酷似肿瘤的脊髓炎性脱髓鞘假瘤

Tumer-like inflammatory demyelinating pseudotumor in the spinal cord

目的总结脊髓脱髓鞘假瘤的临床、影像学和病理特点。方法回顾分析6例脊髓脱髓鞘假瘤的临床、影像学特征,对其中2例误诊为髓内肿瘤术后病理表现进行分析。结果脊髓脱髓鞘假瘤常以急性或亚急性起病,最常累及颈髓和胸髓,可伴有周围神经受损;脊髓脱髓鞘假瘤单时相起病,症状、体征提示为单病灶;MRI检查病灶多单发(偶可多发),占位水肿效应明显,增强后可呈非闭合性花环样或环形强化;病理为炎性脱髓鞘改变,白质内髓鞘破坏并水肿,病灶内以小血管周围大量淋巴细胞浸润、巨噬细胞聚集为主要表现;经激素治疗后好转。结论脊髓炎性脱髓鞘假瘤MRI酷似髓内肿瘤,容易造成误诊。可试用激素治疗或组织活检,不要急于手术。

Objective To discuss the clinical imaging and pathological characters of demyelinating pseudotumor in the spinal cord. Methods A retrospective analysis was done to the clinical imaging and pathological characters of 6 demyelinating pseudotumor in the spinal cord. 2 of them misdiagnosed as tumor were specially analyzed in terms of their features in pathological manifestations. Results Spinal demyelinating pseudotumor, mostly seen in middle age with acute or subacute onset, involves cervical and thoracic spinal cord exclusively. Symptoms, signs and MRI showed a single focal lesion in every case. Occasionally MRI showed multiple loci as well as evident space-occupying edema and even close chaplet-like form by enhancement. Pathologically it showed to be typical inflammatory demyalination, combined with myelinoclasis and edema in the white matter. Inside the loci, a large number of achroacyte infiltrated into and lots of macrophagocytes were aggregated around the small vessels. Clinically hormone therapy by using glucocorticoids was effective for its improvement. Conclusion Spinal demyelinating pseudotumor resembled intraspinal neoplasm clinically and radiologically so that it was liable to be misdiagnosed. For its differential diagnosis, biopsy or glucocorticoids for trial treatment are suggested rather than surgical operation.