1991
DOI: 10.1227/00006123-199109000-00019
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Chordoma in Early Childhood: A Clinicopathological Study

Abstract: A case of clival chordoma in a 4-year-old girl is presented. The tumor regrew rapidly after it was partially removed, and the patient died after a clinical course of 11 months. An autopsy revealed a massive clival mass and widespread metastases in the dura mater, skull bone, bilateral lungs, liver, sternum, left humerus, and vertebrae. Pathological findings showed that the tumor cells were poorly differentiated, with a rare, but typical, physaliphorous appearance. The presence of epithelial differentiation pro… Show more

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Cited by 54 publications
(49 citation statements)
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“…Today, however, immunohistochemical studies permit differentiation between the two lesions. 20,27 Furthermore, the chondroid type is more often observed in young patients 2,7,8,11,16,35 and a better prognosis generally exists for younger patients as well. 10,17,35 According to Forsyth, et al, 10 age less than 40 years is the only significant factor associated with better prognosis.…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…Today, however, immunohistochemical studies permit differentiation between the two lesions. 20,27 Furthermore, the chondroid type is more often observed in young patients 2,7,8,11,16,35 and a better prognosis generally exists for younger patients as well. 10,17,35 According to Forsyth, et al, 10 age less than 40 years is the only significant factor associated with better prognosis.…”
Section: Discussionmentioning
confidence: 99%
“…Alternatively it seems that very young patients (Ͻ 5 years of age) have a very poor prognosis. 20,35 Tumor recurrence and death very often occur in less than 1 year, and there is a high frequency of metastasis. Sex has also been proposed as a prognosis factor 4 but this has not been confirmed.…”
Section: Discussionmentioning
confidence: 99%
“…Distant metastases are mainly pulmonary. 31,37 This pattern has been correlated with a higher proportion of atypical pathological variants compared with adults especially those <5 years of age (approx 65% vs. 20%). This age group is also remarkable for a higher proportion of SC sites (25% vs. 6%), compared with older children.…”
Section: Review Of Literaturementioning
confidence: 96%
“…Metastases are more common in the course of the disease with a pediatric predominance compared with adults: 9-58% range vs. 7-40%. 10,31,37 In these situations, they are frequently associated with a local failure. 38 Some patterns of such "combined" failures are more frequently reported such as local + nodal or local + surgical route.…”
Section: Review Of Literaturementioning
confidence: 99%
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