Soft-tissue sarcomas of the extremities in patients of pediatric age

Abstract

Purpose The extremity site is a peculiar location for softtissue sarcomas (STS) of children and adolescents. Here we report the experience of the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, Italy, concerning 204 patients with STS of the limbs treated between 1977 and 2006. Methods The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma. Patients were treated with a multimodality approach including surgery, chemotherapy, and radiotherapy. Results For the RMS patients, the 5-year event-free survival (EFS) rate was 37.1%, with distant metastases being the main cause of treatment failure. The outcome was particularly poor for patients with large invasive tumors, hand/foot involvement and/or nodal or distant metastases, and for patients who were not given radiotherapy. For the extraosseous Ewing sarcoma cases, 5-year EFS was 74%. For the NRSTS cases, the 5-year EFS was 72.6%: tumor size and local invasiveness, tumor grade, malignant peripheral nerve sheath tumor (MPNST) histology, and distant metastases were the main prognostic factors. Discussion While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results. The treatment of these patients is complex and necessarily multidisciplinary, and it demands not only adequate experience of treating children and adolescents in clinical trials, but also particular skills in the field of orthopedic surgery.

Synovial sarcoma in patients under 20 years of age: A multicenter study with a minimum follow-up of 10 years

“…The mean age at diagnosis was 14.2 years (range: [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20].…”

“…Most present with a painful lump which gradually grows over time. In pediatric cohorts, survival rates of up to 75-84% [2,[4][5][6][7][8][9] have been reported. These are superior to those reported in adults (survival rates ranging from 25-71%) [4,[10][11][12][13][14].…”

“…Prognostic factors in SS remain a controversial topic [13]. Tumor stage [2], male sex, trunk-related tumors [5,8], large tumor size [2,4,5,7,9,17,18], and high tumor grade [7,14] have all been associated with an adverse outcome of SS.…”

Post Traumatic Dorsal Mass of a Child Hand: Rhabdomyosarcoma Must Be Suspected

“…Histologically, RMS has been assigned to three histological categories: embryonal, alveolar and pleomorphic with 15% to 20% of rhabdomyosarcomas not fitting into a conventional classification and remaining either undifferentiated or unclassifiable tumors [5]. Alveolar subtype found in our case is the most frequent RMS of the extremities and has the worst prognosis [11]. Radical surgical ablation by en-bloc excision or amputation affords the best chance of survival [2,12].…”

Treatment of Synovial Sarcoma in Children

“…Taking account of our experience and that of our foreign colleagues, we provided adjuvant RT to 84.2% of the experimental group; only 10.5% of the patients did not receive RT due to operative mutilation. Adjuvant PCT was essential in patients with poor prognosis [6]: age -10 years, lesion localization -trunk and extremities, primary tumor size above 5 cm (T2b), recurrent disease and regional/distant metastases upon diagnosis. Having analyzed the experience of 2 decades, European oncology pediatrics physicians came to the conclusion that PCT in children with SS is an essential asset of treatment (contrary to adult patients who do not respond to PCT [18] believe complex therapy to provide better outcome.…”