Metastasizing chordoma in early childhood: a pathological and immunohistochemical study with review of the literature.
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.
Sibley
R K
RK
Department of Laboratory Medicine, University of Minnesota Medical School and Hospital, Minneapolis 55455.
Day
D L
DL
Dehner
L P
LP
Trueworthy
R C
RC
D001859:Bone Neoplasms / Q000473:pathology*; D002574:Cervical Vertebrae / Q000473:pathology; D002675:Child, Preschool; D002817:Chordoma / Q000473:pathology* / Q000556:secondary; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000556:secondary; D009360:Neoplastic Cells, Circulating; D009777:Occipital Bone / Q000473:pathology; D013100:Sphenoid Bone / Q000473:pathology