Ramesh Shighakolli, Subodh Raju, Boppana Srinadh, Jayashankar Erukkambattu
Department of Neurosurgery, Kamineni Hospitals, Hyderabad, Telangana, India.
Department of Radio Diagnosis, Kamineni Academy of Medical Sciences and Research Centre, Hyderabad, Telangana, India.
J Pediatr Neurosci. 2017 Apr-Jun;12(2):156-159. doi: 10.4103/jpn.JPN_157_16.
Chordomas are primary malignant bone tumors that arise in the axial skeleton, believed to originate from remnants of embryologic notochordal cell rests. Multicentric origin of chordoma is extremely rare. To our literature search, we found only three cases of multicentric chordoma in adults. We report a first case of multicentric chordoma in pediatric age group. A 14-month-old child presented with torticolis and left upper limb monoparesis, imaging showed expansile bony destructive lesion in clivus and dorsal spine simultaneously. The child underwent laminectomy, decompression of cord, excision of lesion, and histopathology was suggestive of chordoma. Pediatric chordomas are aggressive tumors, require multidisciplinary management with maximal safe resection followed by radiotherapy (conventional and/or proton). Even with multidisciplinary management, pediatric chordomas have high morbidity and mortality.
脊索瘤是一种发生于中轴骨骼的原发性恶性骨肿瘤，被认为起源于胚胎脊索细胞残余。脊索瘤多中心起源极为罕见。经文献检索，我们仅发现3例成人多中心脊索瘤病例。我们报告1例儿童多中心脊索瘤病例。一名14个月大的儿童出现斜颈和左上肢单瘫，影像学检查显示斜坡和胸椎同时出现膨胀性骨质破坏病变。该患儿接受了椎板切除术、脊髓减压、病变切除术，组织病理学提示为脊索瘤。儿童脊索瘤是侵袭性肿瘤，需要多学科综合治疗，包括最大程度的安全切除，随后进行放疗（传统放疗和/或质子放疗）。即使采用多学科综合治疗，儿童脊索瘤的发病率和死亡率仍很高。
