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罕见的骶骨骨内黑色素性雪旺细胞瘤病例:文献复习。

A Rare Case of Melanotic Schwannoma Occurred Intraosseous of Sacrum: A Literature Review.

机构信息

Department of Orthopedic Oncology, The Second Affiliated Hospital of Zhejiang University, Hangzhou, China.

Key Laboratory of Motor System Disease Research and Precision Therapy of Zhejiang Province, Hangzhou, China.

出版信息

Orthop Surg. 2023 Feb;15(2):655-662. doi: 10.1111/os.13606. Epub 2022 Dec 1.

Abstract

BACKGROUND

Melanotic schwannoma is a rare tumor when it occurs in the sacrum. Though it is mostly classified as benign, the prognosis is unpredictable due to the possibility of recurrence and metastasis. Here, we reported a case of intraosseous of sacrum with good results and reviewed the literature.

CASE PRESENTATION

A 33-year-old male patient complained of low back pain and was discovered to have an obstruction at S2. Following the necessary imaging diagnosis, we treated the patient with piecemeal excision in conjunction with extended curettage, and the frozen biopsy revealed that the tumor was melanotic schwannoma. The intraosseous portion of the lesion was curettaged using high-speed drill to enlarge the edge of curettage, and piecemeal excision for lesion within the sacral canal. After surgery, the patient received total 56Gy radiotherapy and frequent follow-up. After 15 months follow-up, there was no evidence of recurrence, and the nerve function was normal.

CONCLUSION

Melanotic schwannoma that occurs intraosseous of the sacrum is extremely rare and lacks typical clinical manifestations; however it can be identified through careful pathological and imaging diagnosis. Intralesional extended curettage combined with radiotherapy can achieve a good local control with a satisfactory clinical effect in this rare disease.

摘要

背景

黑色素神经鞘瘤发生在骶骨时较为罕见。尽管大多数情况下被归类为良性,但由于存在复发和转移的可能性,其预后难以预测。在此,我们报告了一例骶骨骨内黑色素神经鞘瘤的病例,并复习了相关文献。

病例介绍

一名 33 岁男性患者因腰痛就诊,发现 S2 有梗阻。经过必要的影像学诊断后,我们采用分块切除联合扩大刮除术对患者进行治疗,冷冻活检显示肿瘤为黑色素神经鞘瘤。采用高速钻头刮除病变的骨内部分,扩大刮除边缘,并对骶管内的病变进行分块切除。术后,患者接受了 56Gy 的全剂量放疗,并进行了频繁的随访。15 个月的随访后,未见复发迹象,神经功能正常。

结论

骶骨骨内黑色素神经鞘瘤极为罕见,缺乏典型的临床表现,但通过仔细的病理和影像学诊断可以明确诊断。对于这种罕见疾病,病灶内广泛刮除联合放疗可实现良好的局部控制,获得满意的临床效果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f05/9891983/d01eb2e8fe63/OS-15-655-g002.jpg

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