Abstract
Purpose: Nonrhabdomyosarcoma soft tissue sarcomas are a heterogeneous group of tumors for which optimal treatment remains controversial. We report on a large group of 182 patients younger than 18 years old treated at a single institution over a 25-year period. Patients and Methods: In this relatively homogeneous subgroup of adult-type histotypes, surgery was the mainstay of treatment; radiotherapy was administered to 73 patients, and chemotherapy was administered to 114 patients (70 received chemotherapy as adjuvant therapy). Results: Overall survival at 5 years was 89% in patients who underwent complete resection at diagnosis, 79% in patients who had marginal resection, 52% in initially unresected patients, and 17% in patients with metastases at onset. Outcome was unsatisfactory in patients with large and high-grade tumors, even after gross resection; adjuvant chemotherapy seemed to improve the results in this group. Initially unresected patients who responded well to chemotherapy and subsequently underwent complete resection had an event-free survival rate of approximately 70%. The rate of response to chemotherapy was 58%. Conclusion: The identification of prognostic variables should enable risk-adapted therapies to be planned. Patients with initially unresectable disease and patients with resected large and high-grade tumors are at high risk of metastases and treatment failure. Although the limits of this retrospective analysis are self-evident, our data would suggest that intensive chemotherapy (with an ifosfamide-doxorubicin regimen) might have a more significant role in these patients than what is generally assumed.
| Original language | English |
|---|---|
| Pages (from-to) | 4021-4030 |
| Number of pages | 10 |
| Journal | Journal of Clinical Oncology |
| Volume | 23 |
| Issue number | 18 |
| DOIs | |
| Publication status | Published - 2005 |
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ASJC Scopus subject areas
- Cancer Research
- Oncology
Cite this
Adult-type soft tissue sarcomas in pediatric-age patients : Experience at the Istituto Nazionale Tumori in Milan. / Ferrari, Andrea; Casanova, Michela; Collini, Paola; Meazza, Cristina; Luksch, Roberto; Massimino, Maura; Cefalo, Graziella; Terenziani, Monica; Spreafico, Filippo; Catania, Serena; Gandola, Lorenza; Gronchi, Alessandro; Mariani, Luigi; Fossati-Bellani, Franca.
In: Journal of Clinical Oncology, Vol. 23, No. 18, 2005, p. 4021-4030.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Adult-type soft tissue sarcomas in pediatric-age patients
T2 - Experience at the Istituto Nazionale Tumori in Milan
AU - Ferrari, Andrea
AU - Casanova, Michela
AU - Collini, Paola
AU - Meazza, Cristina
AU - Luksch, Roberto
AU - Massimino, Maura
AU - Cefalo, Graziella
AU - Terenziani, Monica
AU - Spreafico, Filippo
AU - Catania, Serena
AU - Gandola, Lorenza
AU - Gronchi, Alessandro
AU - Mariani, Luigi
AU - Fossati-Bellani, Franca
PY - 2005
Y1 - 2005
N2 - Purpose: Nonrhabdomyosarcoma soft tissue sarcomas are a heterogeneous group of tumors for which optimal treatment remains controversial. We report on a large group of 182 patients younger than 18 years old treated at a single institution over a 25-year period. Patients and Methods: In this relatively homogeneous subgroup of adult-type histotypes, surgery was the mainstay of treatment; radiotherapy was administered to 73 patients, and chemotherapy was administered to 114 patients (70 received chemotherapy as adjuvant therapy). Results: Overall survival at 5 years was 89% in patients who underwent complete resection at diagnosis, 79% in patients who had marginal resection, 52% in initially unresected patients, and 17% in patients with metastases at onset. Outcome was unsatisfactory in patients with large and high-grade tumors, even after gross resection; adjuvant chemotherapy seemed to improve the results in this group. Initially unresected patients who responded well to chemotherapy and subsequently underwent complete resection had an event-free survival rate of approximately 70%. The rate of response to chemotherapy was 58%. Conclusion: The identification of prognostic variables should enable risk-adapted therapies to be planned. Patients with initially unresectable disease and patients with resected large and high-grade tumors are at high risk of metastases and treatment failure. Although the limits of this retrospective analysis are self-evident, our data would suggest that intensive chemotherapy (with an ifosfamide-doxorubicin regimen) might have a more significant role in these patients than what is generally assumed.
AB - Purpose: Nonrhabdomyosarcoma soft tissue sarcomas are a heterogeneous group of tumors for which optimal treatment remains controversial. We report on a large group of 182 patients younger than 18 years old treated at a single institution over a 25-year period. Patients and Methods: In this relatively homogeneous subgroup of adult-type histotypes, surgery was the mainstay of treatment; radiotherapy was administered to 73 patients, and chemotherapy was administered to 114 patients (70 received chemotherapy as adjuvant therapy). Results: Overall survival at 5 years was 89% in patients who underwent complete resection at diagnosis, 79% in patients who had marginal resection, 52% in initially unresected patients, and 17% in patients with metastases at onset. Outcome was unsatisfactory in patients with large and high-grade tumors, even after gross resection; adjuvant chemotherapy seemed to improve the results in this group. Initially unresected patients who responded well to chemotherapy and subsequently underwent complete resection had an event-free survival rate of approximately 70%. The rate of response to chemotherapy was 58%. Conclusion: The identification of prognostic variables should enable risk-adapted therapies to be planned. Patients with initially unresectable disease and patients with resected large and high-grade tumors are at high risk of metastases and treatment failure. Although the limits of this retrospective analysis are self-evident, our data would suggest that intensive chemotherapy (with an ifosfamide-doxorubicin regimen) might have a more significant role in these patients than what is generally assumed.
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U2 - 10.1200/JCO.2005.02.053
DO - 10.1200/JCO.2005.02.053
M3 - Article
VL - 23
SP - 4021
EP - 4030
JO - Journal of Clinical Oncology
JF - Journal of Clinical Oncology
SN - 0732-183X
IS - 18
ER -