Zico Isma Azam, Anam Jahinul, Khalil Ibrahim
Department of Neurosurgery, Dhaka Medical College and Hospital, Dhaka, Bangladesh.
Dhaka Medical College and Hospital, Dhaka, Bangladesh.
Radiol Case Rep. 2025 May 15;20(8):3755-3760. doi: 10.1016/j.radcr.2025.04.049. eCollection 2025 Aug.
Intramedullary epidermoid cysts are rare, benign spinal tumors originating from ectodermal tissue during embryogenesis, comprising less than 1% of intraspinal lesions and posing diagnostic challenges due to their variable presentation and imaging overlap with other spinal pathologies. This case report describes a 22-year-old female who presented with progressive bilateral lower limb weakness, mid-back pain, and bowel-bladder incontinence, with preoperative MRI revealing a well-circumscribed intradural cystic lesion at the T5-T6 level exhibiting mixed-intensity patterns and a "shouldering effect," initially suggesting an extramedullary location. Surgical exploration, however, confirmed an intramedullary position, and the lesion-characterized intraoperatively as a white, waxy, flaky mass-was excised en-bloc, with histopathology verifying an epidermoid cyst; the patient showed significant neurological recovery by her 12-month follow-up with no recurrence. This case highlights a critical discrepancy between MRI and intraoperative findings, emphasizing the need to integrate clinical, radiological, and surgical observations for accurate diagnosis and management of such rare spinal lesions. It underscores that intramedullary epidermoid cysts, though uncommon, should remain in the differential diagnosis for young adults with progressive neurological deficits, particularly in the thoracic spine, and that complete surgical resection is essential to prevent recurrence. Early diagnosis, meticulous surgical planning, and long-term follow-up are vital for optimizing outcomes, illustrating the importance of adaptability when imaging misaligns with operative reality and reinforcing the value of detailed case reporting to advance understanding of this rare pathology.
髓内表皮样囊肿是一种罕见的良性脊柱肿瘤,起源于胚胎发育过程中的外胚层组织,占脊柱内病变的比例不到1%,由于其表现多样且在影像学上与其他脊柱病变存在重叠,给诊断带来了挑战。本病例报告描述了一名22岁女性,她出现进行性双侧下肢无力、中背部疼痛和膀胱直肠失禁,术前MRI显示T5 - T6水平有一个边界清晰的硬膜内囊性病变,呈现混合强度模式和“肩部效应”,最初提示为髓外位置。然而,手术探查证实为髓内位置,术中将病变(其特征为白色、蜡样、片状肿块)整块切除,组织病理学证实为表皮样囊肿;患者在12个月的随访中神经功能有显著恢复,且无复发。该病例突出了MRI与术中发现之间的关键差异,强调需要综合临床、放射学和手术观察结果,以准确诊断和处理此类罕见的脊柱病变。它强调,髓内表皮样囊肿虽然不常见,但对于出现进行性神经功能缺损的年轻成年人,尤其是胸椎病变,仍应列入鉴别诊断范围,并且完整的手术切除对于预防复发至关重要。早期诊断、精心的手术规划和长期随访对于优化治疗结果至关重要,这说明了当影像学结果与手术实际情况不符时灵活性的重要性,并强化了详细病例报告对于增进对这种罕见病理认识的价值。