Extra-osseous Ewing sarcoma

Hendrik van den Berg; Richard C Heinen; Heleen J van der Pal; Johannes H M Merks

doi:10.1080/08880010902855581

Extra-osseous Ewing sarcoma

Hendrik van den Berg
, Richard C Heinen
, Heleen J van der Pal
, Johannes H M Merks

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

46 Citations (Scopus)

Abstract

BACKGROUND: Clinical data and data on outcome of extra-osseous Ewing tumors are scarce.

PROCEDURE: After a search for Ewing tumors in the database of a single institution over a period of 20 years, 16 out of 192 cases were found to have extra-osseous primary tumors.

RESULTS: Ages at initial diagnosis ranged from 2.5 to 17 years. Follow-up period ranged from 4 months to 24.8 years (mean 8.4 years). Eleven patients were treated according to protocols for Ewing tumors, while in 4 cases soft tissue protocols were used. In a single patient only surgery was done. Two patients had progressive disease despite chemotherapy; a third patient had only tumor response on the initial 2 chemotherapy courses. All 3 patients with initially metastatic disease died. One patient developed a second malignancy. Overall survival at 5 years was 75%. Event-free survival (EFS) at 5 years was 68%; for nonmetastatic patients 5-year EFS was 83%.

CONCLUSION: The authors conclude that nonmetastasized extra-osseous Ewing tumors have a prognosis at least similar to that of osseous Ewing tumors.

Original language	English
Pages (from-to)	175-85
Number of pages	11
Journal	Pediatric hematology and oncology
Volume	26
Issue number	4
DOIs	https://doi.org/10.1080/08880010902855581
Publication status	Published - Jul 2009
Externally published	Yes

Keywords

Adolescent
Antineoplastic Agents, Alkylating/therapeutic use
Antineoplastic Agents, Phytogenic/therapeutic use
Bone Neoplasms/drug therapy
Child
Child, Preschool
Dactinomycin/therapeutic use
Female
Follow-Up Studies
Humans
Ifosfamide/therapeutic use
Kaplan-Meier Estimate
Male
Prognosis
Protein Synthesis Inhibitors/therapeutic use
Sarcoma, Ewing/drug therapy
Treatment Outcome
Vincristine/therapeutic use

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title = "Extra-osseous Ewing sarcoma",

abstract = "BACKGROUND: Clinical data and data on outcome of extra-osseous Ewing tumors are scarce.PROCEDURE: After a search for Ewing tumors in the database of a single institution over a period of 20 years, 16 out of 192 cases were found to have extra-osseous primary tumors.RESULTS: Ages at initial diagnosis ranged from 2.5 to 17 years. Follow-up period ranged from 4 months to 24.8 years (mean 8.4 years). Eleven patients were treated according to protocols for Ewing tumors, while in 4 cases soft tissue protocols were used. In a single patient only surgery was done. Two patients had progressive disease despite chemotherapy; a third patient had only tumor response on the initial 2 chemotherapy courses. All 3 patients with initially metastatic disease died. One patient developed a second malignancy. Overall survival at 5 years was 75\%. Event-free survival (EFS) at 5 years was 68\%; for nonmetastatic patients 5-year EFS was 83\%.CONCLUSION: The authors conclude that nonmetastasized extra-osseous Ewing tumors have a prognosis at least similar to that of osseous Ewing tumors.",

keywords = "Adolescent, Antineoplastic Agents, Alkylating/therapeutic use, Antineoplastic Agents, Phytogenic/therapeutic use, Bone Neoplasms/drug therapy, Child, Child, Preschool, Dactinomycin/therapeutic use, Female, Follow-Up Studies, Humans, Ifosfamide/therapeutic use, Kaplan-Meier Estimate, Male, Prognosis, Protein Synthesis Inhibitors/therapeutic use, Sarcoma, Ewing/drug therapy, Treatment Outcome, Vincristine/therapeutic use",

author = "\{van den Berg\}, Hendrik and Heinen, \{Richard C\} and \{van der Pal\}, \{Heleen J\} and Merks, \{Johannes H M\}",

year = "2009",

month = jul,

doi = "10.1080/08880010902855581",

language = "English",

volume = "26",

pages = "175--85",

journal = "Pediatric hematology and oncology",

issn = "0888-0018",

publisher = "Taylor and Francis Ltd.",

number = "4",

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TY - JOUR

T1 - Extra-osseous Ewing sarcoma

AU - van den Berg, Hendrik

AU - Heinen, Richard C

AU - van der Pal, Heleen J

AU - Merks, Johannes H M

PY - 2009/7

Y1 - 2009/7

N2 - BACKGROUND: Clinical data and data on outcome of extra-osseous Ewing tumors are scarce.PROCEDURE: After a search for Ewing tumors in the database of a single institution over a period of 20 years, 16 out of 192 cases were found to have extra-osseous primary tumors.RESULTS: Ages at initial diagnosis ranged from 2.5 to 17 years. Follow-up period ranged from 4 months to 24.8 years (mean 8.4 years). Eleven patients were treated according to protocols for Ewing tumors, while in 4 cases soft tissue protocols were used. In a single patient only surgery was done. Two patients had progressive disease despite chemotherapy; a third patient had only tumor response on the initial 2 chemotherapy courses. All 3 patients with initially metastatic disease died. One patient developed a second malignancy. Overall survival at 5 years was 75%. Event-free survival (EFS) at 5 years was 68%; for nonmetastatic patients 5-year EFS was 83%.CONCLUSION: The authors conclude that nonmetastasized extra-osseous Ewing tumors have a prognosis at least similar to that of osseous Ewing tumors.

AB - BACKGROUND: Clinical data and data on outcome of extra-osseous Ewing tumors are scarce.PROCEDURE: After a search for Ewing tumors in the database of a single institution over a period of 20 years, 16 out of 192 cases were found to have extra-osseous primary tumors.RESULTS: Ages at initial diagnosis ranged from 2.5 to 17 years. Follow-up period ranged from 4 months to 24.8 years (mean 8.4 years). Eleven patients were treated according to protocols for Ewing tumors, while in 4 cases soft tissue protocols were used. In a single patient only surgery was done. Two patients had progressive disease despite chemotherapy; a third patient had only tumor response on the initial 2 chemotherapy courses. All 3 patients with initially metastatic disease died. One patient developed a second malignancy. Overall survival at 5 years was 75%. Event-free survival (EFS) at 5 years was 68%; for nonmetastatic patients 5-year EFS was 83%.CONCLUSION: The authors conclude that nonmetastasized extra-osseous Ewing tumors have a prognosis at least similar to that of osseous Ewing tumors.

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KW - Child, Preschool

KW - Dactinomycin/therapeutic use

KW - Female

KW - Follow-Up Studies

KW - Humans

KW - Ifosfamide/therapeutic use

KW - Kaplan-Meier Estimate

KW - Male

KW - Prognosis

KW - Protein Synthesis Inhibitors/therapeutic use

KW - Sarcoma, Ewing/drug therapy

KW - Treatment Outcome

KW - Vincristine/therapeutic use

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DO - 10.1080/08880010902855581

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VL - 26

SP - 175

EP - 185

JO - Pediatric hematology and oncology

JF - Pediatric hematology and oncology

IS - 4

ER -

Extra-osseous Ewing sarcoma

Abstract

Keywords

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