Adult-type soft tissue sarcomas in pediatric-age patients

Experience at the Istituto Nazionale Tumori in Milan

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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 languageEnglish
Pages (from-to)4021-4030
Number of pages10
JournalJournal of Clinical Oncology
Volume23
Issue number18
DOIs
Publication statusPublished - 2005

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Sarcoma
Pediatrics
Drug Therapy
Adjuvant Chemotherapy
Neoplasm Metastasis
Neoplasms
Ifosfamide
Therapeutics
Treatment Failure
Doxorubicin
Disease-Free Survival
Radiotherapy
Survival Rate

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

@article{a16ff791f9794359b701465be001aa63,
title = "Adult-type soft tissue sarcomas in pediatric-age patients: Experience at the Istituto Nazionale Tumori in Milan",
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.",
author = "Andrea Ferrari and Michela Casanova and Paola Collini and Cristina Meazza and Roberto Luksch and Maura Massimino and Graziella Cefalo and Monica Terenziani and Filippo Spreafico and Serena Catania and Lorenza Gandola and Alessandro Gronchi and Luigi Mariani and Franca Fossati-Bellani",
year = "2005",
doi = "10.1200/JCO.2005.02.053",
language = "English",
volume = "23",
pages = "4021--4030",
journal = "Journal of Clinical Oncology",
issn = "0732-183X",
publisher = "American Society of Clinical Oncology",
number = "18",

}

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 -