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Metastasizing chordoma in early childhood: a pathological and immunohistochemical study with review of the literature. 1987

R K Sibley, and D L Day, and L P Dehner, and R C Trueworthy
Department of Laboratory Medicine, University of Minnesota Medical School and Hospital, Minneapolis 55455.

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.

UI MeSH Term Description Entries
D008175 Lung Neoplasms Tumors or cancer of the LUNG. Cancer of Lung,Lung Cancer,Pulmonary Cancer,Pulmonary Neoplasms,Cancer of the Lung,Neoplasms, Lung,Neoplasms, Pulmonary,Cancer, Lung,Cancer, Pulmonary,Cancers, Lung,Cancers, Pulmonary,Lung Cancers,Lung Neoplasm,Neoplasm, Lung,Neoplasm, Pulmonary,Pulmonary Cancers,Pulmonary Neoplasm
D009360 Neoplastic Cells, Circulating Exfoliate neoplastic cells circulating in the blood and associated with metastasizing tumors. Circulating Neoplastic Cells,Embolic Tumor Cells,Embolism, Tumor,Neoplasm Circulating Cells,Tumor Cells, Embolic,Cells, Neoplasm Circulating,Circulating Cells, Neoplasm,Circulating Tumor Cells,Cell, Circulating Neoplastic,Cell, Circulating Tumor,Cell, Embolic Tumor,Cell, Neoplasm Circulating,Cells, Circulating Neoplastic,Cells, Circulating Tumor,Cells, Embolic Tumor,Circulating Neoplastic Cell,Circulating Tumor Cell,Embolic Tumor Cell,Embolisms, Tumor,Neoplasm Circulating Cell,Neoplastic Cell, Circulating,Tumor Cell, Circulating,Tumor Cell, Embolic,Tumor Cells, Circulating,Tumor Embolism,Tumor Embolisms
D009777 Occipital Bone Part of the back and base of the CRANIUM that encloses the FORAMEN MAGNUM. Basilar Bone,Basilar Bones,Bone, Basilar,Bone, Occipital,Bones, Basilar,Bones, Occipital,Occipital Bones
D001859 Bone Neoplasms Tumors or cancer located in bone tissue or specific BONES. Bone Cancer,Cancer of Bone,Cancer of the Bone,Neoplasms, Bone,Bone Neoplasm,Neoplasm, Bone
D002574 Cervical Vertebrae The first seven VERTEBRAE of the SPINAL COLUMN, which correspond to the VERTEBRAE of the NECK. Cervical Spine,Cervical Spines,Spine, Cervical,Vertebrae, Cervical
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D002817 Chordoma A malignant tumor arising from the embryonic remains of the notochord. It is also called chordocarcinoma, chordoepithelioma, and notochordoma. (Dorland, 27th ed) Chordomas
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013100 Sphenoid Bone An irregular unpaired bone situated at the SKULL BASE and wedged between the frontal, temporal, and occipital bones (FRONTAL BONE; TEMPORAL BONE; OCCIPITAL BONE). Sphenoid bone consists of a median body and three pairs of processes resembling a bat with spread wings. The body is hollowed out in its inferior to form two large cavities (SPHENOID SINUS). Greater Sphenoid Wing,Bone, Sphenoid,Greater Sphenoid Wings,Sphenoid Bones,Sphenoid Wing, Greater

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