Base of skull chordomas in children and adolescents: A clinicopathologic study of 73 cases

Benjamin L. Hoch, Gunnlaugur P. Nielsen, Norbert J. Liebsch, Andrew Rosenberg

Research output: Contribution to journalArticle

96 Citations (Scopus)

Abstract

Chordomas in children and adolescents comprise <5% of all chordomas and most frequently develop in the skull base. These tumors are believed to behave more aggressively than chordomas in adults and may have unusual morphology. This study examines a large series of pediatric skull base chordomas treated with a standardized protocol to characterize the behavior and morphology of these tumors. There were 31 males and 42 females ranging from 1 to 18 (mean 9.7) years. Forty-two cases (58%) were conventional chordomas, some of which had unusual histopathologic features. Chondroid chordomas comprised 23% of cases. Fourteen tumors (19%) were highly cellular and had a solid growth pattern with no myxoid matrix or lobular architecture. Eight of these had cytologic features of conventional chordoma cells including physaliferous cells (cellular chordoma). The remaining cellular tumors were composed of poorly differentiated epithelioid cells set in a fibrous stroma and lacked physaliferous cells (poorly differentiated chordoma). All variants studied by immunohistochemistry showed positive staining for cytokeratin, epithelial membrane antigen, S100 protein, and vimentin. Mitoses and necrosis were seen in all variants. Follow-up data were available for all patients and ranged from 1 to 21 (mean 7.25) years. The survival rate was 81%. All but 1 patient with poorly differentiated chordoma died of disease. Overall, base of skull chordomas in children and adolescents treated with proton beam radiation have better survival than chordomas in adults. However, poorly differentiated chordomas are highly aggressive tumors.

Original languageEnglish
Pages (from-to)811-818
Number of pages8
JournalAmerican Journal of Surgical Pathology
Volume30
Issue number7
DOIs
StatePublished - Jul 1 2006
Externally publishedYes

Fingerprint

Chordoma
Skull Base
Neoplasms
Epithelioid Cells
Mucin-1
S100 Proteins
Vimentin
Keratins
Mitosis
Protons

Keywords

  • Adolescents
  • Children
  • Chordoma
  • Skull base

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine

Cite this

Base of skull chordomas in children and adolescents : A clinicopathologic study of 73 cases. / Hoch, Benjamin L.; Nielsen, Gunnlaugur P.; Liebsch, Norbert J.; Rosenberg, Andrew.

In: American Journal of Surgical Pathology, Vol. 30, No. 7, 01.07.2006, p. 811-818.

Research output: Contribution to journalArticle

Hoch, Benjamin L. ; Nielsen, Gunnlaugur P. ; Liebsch, Norbert J. ; Rosenberg, Andrew. / Base of skull chordomas in children and adolescents : A clinicopathologic study of 73 cases. In: American Journal of Surgical Pathology. 2006 ; Vol. 30, No. 7. pp. 811-818.
@article{37ef5c4e7adc4ca5afbb9d0c4bc58c3f,
title = "Base of skull chordomas in children and adolescents: A clinicopathologic study of 73 cases",
abstract = "Chordomas in children and adolescents comprise <5{\%} of all chordomas and most frequently develop in the skull base. These tumors are believed to behave more aggressively than chordomas in adults and may have unusual morphology. This study examines a large series of pediatric skull base chordomas treated with a standardized protocol to characterize the behavior and morphology of these tumors. There were 31 males and 42 females ranging from 1 to 18 (mean 9.7) years. Forty-two cases (58{\%}) were conventional chordomas, some of which had unusual histopathologic features. Chondroid chordomas comprised 23{\%} of cases. Fourteen tumors (19{\%}) were highly cellular and had a solid growth pattern with no myxoid matrix or lobular architecture. Eight of these had cytologic features of conventional chordoma cells including physaliferous cells (cellular chordoma). The remaining cellular tumors were composed of poorly differentiated epithelioid cells set in a fibrous stroma and lacked physaliferous cells (poorly differentiated chordoma). All variants studied by immunohistochemistry showed positive staining for cytokeratin, epithelial membrane antigen, S100 protein, and vimentin. Mitoses and necrosis were seen in all variants. Follow-up data were available for all patients and ranged from 1 to 21 (mean 7.25) years. The survival rate was 81{\%}. All but 1 patient with poorly differentiated chordoma died of disease. Overall, base of skull chordomas in children and adolescents treated with proton beam radiation have better survival than chordomas in adults. However, poorly differentiated chordomas are highly aggressive tumors.",
keywords = "Adolescents, Children, Chordoma, Skull base",
author = "Hoch, {Benjamin L.} and Nielsen, {Gunnlaugur P.} and Liebsch, {Norbert J.} and Andrew Rosenberg",
year = "2006",
month = "7",
day = "1",
doi = "10.1097/01.pas.0000209828.39477.ab",
language = "English",
volume = "30",
pages = "811--818",
journal = "American Journal of Surgical Pathology",
issn = "0147-5185",
publisher = "Lippincott Williams and Wilkins",
number = "7",

