Department of Pediatric Neurosurgery, Matsudo City General Hospital, Chiba, Japan.
Pediatr Neurosurg. 2023;58(3):168-172. doi: 10.1159/000531548. Epub 2023 Jun 14.
Lipoblastoma and lipoblastomatosis are rare benign mesenchymal adipose tumors that originate from embryonic white adipocytes and occur most commonly in infancy and early childhood. Lipoblastomas occur in the extremities and trunk, including the retroperitoneum and peritoneal cavity. Therefore, infiltration into the spinal canal has rarely been reported.
A 4-year-old girl presented to our clinic because of difficulty sitting on the floor with her legs straight. She also complained of enuresis and constipation for the past 6 months with persistent headaches and back pain evoked by body anteflexion. A magnetic resonance imaging revealed a massive lesion of the psoas major muscle, retroperitoneal, and subcutaneous spaces, extending into the spinal epidural space between L2 and S1. The patient underwent surgery which resulted in gross total removal of the tumor from the spinal canal. The mass was yellowish, soft, lobulated, fatty, and easily removed from the surrounding structures. Pathology confirmed the diagnosis of lipoblastoma. The postoperative course was uneventful, and the patient was discharged without any signs of neurological deficit.
We herein discuss a rare case of lipoblastoma extending into the spinal canal, resulting in neurological symptoms. Although this tumor is benign with no potential for metastasis, it is prone to local recurrence. Therefore, close postoperative observation should be performed.
脂肪母细胞瘤和脂肪母细胞瘤病是罕见的良性间叶脂肪肿瘤,起源于胚胎白色脂肪细胞,最常发生于婴儿和幼儿期。脂肪母细胞瘤发生于四肢和躯干,包括腹膜后和腹腔。因此,很少有报道称其浸润椎管。
一名 4 岁女孩因双腿无法伸直坐在地板上而到我院就诊。她还主诉过去 6 个月有遗尿和便秘,并且身体前屈时会出现持续性头痛和背痛。磁共振成像显示腰大肌、腹膜后和皮下间隙有一个巨大病变,延伸至 L2 和 S1 之间的椎管硬膜外间隙。患者接受了手术,从椎管内大体上完全切除了肿瘤。肿块呈黄色,柔软,分叶状,脂肪样,很容易从周围结构中移除。病理检查证实了脂肪母细胞瘤的诊断。术后过程顺利,患者出院时没有任何神经功能缺损的迹象。
我们在此讨论了一例罕见的脂肪母细胞瘤延伸至椎管并导致神经症状的病例。尽管该肿瘤为良性,无转移潜能,但易局部复发。因此,应密切观察术后情况。