P.068 脊髓皮样囊肿恶变为癌肉瘤

JA Chaiton
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摘要

背景:脊柱蝶形囊肿是一种不常见的外胚层良性肿瘤,通常与脊柱发育不良有关。脊髓皮样囊肿的恶性变是一种非常罕见的病变,以前从未报道过恶性变为癌肉瘤的病例。方法:病例报告和文献综述病例报告和文献综述 结果:一名 41 岁的男性在切除真皮窦道和相关的皮样囊肿 28 年后,出现复发性腰椎硬膜内肿块。术中外观和随后的病理结果再次与皮样囊肿一致。术后两周,患者再次因脑积水出现复视和头痛,被认为是化学性脑膜炎所致。植入脑室腹腔分流术后,患者病情迅速恶化,出现进行性神经功能缺损和广泛的脑膜强化。患者再次接受脊髓脑膜活检,结果显示蝶形囊肿恶变为浸润性癌肉瘤。由于没有根治性治疗方案,患者只能接受姑息治疗,入院85天后死亡。结论在对患有蝶形囊肿和进行性脑膜强化的患者进行鉴别诊断时,应考虑脊髓蝶形囊肿的恶性转化。最初的肿瘤病理学检查可能会出现假阴性,因此可能需要重复取样以明确诊断。据作者所知,这是首例脊髓蝶窦囊肿恶变为癌肉瘤的报告。
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P.068 Malignant transformation of a spinal dermoid cyst into carcinosarcoma
Background: Spinal dermoid cysts are uncommon, benign tumours of ectodermal origin, often associated with spinal dysraphism. Malignant transformation of spinal dermoid cysts is an exceptionally rare entity, with transformation into carcinosarcoma not previously reported. Methods: Case report and literature review Results: A 41-year-old male presented with a recurrent lumbar intradural mass, 28 years after resection of a dermal sinus tract and associated dermoid cyst. Intraoperative appearance and subsequent pathology were again consistent with a dermoid cyst. The patient re-presented 2 weeks after surgery with diplopia and headache due to hydrocephalus, thought to be due to chemical meningitis. Following ventriculoperitoneal shunt implantation, the patient rapidly deteriorated with progressive neurologic deficits and widespread leptomeningeal enhancement. A repeat spinal leptomeningeal biopsy was pursued, which revealed malignant transformation of the dermoid cyst into invasive carcinosarcoma. Without curative treatment options, the patient was palliated and died 85 days after admission. Conclusions: Malignant transformation of spinal dermoid cysts should be considered in the differential diagnosis of patients with dermoid cysts and progressive leptomeningeal enhancement. False negatives can occur with initial tumour pathology and repeat sampling may be warranted for diagnostic clarity. To the authors knowledge, this is the first report of a spinal dermoid cyst with malignant transformation into carcinosarcoma.
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