}

TY - JOUR

T1 - Base of skull chordomas in children and adolescents

T2 - A clinicopathologic study of 73 cases

AU - Hoch, Benjamin L.

AU - Nielsen, Gunnlaugur P.

AU - Liebsch, Norbert J.

AU - Rosenberg, Andrew

PY - 2006/7/1

Y1 - 2006/7/1

N2 - Chordomas in children and adolescents comprise <5% of all chordomas and most frequently develop in the skull base. These tumors are believed to behave more aggressively than chordomas in adults and may have unusual morphology. This study examines a large series of pediatric skull base chordomas treated with a standardized protocol to characterize the behavior and morphology of these tumors. There were 31 males and 42 females ranging from 1 to 18 (mean 9.7) years. Forty-two cases (58%) were conventional chordomas, some of which had unusual histopathologic features. Chondroid chordomas comprised 23% of cases. Fourteen tumors (19%) were highly cellular and had a solid growth pattern with no myxoid matrix or lobular architecture. Eight of these had cytologic features of conventional chordoma cells including physaliferous cells (cellular chordoma). The remaining cellular tumors were composed of poorly differentiated epithelioid cells set in a fibrous stroma and lacked physaliferous cells (poorly differentiated chordoma). All variants studied by immunohistochemistry showed positive staining for cytokeratin, epithelial membrane antigen, S100 protein, and vimentin. Mitoses and necrosis were seen in all variants. Follow-up data were available for all patients and ranged from 1 to 21 (mean 7.25) years. The survival rate was 81%. All but 1 patient with poorly differentiated chordoma died of disease. Overall, base of skull chordomas in children and adolescents treated with proton beam radiation have better survival than chordomas in adults. However, poorly differentiated chordomas are highly aggressive tumors.

AB - Chordomas in children and adolescents comprise <5% of all chordomas and most frequently develop in the skull base. These tumors are believed to behave more aggressively than chordomas in adults and may have unusual morphology. This study examines a large series of pediatric skull base chordomas treated with a standardized protocol to characterize the behavior and morphology of these tumors. There were 31 males and 42 females ranging from 1 to 18 (mean 9.7) years. Forty-two cases (58%) were conventional chordomas, some of which had unusual histopathologic features. Chondroid chordomas comprised 23% of cases. Fourteen tumors (19%) were highly cellular and had a solid growth pattern with no myxoid matrix or lobular architecture. Eight of these had cytologic features of conventional chordoma cells including physaliferous cells (cellular chordoma). The remaining cellular tumors were composed of poorly differentiated epithelioid cells set in a fibrous stroma and lacked physaliferous cells (poorly differentiated chordoma). All variants studied by immunohistochemistry showed positive staining for cytokeratin, epithelial membrane antigen, S100 protein, and vimentin. Mitoses and necrosis were seen in all variants. Follow-up data were available for all patients and ranged from 1 to 21 (mean 7.25) years. The survival rate was 81%. All but 1 patient with poorly differentiated chordoma died of disease. Overall, base of skull chordomas in children and adolescents treated with proton beam radiation have better survival than chordomas in adults. However, poorly differentiated chordomas are highly aggressive tumors.

KW - Adolescents

KW - Children

KW - Chordoma

KW - Skull base

UR - http://www.scopus.com/inward/record.url?scp=33746922386&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=33746922386&partnerID=8YFLogxK

U2 - 10.1097/01.pas.0000209828.39477.ab

DO - 10.1097/01.pas.0000209828.39477.ab

M3 - Article

C2 - 16819322

AN - SCOPUS:33746922386

VL - 30

SP - 811

EP - 818

JO - American Journal of Surgical Pathology

JF - American Journal of Surgical Pathology

SN - 0147-5185

IS - 7

ER -