Metastasizing chordoma in early childhood: A pathological and immunohistochemical study with review of the literature

Richard K. Sibley; Deborah L. Day; Louis P. Dehner; Robert C. Trueworthy

doi:10.1080/15513818709177131

Metastasizing chordoma in early childhood: A pathological and immunohistochemical study with review of the literature

Richard K. Sibley
, Deborah L. Day
, Louis P. Dehner
, Robert C. Trueworthy

Division of Anatomic and Molecular Pathology (AMP)

Research output: Contribution to journal › Article › peer-review

50 Scopus citations

Abstract

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..

Original language	English
Pages (from-to)	287-301
Number of pages	15
Journal	Fetal and Pediatric Pathology
Volume	7
Issue number	3
DOIs	https://doi.org/10.1080/15513818709177131
State	Published - Jan 1 1987

Keywords

Chordoma
Clivus
Lung metastasis

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10.1080/15513818709177131

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title = "Metastasizing chordoma in early childhood: A pathological and immunohistochemical study with review of the literature",

abstract = "A 21\textbackslash{}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..",

keywords = "Chordoma, Clivus, Lung metastasis",

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year = "1987",

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doi = "10.1080/15513818709177131",

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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.

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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

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DO - 10.1080/15513818709177131

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JO - Fetal and Pediatric Pathology

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ER -

Metastasizing chordoma in early childhood: A pathological and immunohistochemical study with review of the literature

Abstract

Keywords

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