Remaining Problems and Controversies in the Management of Childhood Head and Neck Soft Tissue Sarcomas: Retrospective (National) Multicenter Study of the Polish Pediatric Solid Tumors Group

Abstract

Soft tissue sarcomas in children are a heterogeneous group of malignant diseases. Among these, tumors localized in the head and neck region are especially difficult to treat. While multidisciplinary care has dramatically improved the prognosis of sarcoma patients, their treatment remains uncertain because of demand on radical surgical resection of the tumor. Achieving cure without deforming or mutilating the child remains the primary goal of treatment. This study is the multicenter (nationwide, 11 Polish centers) retrospective analysis of the treatment results in children having soft tissue sarcoma in the head and neck region during the previous decade (from 1991 to 2001). Late effects of the treatment are documented in long-term survivals. Eighty-five children from 1 to 212 months of age were included. Different multimodal treatment protocols were utilized (CWS-91, SIOP-MMT-91, and CWS-96). The median observation time was 25 months. Data on long-term effects were collected in 34 long-term survivals. Complete remission was achieved in 68 (80%) patients. Primary treatment failure occurred in 13 (15.3%) patients, all of whom succumbed in disease progression. Relapse occurred in 21 (30.9%) patients primarily achieving complete remission. Second primary neoplasm occurred in 3 children. The estimated 5-year event-free survival and the 5-year total survival rates for the whole group are 0.38 and 0.55, respectively. The main late effect documented in long-term survivals were cosmetic defects in 12 (35.3%) and visual field deficit or blindness in 8 (26.5%). Despite substantial improvement of the prognosis of pediatric soft tissue sarcomas, the multimodal treatment of head and neck region tumors remains controversial. Improved long-term outcome and focusing on psychosocial difficulties raise the important and difficult problem of functional results and cosmesis. Tumors localized in the orbit carry an excellent prognosis. However, the main late sequela is vision impairment and cosmetic defect due to the therapy given many years earlier. Two other tumor localizations--the parameningeal and nonparameningeal ones--still have bad prognosis. The observations made in this study confirm that main prognostic factors are the size of the primary tumor and the tumor stage. The worst prognosis remains invasive tumor (T2-stage) with a size over 5 cm. Individually adjusted multimodal therapy, which imperatively needs to be radical, though not mutilating, might minimize the late effects. Psychosocial problems in long-term survivors need to be focused on at the national level and better support must be provided in the future, involving a team of different medical and paramedical profiles.

Management of pediatric head and neck rhabdomyosarcoma: A case-series of 36 patients

“…The essential aim of surgical treatment is the complete and radical resection of the primary RMS tumor with an acceptable functional and cosmetic result (27). Radical resection with histopathologically confirmed cancer-free surgical margins warrants a favorable prognosis (16,28). Surgical procedures play a major role in controlling cancer locally, particularly in patients who, due to their age (<2 years), are disqualified from irradiation (11,27).…”

“…Retrospective analysis was performed on a cohort of 36 pediatric patients diagnosed with H&N RMS who were treated at the Department of Otorhinolaryngology in the Faculty of Medicine and Dentistry of the Medical University of Warsaw (Warsaw, Poland) from January 2000 to December 2013. All patients underwent treatment with current therapy protocols for RMS, according to the guidelines of the Polish Pediatric Solid Tumors Group (15,16,22). Therapy was conducted in collaboration with pediatric oncologists who supervised the treatment regime.…”

“…This improvement may be attributed to the development of multimodal therapy combining surgery, radio-and chemotherapy (11). According to the Intergroup Rhabdomyosarcoma Study (IRS) IV, the 5-year RMS survival increased from 25% in 1970 to 73% in 2001 (15,16), but at the same time, almost 15% of children with RMS present with metastatic disease (group IV), and their prognosis has not improved significantly over the last 20 years (9). Radiation therapy is generally reserved for those patients with high-grade, unresectable tumors, and as adjuvant therapy following resection with cancer-positive surgical margins (4).…”

Review Rhabdomyosarcoma of the head and neck in children

“…Progress that has been accomplished in oncology in the last three decades significantly improved outcomes. With regard to soft tissues sarcomas the 5-year survival rate raised from 25% in 1970 to 73% in 2001, according to IRS-IV data [ 2 , 3 ]. This progress results from introducing complex treatment based on modern aggressive chemotherapy, advanced radiologic imaging and still improving surgical techniques (especially in skull base surgery, microsurgery and reconstruction surgery).…”

Correlations between DWI, IVIM, and HIF‐1α expression based on MRI and pathology in a murine model of rhabdomyosarcoma

“…Whereas the 5-year survival rate of patients with RMS is approximately 73%, the 5-year survival rate of RMS patients with distant metastases at diagnosis is only 20%-30%. [1][2][3][4][5] Several studies have demonstrated that the low oxygen environment inside tumors is a key factor that contributes to the poor prognosis of cancer. [6][7][8] This is due to hypoxia-inducible factor 1-alpha (HIF-1α) stimulating the production of vascular endothelial growth factor and tumor metastasis.…”