Department of Orthopedic Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Department of Orthopedic Surgery, Okazaki City Hospital, Okazaki, Japan.
Nagoya J Med Sci. 2022 Aug;84(3):656-663. doi: 10.18999/nagjms.84.3.656.
We report the case of a rare lipoma arising in the epidural space of a 14-year-old boy without spinal dysraphism. Lipomas are rare in pediatric soft tissue tumors, accounting for only about 4% of cases. The incidence of an intraspinal epidural lipoma without spinal dysraphism is extremely rare in pediatric patients. In this case, the patient had progressive motor deficits in the lower extremities and difficulty in urination and defecation. Magnetic resonance imaging showed an extradural tumor compressing the spinal cord at the T3-T7 level. Because of the progressive neurological deficits, we performed an emergency surgery. The tumor was completely resected en bloc, and histopathology revealed mature adipose tissue with fibrous septa, diagnosed as atypical lipomatous tumor / well-differentiated liposarcoma. The patient fully recovered and there was no tumor recurrence for 6 years since the surgery. However, re-examination using fluorescence in situ hybridization after 6 years of surgery changed the diagnosis to lipoma as no amplification of murine double-minute type 2 oncogene was observed. In liposarcoma, histopathological diagnosis using fluorescence in situ hybridization is mandatory. Our case illustrates that immunohistochemical diagnosis alone can be misleading. Hence, prompt surgery is required for progressive neuropathy.
我们报告了一例发生在 14 岁男孩硬膜外间隙的罕见脂肪瘤,该男孩无脊髓脊膜裂。脂肪瘤在儿科软组织肿瘤中较为罕见,仅占约 4%。在儿科患者中,无脊髓脊膜裂的椎管内硬膜外脂肪瘤的发病率极低。在本例中,患者出现下肢进行性运动功能障碍,伴有排尿和排便困难。磁共振成像显示 T3-T7 水平硬膜外肿瘤压迫脊髓。由于存在进行性神经功能缺损,我们进行了紧急手术。整块切除肿瘤,组织病理学显示成熟脂肪组织伴纤维间隔,诊断为非典型性脂肪肉瘤/高分化脂肪肉瘤。患者完全康复,手术后 6 年无肿瘤复发。然而,手术后 6 年使用荧光原位杂交进行复查时,改变了诊断为脂肪瘤,因为未观察到鼠双微体 2 癌基因的扩增。在脂肪肉瘤中,荧光原位杂交的组织病理学诊断是强制性的。我们的病例表明,单独的免疫组织化学诊断可能具有误导性。因此,对于进行性神经病,需要及时进行手术。