract

RATIONALE

Primary thoracic dumb-bell yolk sac tumor (YST) with both epidural and extraspinal extension is a rare disease with no standard curative managements yet. The objective of this study is to report a primary thoracic dumb-bell YST presenting with severe spinal cord compression successfully treated with posterior-only approach operation, followed by chemotherapy. The management of these unique cases has not been fully documented.

PATIENT CONCERNS

A 26-mounth-old, previously healthy girl presented with progressive numbness and weakness of the lower extremities. Neurological examination revealed paralysis of both lower extremities, sensory disturbance below T-8 and bladder-bowel dysfunction.

DIAGNOSIS

CT and MRI of spine showed a dumb-bell mass lesion with both epidural and extraspinal extension through enlarged intervertebral foramina and marked spinal cord compression at T7-T9. The AFP level was 13738 ng/ml. Preoperative puncture and Postoperative pathology confirmed the diagnosis of YST.

INTERVENTIONS

By needle biopsy, we identified the pathological diagnosis is YST. Subsequently, the patient was treated with one-stage posterior-only approach operation, followed by 9 courses of chemotherapy based on cisplatin, bleomycin, etoposide.

OUTCOMES

The patient has a complete neurologic recovery and remains recurrence free as of more than 2 years after the completion of operation. There were no other complications associated with the operation during the follow-up period.

LESSONS

YST should be considered in the range of children with thoracic dumb-bell tumor presenting with spinal cord compression. Needle biopsy is valuable for preoperative diagnosis and design of the treatment strategy. If there is no evidence of CSF spread, metastasis or multiple diseases, it is effective to remove tumors as thoroughly as possible immediately, avoid further nerve injury and conduct enough chemotherapy. This case suggests that this treatment strategy is an effective option for primary YST with both epidural and extraspinal extension and severe spinal cord compression.

摘要

原理

原发性胸段哑铃形卵黄囊瘤(YST)伴硬膜外和椎管外扩展是一种罕见疾病,目前尚无标准的治愈性治疗方法。本研究的目的是报告一例原发性胸段哑铃形YST,其表现为严重脊髓压迫,通过单纯后路手术成功治疗,随后进行化疗。这些特殊病例的治疗方法尚未得到充分记录。

患者情况

一名26个月大、此前健康的女孩出现下肢进行性麻木和无力。神经学检查显示双下肢瘫痪、T-8以下感觉障碍以及膀胱直肠功能障碍。

诊断

脊柱CT和MRI显示一个哑铃形肿块病变,通过扩大的椎间孔有硬膜外和椎管外扩展,T7-T9水平有明显脊髓压迫。甲胎蛋白水平为13738 ng/ml。术前穿刺及术后病理证实为YST。

干预措施

通过针吸活检,我们确定病理诊断为YST。随后,患者接受了一期单纯后路手术,然后基于顺铂、博来霉素、依托泊苷进行了9个疗程的化疗。

结果

患者神经功能完全恢复,截至手术完成后2年多无复发。随访期间未出现与手术相关的其他并发症。

经验教训

对于出现脊髓压迫的胸段哑铃形肿瘤患儿,应考虑YST。针吸活检对术前诊断和治疗策略设计有价值。如果没有脑脊液播散、转移或多病灶的证据,立即尽可能彻底切除肿瘤、避免进一步神经损伤并进行充分化疗是有效的。该病例表明,这种治疗策略是原发性YST伴硬膜外和椎管外扩展及严重脊髓压迫的有效选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/82a8/6824751/f87c195d81c0/medi-98-e17610-g001.jpg

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