Kaufman B A, Francel P C, Roberts R L, Argemand E, Park T S, Dehner L P
Department of Neurosurgery, Washington University School of Medicine, St. Louis, Mo 63110, USA.
Pediatr Neurosurg. 1995;23(3):159-65. doi: 10.1159/000120953.
Clinical, radiological, and pathologic features of an intracranial chondroid chordoma in a 9-year-old boy are described. This is the first reported case of a chordoma, the center of which was laterally situated in the cranial base, lying in or near jugular foramen and carotid canal, but without midline involvement. Although cranial chordomas in childhood are extremely rare, and all previously reported cases appeared to have arisen in the clivus, this location should not be considered ectopic. Forking at the rostral end of the notochord has been demonstrated in embryos, and would be the presumed embryological source for this tumor. This is also the first reported case of a chondroid chordoma in a child with immunohistochemical documentation distinguishing it from a chondrosarcoma. This chondroid chordoma contained two populations of cells: neoplastic cartilage and chordoid tissue in a myxoid stroma. The distinction between chordoma and chondrosarcoma and the implications on treatment will be discussed.
本文描述了一名9岁男孩颅内软骨样脊索瘤的临床、放射学及病理学特征。这是首例报道的脊索瘤病例，其瘤体中心位于颅底外侧，位于颈静脉孔和颈动脉管内或其附近，但未累及中线。尽管儿童期颅脊索瘤极为罕见，且既往报道的所有病例似乎均起源于斜坡，但该位置不应被视为异位。在胚胎中已证实脊索前端存在分叉，这可能是该肿瘤的胚胎学起源。这也是首例有免疫组化证据的儿童软骨样脊索瘤病例，可将其与软骨肉瘤区分开来。该软骨样脊索瘤包含两种细胞成分：肿瘤性软骨和黏液样基质中的脊索样组织。本文将讨论脊索瘤与软骨肉瘤的鉴别以及对治疗的影响。
