Abstract
BACKGROUND. Epithelioid sarcoma (ES) is an uncommon malignant soft tissue tumor. To the authors' knowledge, little information is available to date concerning its clinical features and management in children and adolescents, particularly with regard to the recently described proximal-type variant. The current study concerns 30 patients age <18 years who were enrolled in the Italian Soft Tissue Sarcoma Committee protocols. METHODS. Histopathologic specimens, clinical data, and treatment modalities were reviewed for the current analysis. Nineteen patients had classic ES and 11 had proximal-type ES. Surgery was the mainstay of treatment; radiotherapy was given to six patients considered to be at risk of local disease recurrence due to incomplete resection, and chemotherapy was administered to eight patients. RESULTS. With a median follow-up of 66 months, the 5-year event-free survival (EFS) and overall survival (OS) rates were 61.7% and 92.4%, respectively, but the OS rate dropped to 86.9% and 72.4%, respectively, at 10 years and 15 years. Local disease recurrence was the major cause of treatment failure. The most significant finding influencing both EFS and OS was tumor site, with a tumor locatipn in the extremities predicting a favorable outcome. Initially unfavorable clinical findings and a worse outcome were associated with the proximal-type variant of ES. A response to chemotherapy was noted in three of the seven patients with measurable disease. CONCLUSIONS. The current study confirms some typical features of ES (i.e., the peculiar superficial distal location [i.e., the hand, fingers], indolent growth, and a tendency to recur locally). The current study data do not clearly confirm the strong tendency for the lymph node involvement described in adult ES patients. Further studies are needed to better define the clinical behavior and biology of the proximal-type variant of ES.
| Original language | English |
|---|---|
| Pages (from-to) | 708-717 |
| Number of pages | 10 |
| Journal | Cancer |
| Volume | 106 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - Feb 1 2006 |
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Keywords
- Epithelioid sarcoma
- Lymph node metastases
- Nonrhabdomyosarcoma soft tissue sarcoma
- Pediatric soft tissue sarcomas
- Proximal-type epithelioid sarcoma
ASJC Scopus subject areas
- Cancer Research
- Oncology
Cite this
Epithelioid sarcoma in children and adolescents : A report from the Italian Soft Tissue Sarcoma Committee. / Casanova, Michela; Ferrari, Andrea; Collini, Paola; Bisogno, Gianni; Alaggio, Rita; Cecchetto, Giovanni; Gronchi, Alessandro; Meazza, Cristina; Garaventa, Alberto; Di Cataldo, Andrea; Carli, Modesto.
In: Cancer, Vol. 106, No. 3, 01.02.2006, p. 708-717.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Epithelioid sarcoma in children and adolescents
T2 - A report from the Italian Soft Tissue Sarcoma Committee
AU - Casanova, Michela
AU - Ferrari, Andrea
AU - Collini, Paola
AU - Bisogno, Gianni
AU - Alaggio, Rita
AU - Cecchetto, Giovanni
AU - Gronchi, Alessandro
AU - Meazza, Cristina
AU - Garaventa, Alberto
AU - Di Cataldo, Andrea
AU - Carli, Modesto
PY - 2006/2/1
Y1 - 2006/2/1
N2 - BACKGROUND. Epithelioid sarcoma (ES) is an uncommon malignant soft tissue tumor. To the authors' knowledge, little information is available to date concerning its clinical features and management in children and adolescents, particularly with regard to the recently described proximal-type variant. The current study concerns 30 patients age <18 years who were enrolled in the Italian Soft Tissue Sarcoma Committee protocols. METHODS. Histopathologic specimens, clinical data, and treatment modalities were reviewed for the current analysis. Nineteen patients had classic ES and 11 had proximal-type ES. Surgery was the mainstay of treatment; radiotherapy was given to six patients considered to be at risk of local disease recurrence due to incomplete resection, and chemotherapy was administered to eight patients. RESULTS. With a median follow-up of 66 months, the 5-year event-free survival (EFS) and overall survival (OS) rates were 61.7% and 92.4%, respectively, but the OS rate dropped to 86.9% and 72.4%, respectively, at 10 years and 15 years. Local disease recurrence was the major cause of treatment failure. The most significant finding influencing both EFS and OS was tumor site, with a tumor locatipn in the extremities predicting a favorable outcome. Initially unfavorable clinical findings and a worse outcome were associated with the proximal-type variant of ES. A response to chemotherapy was noted in three of the seven patients with measurable disease. CONCLUSIONS. The current study confirms some typical features of ES (i.e., the peculiar superficial distal location [i.e., the hand, fingers], indolent growth, and a tendency to recur locally). The current study data do not clearly confirm the strong tendency for the lymph node involvement described in adult ES patients. Further studies are needed to better define the clinical behavior and biology of the proximal-type variant of ES.
AB - BACKGROUND. Epithelioid sarcoma (ES) is an uncommon malignant soft tissue tumor. To the authors' knowledge, little information is available to date concerning its clinical features and management in children and adolescents, particularly with regard to the recently described proximal-type variant. The current study concerns 30 patients age <18 years who were enrolled in the Italian Soft Tissue Sarcoma Committee protocols. METHODS. Histopathologic specimens, clinical data, and treatment modalities were reviewed for the current analysis. Nineteen patients had classic ES and 11 had proximal-type ES. Surgery was the mainstay of treatment; radiotherapy was given to six patients considered to be at risk of local disease recurrence due to incomplete resection, and chemotherapy was administered to eight patients. RESULTS. With a median follow-up of 66 months, the 5-year event-free survival (EFS) and overall survival (OS) rates were 61.7% and 92.4%, respectively, but the OS rate dropped to 86.9% and 72.4%, respectively, at 10 years and 15 years. Local disease recurrence was the major cause of treatment failure. The most significant finding influencing both EFS and OS was tumor site, with a tumor locatipn in the extremities predicting a favorable outcome. Initially unfavorable clinical findings and a worse outcome were associated with the proximal-type variant of ES. A response to chemotherapy was noted in three of the seven patients with measurable disease. CONCLUSIONS. The current study confirms some typical features of ES (i.e., the peculiar superficial distal location [i.e., the hand, fingers], indolent growth, and a tendency to recur locally). The current study data do not clearly confirm the strong tendency for the lymph node involvement described in adult ES patients. Further studies are needed to better define the clinical behavior and biology of the proximal-type variant of ES.
KW - Epithelioid sarcoma
KW - Lymph node metastases
KW - Nonrhabdomyosarcoma soft tissue sarcoma
KW - Pediatric soft tissue sarcomas
KW - Proximal-type epithelioid sarcoma
UR - http://www.scopus.com/inward/record.url?scp=31544480125&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=31544480125&partnerID=8YFLogxK
U2 - 10.1002/cncr.21630
DO - 10.1002/cncr.21630
M3 - Article
C2 - 16353216
AN - SCOPUS:31544480125
VL - 106
SP - 708
EP - 717
JO - Cancer
JF - Cancer
SN - 0008-543X
IS - 3
ER -