Ewing's sarcoma and primitive neuroectodermal tumour in adults: single-centre experience in The Netherlands

C H Smorenburg, C J van Groeningen, O W M Meijer, M Visser, E Boven

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

BACKGROUND: Ewing's sarcoma and peripheral primitive neuroectodermal tumours (PNET) are rare tumours and closely related. They occur most often in children and adolescents. Few studies have been published on treatment outcome in adult patients.

METHODS: We performed a retrospective analysis of patients aged >16 years who were primarily treated at our university hospital for Ewing's sarcoma or PNET. In general, treatment consisted of long-term multiagent chemotherapy, interrupted by individualised local treatment consisting of surgery and/or radiotherapy. We reviewed clinical features and outcomes to present our experience with Ewing's sarcoma and PNET in adults.

RESULTS: From 1979 to 2002, 27 patients with Ewing's sarcoma (20) or PNET (7) were treated. There were 22 men and 5 women, with a median age of 25 years (range 17-49). Ten patients presented with metastases predominantly in lungs (4) or bones (6). Combination therapy consisted of chemotherapy (27), surgery (16) and radiotherapy (16). After a median follow-up of ten years, 14 patients have died (toxicity = 2, progressive disease = 12) and 13 patients are alive and free of disease. Five-year overall survival was 58%. All four patients with bone metastases died, while all five patients presenting with lung metastases are disease-free.

CONCLUSION: The five-year overall survival of 58% in this small series on adult patients is in line with paediatric study outcomes. Patients with lung metastases may even be cured by multimodality therapy. We therefore strongly advocate referral of patients with this rare disease to a specialised oncology centre.

Original languageEnglish
Pages (from-to)132-6
Number of pages5
JournalNetherlands Journal of Medicine
Volume65
Issue number4
Publication statusPublished - Apr 2007

Cite this

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title = "Ewing's sarcoma and primitive neuroectodermal tumour in adults: single-centre experience in The Netherlands",
abstract = "BACKGROUND: Ewing's sarcoma and peripheral primitive neuroectodermal tumours (PNET) are rare tumours and closely related. They occur most often in children and adolescents. Few studies have been published on treatment outcome in adult patients.METHODS: We performed a retrospective analysis of patients aged >16 years who were primarily treated at our university hospital for Ewing's sarcoma or PNET. In general, treatment consisted of long-term multiagent chemotherapy, interrupted by individualised local treatment consisting of surgery and/or radiotherapy. We reviewed clinical features and outcomes to present our experience with Ewing's sarcoma and PNET in adults.RESULTS: From 1979 to 2002, 27 patients with Ewing's sarcoma (20) or PNET (7) were treated. There were 22 men and 5 women, with a median age of 25 years (range 17-49). Ten patients presented with metastases predominantly in lungs (4) or bones (6). Combination therapy consisted of chemotherapy (27), surgery (16) and radiotherapy (16). After a median follow-up of ten years, 14 patients have died (toxicity = 2, progressive disease = 12) and 13 patients are alive and free of disease. Five-year overall survival was 58{\%}. All four patients with bone metastases died, while all five patients presenting with lung metastases are disease-free.CONCLUSION: The five-year overall survival of 58{\%} in this small series on adult patients is in line with paediatric study outcomes. Patients with lung metastases may even be cured by multimodality therapy. We therefore strongly advocate referral of patients with this rare disease to a specialised oncology centre.",
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author = "Smorenburg, {C H} and {van Groeningen}, {C J} and Meijer, {O W M} and M Visser and E Boven",
year = "2007",
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Ewing's sarcoma and primitive neuroectodermal tumour in adults : single-centre experience in The Netherlands. / Smorenburg, C H; van Groeningen, C J; Meijer, O W M; Visser, M; Boven, E.

In: Netherlands Journal of Medicine, Vol. 65, No. 4, 04.2007, p. 132-6.

