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

Abstract

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.

Incidence and Outcomes of Extremity Soft-Tissue Sarcomas in Children

“…By multivariate analysis, we found that local disease, NR-STS histology, and surgical resection were all independent prognostic indicators of survival for extremity tumors. These findings have been corroborated by other groups [4,5,7,8,16]. In addition, we found that muscle (not synovial sarcoma) and nerve tissue origin was an indicator of poor outcome, while adipose and fibrous origin predicted improved survival.…”

“…In our cohort of 1175 patients with extremity STS, we confirmed that most are NR-STS and present in patients older than 10 y of age [4,5,8]. In contrast, RMS were more prevalent in younger patients with alveolar type as the most common histologic subtype [8].…”

“…The cure rates of STS have improved significantly over the past 30 y, and about 70% of patients with localized disease can now be cured [4]. Outcome of patients with STS is directly influenced by such prognostic indicators as histology, tumor site, tumor size, local invasiveness, distant metastasis, and modalities of therapy [5][6][7].…”

New concepts for the treatment of pediatric nonrhabdomyosarcoma soft tissue sarcomas

“…Pediatric NRSTS have been considered as orphan diseases: despite the global incidence of NRSTS falling only a little short of the figure for RMS, the published studies available on pediatric NRSTS are far less numerous than those on RMS [12,13,[19][20][21][22][23][24][25][26], and most of the experience gained on the treatment of pediatric NRSTS has been based on principles derived from the management of RMS, which is a clearly distinct entity.…”

Novel agents in development for pediatric sarcomas

“…Because they represent a diversity of originating tissues, they have distinct biology, and therefore respond differently to therapy [1][2][3][4]. Some of the sarcomas commonly seen in children are also present in adults [5], which allow the pediatric therapies to exploit knowledge developed in adults with the same tumor types. As a result of their diverse biology, conventional therapeutics for pediatric sarcomas must be tailored to the particular tissue type [1,3,6].…”