Originally published as JCO Early Release 10.1200/JCO.2005.02.053 on March 14 2005

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Adult-Type Soft Tissue Sarcomas in Pediatric-Age Patients: Experience at the Istituto Nazionale Tumori in Milan

From the Pediatric Oncology Unit, Departments of Pathology and Radiotherapy, Melanoma Sarcoma Surgical Unit, and Division of Medical Statistics and Biometry, Istituto Nazionale per lo Studio e la Cura dei Tumori, Milano, Italy

Address reprint requests to Andrea Ferrari, MD, Pediatric Oncology Unit, Istituto Nazionale Tumori, Via G. Venezian, 1 20133 Milano, Italy; e-mail: andrea.ferrari{at}istitutotumori.mi.it

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.

Supported in part by the Associazione Bianca Garavaglia.

Authors' disclosures of potential conflicts of interest are found at the end of this article.

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