Géhanne C, Delpierre I, Damry N, Devroede B, Brihaye P, Christophe C
Department of Paediatric Radiology, Queen Fabiola University Hospital for Children, Brussels, Belgium.
JBR-BTR. 2005 Nov-Dec;88(6):325-7.
We report the case of a 10-year-old boy who presented an intracranial chordoma and we stress the importance of sectional imaging to approach the diagnosis. Clinical presentation is related to local invasion. Lesion of the body of the clivus can extend ventrally or dorsally and cause cranial nerve palsies, brain stem compression or hydrocephalus. Pathologically, they form soft, grey masses that are histologically benign but locally invasive and destructive. Both computed tomography (CT) and magnetic resonance (MR) imaging clearly depict the lesion. CT better demonstrates bone destruction and intralesional calcifications. In our case, MR was performed first and showed all the classical signs of skull base chordoma. CT provided complementary information about bone destruction which are also usual in this type of lesion. Major differential diagnoses of the chordoma in the clivus are the other central skull base masses. Biopsy and histology make the diagnosis. Usually treatment consists in surgery and radiotherapy but more recently proton beam therapy is used and seems to yield better results.
我们报告了一例10岁男孩患颅内脊索瘤的病例，并强调断层成像在诊断中的重要性。临床表现与局部侵犯有关。斜坡体部病变可向腹侧或背侧延伸，导致颅神经麻痹、脑干受压或脑积水。病理上，它们形成柔软的灰色肿块，组织学上为良性，但具有局部侵袭性和破坏性。计算机断层扫描（CT）和磁共振成像（MR）均能清晰显示病变。CT能更好地显示骨质破坏和瘤内钙化。在我们的病例中，首先进行了MR检查，显示了颅底脊索瘤的所有典型征象。CT提供了关于骨质破坏的补充信息，这在这类病变中也很常见。斜坡脊索瘤的主要鉴别诊断是其他颅底中央肿块。活检和组织学检查可明确诊断。通常治疗包括手术和放疗，但最近质子束治疗也被采用，且似乎能取得更好的效果。
