Alveolar soft part sarcoma in children and adolescents: The European Paediatric Soft Tissue Sarcoma study group prospective trial (EpSSG NRSTS 2005)

Abstract

This report demonstrates the ability to run prospective pediatric studies in NRSTS in multiple European countries, despite the small numbers of ASPS patients. We can conclude that for the majority with small resected tumors, there were few events and no deaths.

Outcome and prognostic factors in pediatric malignant peripheral nerve sheath tumors: An analysis of the European Pediatric Soft Tissue Sarcoma Group (EpSSG) NRSTS‐2005 prospective study

“…Carli et al showed an OS and PFS of 82% and 61% for group I tumors, and 62% and 37% for group II tumors . The excellent outcome for small resectable tumors was also observed in other NRSTS tumors of the prospective EpSSG‐NRSTS‐2005 study, that is, synovial sarcoma, infantile fibrosarcoma, and alveolar soft part sarcoma …”

Lingual Alveolar Soft Part Sarcoma in a 1-Year-Old Infant: Youngest Reported Case With Characteristic ASPSCR1-TFE3 Fusion

“…2 Median age at diagnosis in the studies was 11.5 and 17 years, with a range from 2.7 to 17.5 and 1.5 to 30 years, respectively. 1,2 Six of 22 cases (27%) were at head and neck sites, and 6 of 69 (9%) specifically at oral (pharynx/lingual) sites in the European and U.S. studies, respectively. 1,2 These data are consistent with lingual ASPS compromising only a small proportion of all ASPS cases.…”

“…1,2 Six of 22 cases (27%) were at head and neck sites, and 6 of 69 (9%) specifically at oral (pharynx/lingual) sites in the European and U.S. studies, respectively. 1,2 These data are consistent with lingual ASPS compromising only a small proportion of all ASPS cases. 3 Here, with full informed parental consent, we report the youngest described case of confirmed translocation-positive lingual ASPS.…”

Alveolar soft‐part sarcoma: Primary metastatic disease and metastatic relapse occurring during long‐term follow‐up

“…ASPS is considered to be chemo‐insensitive with a complete /partial remission rate of less than 10% following conventional chemotherapy . Surgery remains the mainstay of treatment, which has been recently confirmed for localized tumors . However, depending on tumor size, location, and spread, nonmutilating surgery may not be feasible in children.…”

“…2,5 Surgery remains the mainstay of treatment, which has been recently confirmed for localized tumors. 9,10 However, depending on tumor size, location, and spread, nonmutilating surgery may not be feasible in children. Therapy of metastatic disease (MD) in ASPS still remains unclear.…”