Research output: Contribution to journalArticleAcademicpeer-review

TY - JOUR

T1 - Ewing's sarcoma and primitive neuroectodermal tumour in adults

T2 - single-centre experience in The Netherlands

AU - Smorenburg, C H

AU - van Groeningen, C J

AU - Meijer, O W M

AU - Visser, M

AU - Boven, E

PY - 2007/4

Y1 - 2007/4

N2 - BACKGROUND: Ewing's sarcoma and peripheral primitive neuroectodermal tumours (PNET) are rare tumours and closely related. They occur most often in children and adolescents. Few studies have been published on treatment outcome in adult patients.METHODS: We performed a retrospective analysis of patients aged >16 years who were primarily treated at our university hospital for Ewing's sarcoma or PNET. In general, treatment consisted of long-term multiagent chemotherapy, interrupted by individualised local treatment consisting of surgery and/or radiotherapy. We reviewed clinical features and outcomes to present our experience with Ewing's sarcoma and PNET in adults.RESULTS: From 1979 to 2002, 27 patients with Ewing's sarcoma (20) or PNET (7) were treated. There were 22 men and 5 women, with a median age of 25 years (range 17-49). Ten patients presented with metastases predominantly in lungs (4) or bones (6). Combination therapy consisted of chemotherapy (27), surgery (16) and radiotherapy (16). After a median follow-up of ten years, 14 patients have died (toxicity = 2, progressive disease = 12) and 13 patients are alive and free of disease. Five-year overall survival was 58%. All four patients with bone metastases died, while all five patients presenting with lung metastases are disease-free.CONCLUSION: The five-year overall survival of 58% in this small series on adult patients is in line with paediatric study outcomes. Patients with lung metastases may even be cured by multimodality therapy. We therefore strongly advocate referral of patients with this rare disease to a specialised oncology centre.

AB - BACKGROUND: Ewing's sarcoma and peripheral primitive neuroectodermal tumours (PNET) are rare tumours and closely related. They occur most often in children and adolescents. Few studies have been published on treatment outcome in adult patients.METHODS: We performed a retrospective analysis of patients aged >16 years who were primarily treated at our university hospital for Ewing's sarcoma or PNET. In general, treatment consisted of long-term multiagent chemotherapy, interrupted by individualised local treatment consisting of surgery and/or radiotherapy. We reviewed clinical features and outcomes to present our experience with Ewing's sarcoma and PNET in adults.RESULTS: From 1979 to 2002, 27 patients with Ewing's sarcoma (20) or PNET (7) were treated. There were 22 men and 5 women, with a median age of 25 years (range 17-49). Ten patients presented with metastases predominantly in lungs (4) or bones (6). Combination therapy consisted of chemotherapy (27), surgery (16) and radiotherapy (16). After a median follow-up of ten years, 14 patients have died (toxicity = 2, progressive disease = 12) and 13 patients are alive and free of disease. Five-year overall survival was 58%. All four patients with bone metastases died, while all five patients presenting with lung metastases are disease-free.CONCLUSION: The five-year overall survival of 58% in this small series on adult patients is in line with paediatric study outcomes. Patients with lung metastases may even be cured by multimodality therapy. We therefore strongly advocate referral of patients with this rare disease to a specialised oncology centre.

KW - Adolescent

KW - Adult

KW - Antineoplastic Combined Chemotherapy Protocols

KW - Bone Neoplasms/drug therapy

KW - Combined Modality Therapy

KW - Cyclophosphamide/administration & dosage

KW - Dactinomycin/administration & dosage

KW - Doxorubicin/administration & dosage

KW - Etoposide/administration & dosage

KW - Female

KW - Follow-Up Studies

KW - Humans

KW - Ifosfamide/administration & dosage

KW - Kaplan-Meier Estimate

KW - Lung Neoplasms/radiotherapy

KW - Male

KW - Middle Aged

KW - Netherlands

KW - Neuroectodermal Tumors, Primitive, Peripheral/drug therapy

KW - Retrospective Studies

KW - Sarcoma, Ewing/drug therapy

KW - Survival Analysis

KW - Treatment Outcome

KW - Vincristine/administration & dosage

M3 - Article

VL - 65

SP - 132

EP - 136

JO - Netherlands Journal of Medicine

JF - Netherlands Journal of Medicine

SN - 0300-2977

IS - 4

ER -