Factors Predicting Local Recurrence, Metastasis, and Survival in Pediatric Soft Tissue Sarcoma in Extremities

Abstract

Background Pediatric soft tissue sarcomas are rare and differ from those in adults regarding the spectrum of diagnoses and treatment. Sarcomas in extremities may have different prognoses from those located elsewhere. Questions/purposes We sought risk factors predicting local recurrence, metastasis, and overall survival and asked whether radiation and chemotherapy influenced local recurrence, metastasis, and overall survival. Methods We retrospectively reviewed all 98 patients aged 18 years or younger diagnosed with soft tissue sarcomas in extremities from 1990 to 2008. Age, tumor size, depth, location, bone or neurovascular involvement, histologic subtypes, unplanned excision, surgical margins, metastasis at diagnosis, and adjuvant treatments were reviewed for each patient. We determined the effect of each prognostic variable on local recurrence, metastasis, and overall survival. Results Ninety-four patients underwent surgical excision and seven patients had local recurrence at a median time of 18.6 months. Radiation therapy reduced the rate of local recurrence. Fourteen patients had metastasis at diagnosis and seven patients later developed metastasis. The median time to metastasis was 20.9 months. Six patients died and the median time to death was 28.0 months. Metastasis at diagnosis was a predictive factor for death. Conclusions When limited to extremities, radiation therapy reduced the rate of local recurrence in pediatric soft tissue sarcomas. Metastases at diagnosis predict death. Level of Evidence Level II, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.Each author certifies that he or she has no commercial associations (eg, consultancies, stock ownership, equity interest, patent/licensing arrangements, etc) that might pose a conflict of interest in connection with the submitted article. Each author certifies that his or her institution approved the human protocol for this investigation and that all investigations were conducted in conformity with ethical principles of research. This work was performed at

What Are Risk Factors for Local Recurrence of Deep High-grade Soft-tissue Sarcomas?

“…Patients with unplanned excisions had a tendency to have superficial tumors, and some reports show no adverse impact of unplanned excision on local recurrence [2,13]. However, our series exclusively analyzed sarcomas located deep in the fascia.…”

“…Whether patients undergoing unplanned excision of their primary sarcoma have a higher risk of local recurrence is controversial [2,4,10,13]. Several studies suggest unplanned excisions of soft-tissue sarcomas result in increased rates of local recurrence, mainly because of residual tumor [4,10].…”

“…Several studies suggest unplanned excisions of soft-tissue sarcomas result in increased rates of local recurrence, mainly because of residual tumor [4,10]. While patients with unplanned excisions tend to have superficial tumors, other reports show no adverse impact of unplanned excision on local recurrence [2,13]. Additionally, whether local recurrence, when it occurs, lessens the chance of local control of the tumor is also controversial [6,11,16].…”

“…The effect of local recurrence or unplanned excision on subsequent local recurrence in patients with deep, high-grade tumors may differ from recurrence of unplanned excision of subcutaneous or lowgrade sarcomas; however, there is no large series specifically addressing these issues. We presume (1) positive margins after surgical resection are associated with an increase in local recurrence [3,14], (2) presentation with local recurrence is a risk for subsequent local recurrence [6,11], and (3) unplanned excision may be a risk factor for local recurrence in deep, high-grade soft-tissue sarcomas [2,13].…”

Sarcomas de partes blandas en pacientes pediátricos. Análisis de una serie de casos del subtipo no rabdomiosarcoma

“…Se describió una supervivencia del 72% a los 5 años, similar a los resultados antes reportados. 9,10 La resección quirúrgica ha sido descrita como el pilar fundamental en el tratamiento de este grupo de tumores, orientada a obtener márgenes libres del tumor.…”