Sibley R K, Day D L, Dehner L P, Trueworthy R C
Department of Laboratory Medicine, University of Minnesota Medical School and Hospital, Minneapolis 55455.
Pediatr Pathol. 1987;7(3):287-301. doi: 10.1080/15513818709177131.
A 2 1/2-year-old female with a sphenooccipital-vertebral chordoma presented with neck pain, torticollis, fever, a lytic lesion of C2 vertebra, and bilateral nodular infiltrates in the lung. The lung biopsy revealed multiple tumor emboli by an enigmatic epithelioid-appearing neoplasm with immunohistochemical staining for vimentin, cytokeratin, and epithelial membrane antigen. A thorough roentgenographic evaluation disclosed a destructive, prepontine mass in the region of the clivus, erosion of the odontoid process, and compression of the cervical spinal cord. The patient died after a clinical course of 3 months. We identified 16 additional cases of metastasizing chordomas in the pediatric-age population; this case is the first to our knowledge with pathologically documented pulmonary metastasis at presentation.
一名2岁半的女性患有蝶枕-椎体脊索瘤，表现为颈部疼痛、斜颈、发热、C2椎体溶骨性病变以及肺部双侧结节状浸润。肺活检显示多个肿瘤栓子，由一种形态神秘的上皮样肿瘤构成，该肿瘤对波形蛋白、细胞角蛋白和上皮膜抗原进行免疫组化染色。全面的X线评估显示斜坡区域有一个破坏性的脑桥前肿块、齿突侵蚀以及颈髓受压。该患者在3个月的临床病程后死亡。我们在儿童年龄组中又发现了16例转移性脊索瘤病例；据我们所知，该病例是首例在就诊时经病理证实有肺转移的病例。
