TY - JOUR
T1 - Metastasizing chordoma in early childhood
T2 - A pathological and immunohistochemical study with review of the literature
AU - Sibley, Richard K.
AU - Day, Deborah L.
AU - Dehner, Louis P.
AU - Trueworthy, Robert C.
PY - 1987/1/1
Y1 - 1987/1/1
N2 - A 21\2year-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..
AB - A 21\2year-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..
KW - Chordoma
KW - Clivus
KW - Lung metastasis
UR - http://www.scopus.com/inward/record.url?scp=0023491617&partnerID=8YFLogxK
U2 - 10.1080/15513818709177131
DO - 10.1080/15513818709177131
M3 - Article
C2 - 3684809
AN - SCOPUS:0023491617
SN - 1551-3815
VL - 7
SP - 287
EP - 301
JO - Fetal and Pediatric Pathology
JF - Fetal and Pediatric Pathology
IS - 3
ER -