University of Cincinnati College of Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Spine (Phila Pa 1976). 2011 Jan 1;36(1):E69-73. doi: 10.1097/BRS.0b013e318201b7f5.
Case report.
To report a very rare case of juvenile xanthogranuloma (JXG) of the spine in an adult.
JXG is very rare in the spine, with only five prior reports in infants and children. To the best of our knowledge, this tumor has never been reported in an adult spine.
The patient is a 47-year-old woman who presented with bowel and bladder incontinence. Magnetic resonance imaging showed a very large lesion arising from the L2 vertebral body, with massive extension into the retroperitoneum with extensive intradural involvement. She had decreased rectal tone, had 4/5 strength in the right hip flexor, and had diminished sensation in her anterior right thigh and perineal region. She was otherwise neurologically intact. After preoperative embolization, a decompressive laminectomy was performed and the tumor was resected through a posterolateral transpedicular approach, followed by stabilization. Because of extensive involvement of retroperitoneum, complete resection was not possible.
After pathologic evaluation of the specimen, a diagnosis of JXG was made. Patient underwent postoperative radiation therapy, and her neurologic examination improved significantly over the next several months.
To the best of our knowledge, this is the first reported case of JXG in an adult spine. Although complete resection of the tumor was not possible, decompression of the dural sac followed by postoperative radiation led to an excellent clinical outcome.
病例报告。
报告一例成人脊柱幼年黄色肉芽肿(JXG)的罕见病例。
JXG 在脊柱中非常罕见,仅有五例先前在婴儿和儿童中报道过。据我们所知,这种肿瘤在成人脊柱中从未有过报道。
患者为 47 岁女性,表现为大小便失禁。磁共振成像显示起源于 L2 椎体的非常大的病变,大量延伸至腹膜后,广泛累及硬脊膜内。她直肠张力降低,右侧髋关节屈肌肌力为 4/5,前大腿和会阴区感觉减退。她的其他神经系统均完整。术前栓塞后,进行减压椎板切除术,并通过后路经椎弓根入路切除肿瘤,随后进行稳定。由于腹膜后广泛受累,无法进行完全切除。
标本病理评估后诊断为 JXG。患者接受术后放疗,其神经功能检查在接下来的几个月内显著改善。
据我们所知,这是首例成人脊柱 JXG 的报道。尽管无法完全切除肿瘤,但硬脊膜减压后术后放疗导致了极好的临床结果。