Primary Cutaneous Ewing Sarcoma: A Systematic Review
Primary Cutaneous Ewing Sarcoma: A Systematic Review
Background Despite intensive treatment, the overall prognosis of Ewing sarcoma of the bone remains poor. Primary cutaneous Ewing sarcoma very rarely occurs and the prognosis has been reported to be better in some small series. All cases of Ewing sarcoma are currently treated in the same way, regardless of their location.
Objectives To determine whether Ewing sarcoma of the bone and primary cutaneous Ewing sarcoma are different in terms of epidemiology and prognosis.
Methods A systematic review of the literature was carried out using the keywords 'cutaneous Ewing's sarcoma', 'primary Ewing's sarcoma of skin' and 'primary neuroectodermal tumour and skin' in the Medline database. Series of five or more cases were included.
Results Six series met the inclusion criteria, making a total of 61 patients. Median age at diagnosis was 17 years and 33% were male. The median size of the tumour was 2·3 cm. The treatment consisted of surgery in all cases, adjuvant multiagent chemotherapy in 69% of cases, adjuvant chemoradiotherapy in 38% of cases and adjuvant radiotherapy without adjuvant chemotherapy in 3% of cases. Six patients developed metastases, four of whom died. The overall survival was 93% and the 10-year probability of survival was estimated at 91% (95% confidence interval 83–100).
Conclusion This systematic review demonstrated epidemiological and prognostic differences between Ewing sarcoma of the bone and primary cutaneous Ewing sarcoma. Primary cutaneous Ewing sarcoma has a female predominance, occurs at a later age, but, more importantly, has a better outcome. Multimodal therapy for Ewing sarcoma is associated with immediate and long-term morbidity and mortality. Although the size of our study does not allow a definitive conclusion about treatment modalities, we suggest that a less toxic approach compared with conventional treatment should be investigated in primary cutaneous Ewing sarcoma.
The Ewing sarcoma family of tumours conventionally includes three main entities: Ewing sarcoma, Askin tumour of the chest wall and peripheral neuroectodermal tumour (PNET). Histologically, Ewing sarcoma tumours comprise small round cells, mostly expressing CD99, and characterized by a specific chromosomal translocation involving the EWSR1 gene on chromosome 22, with an erythroblastosis virus-transforming gene (FLI1 in 85% of cases), resulting in a fusion oncogene.
Ewing sarcoma usually occurs in the bone. In this location, it is the second most common primary bone tumour in children and adolescents. In one American study, the overall incidence of Ewing sarcoma seemed to remain unchanged, with an average of 2·93 cases per 1 million reported annually between 1973 and 2004. Intensive therapeutic management has improved the prognosis of localized Ewing sarcoma of the bone, but not of disseminated disease, the overall prognosis of which remains poor. Overall 5-year survival varies depending on the period of the study, tumour volume and the metastatic stage. Overall 5-year survival has been 68% for localized tumours and 39% for metastatic disease in recent years.
A primary cutaneous location of Ewing sarcoma is very unusual. The literature reports only a few isolated cases or small series. The outcome seems better than for Ewing sarcoma of the bone. However, current recommendations for the treatment of Ewing sarcoma are the same, regardless of whether the location is osseous or extraosseous, i.e. extensive removal if possible, radiotherapy when surgical excision is impossible, and multiagent chemotherapy in all cases.
Our main objectives were to determine whether primary cutaneous Ewing sarcoma and Ewing sarcoma of the bone have different epidemiological characteristics, and whether they have a better prognosis and are treated differently. We therefore performed a systematic review of reported cases of primary cutaneous Ewing sarcoma published in the literature since 1997, and compiled the data.
Abstract and Introduction
Abstract
Background Despite intensive treatment, the overall prognosis of Ewing sarcoma of the bone remains poor. Primary cutaneous Ewing sarcoma very rarely occurs and the prognosis has been reported to be better in some small series. All cases of Ewing sarcoma are currently treated in the same way, regardless of their location.
Objectives To determine whether Ewing sarcoma of the bone and primary cutaneous Ewing sarcoma are different in terms of epidemiology and prognosis.
Methods A systematic review of the literature was carried out using the keywords 'cutaneous Ewing's sarcoma', 'primary Ewing's sarcoma of skin' and 'primary neuroectodermal tumour and skin' in the Medline database. Series of five or more cases were included.
Results Six series met the inclusion criteria, making a total of 61 patients. Median age at diagnosis was 17 years and 33% were male. The median size of the tumour was 2·3 cm. The treatment consisted of surgery in all cases, adjuvant multiagent chemotherapy in 69% of cases, adjuvant chemoradiotherapy in 38% of cases and adjuvant radiotherapy without adjuvant chemotherapy in 3% of cases. Six patients developed metastases, four of whom died. The overall survival was 93% and the 10-year probability of survival was estimated at 91% (95% confidence interval 83–100).
Conclusion This systematic review demonstrated epidemiological and prognostic differences between Ewing sarcoma of the bone and primary cutaneous Ewing sarcoma. Primary cutaneous Ewing sarcoma has a female predominance, occurs at a later age, but, more importantly, has a better outcome. Multimodal therapy for Ewing sarcoma is associated with immediate and long-term morbidity and mortality. Although the size of our study does not allow a definitive conclusion about treatment modalities, we suggest that a less toxic approach compared with conventional treatment should be investigated in primary cutaneous Ewing sarcoma.
Introduction
The Ewing sarcoma family of tumours conventionally includes three main entities: Ewing sarcoma, Askin tumour of the chest wall and peripheral neuroectodermal tumour (PNET). Histologically, Ewing sarcoma tumours comprise small round cells, mostly expressing CD99, and characterized by a specific chromosomal translocation involving the EWSR1 gene on chromosome 22, with an erythroblastosis virus-transforming gene (FLI1 in 85% of cases), resulting in a fusion oncogene.
Ewing sarcoma usually occurs in the bone. In this location, it is the second most common primary bone tumour in children and adolescents. In one American study, the overall incidence of Ewing sarcoma seemed to remain unchanged, with an average of 2·93 cases per 1 million reported annually between 1973 and 2004. Intensive therapeutic management has improved the prognosis of localized Ewing sarcoma of the bone, but not of disseminated disease, the overall prognosis of which remains poor. Overall 5-year survival varies depending on the period of the study, tumour volume and the metastatic stage. Overall 5-year survival has been 68% for localized tumours and 39% for metastatic disease in recent years.
A primary cutaneous location of Ewing sarcoma is very unusual. The literature reports only a few isolated cases or small series. The outcome seems better than for Ewing sarcoma of the bone. However, current recommendations for the treatment of Ewing sarcoma are the same, regardless of whether the location is osseous or extraosseous, i.e. extensive removal if possible, radiotherapy when surgical excision is impossible, and multiagent chemotherapy in all cases.
Our main objectives were to determine whether primary cutaneous Ewing sarcoma and Ewing sarcoma of the bone have different epidemiological characteristics, and whether they have a better prognosis and are treated differently. We therefore performed a systematic review of reported cases of primary cutaneous Ewing sarcoma published in the literature since 1997, and compiled the data.
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