Adult-type soft tissue sarcomas in pediatric-age patients: experience at the Istituto Nazionale Tumori in Milan.
OBJECTIVE
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.


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%.


CONCLUSIONS
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.
Ferrari
Andrea
A
Pediatric Oncology Unit, Department of Pathology, Istituto Nazionale Tumori, Via G. Venezian, 1 20133 Milano, Italy. andrea.ferrari@istitutotumori.mi.it
Casanova
Michela
M
Collini
Paola
P
Meazza
Cristina
C
Luksch
Roberto
R
Massimino
Maura
M
Cefalo
Graziella
G
Terenziani
Monica
M
Spreafico
Filippo
F
Catania
Serena
S
Gandola
Lorenza
L
Gronchi
Alessandro
A
Mariani
Luigi
L
Fossati-Bellani
Franca
F
D000293:Adolescent; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012509:Sarcoma / Q000453:epidemiology / Q000473:pathology* / Q000628:therapy*; D012983:Soft Tissue Neoplasms / Q000453:epidemiology / Q000473:pathology* / Q000628:therapy*; D015996:Survival Rate; D016896:Treatment Outcome