Clinical prognostic factors and treatment strategies for rhabdomyosarcoma in children

  OBJECTIVE: To perform a multifactorial analysis of 66 cases of rhabdomyosarcoma under the age of 18 years, to analyze and discuss independent factors affecting survival and to discuss and review the literature on treatment strategies for soft tissue sarcoma.  METHODS: Sixty-six cases, 30 males and 36 females, were followed up from January 1989 to December 2007; age at diagnosis: 9 months-18 years, median age 9 years, primary sites: 35 cases in the limbs and trunk, 20 cases in the head and neck, 7 cases in the genitourinary system, and 3 cases in the abdomen; histologic type: embryonal 56 cases, 8 cases in the glandular follicle, and 2 cases in the pleomorphic form; IRSG stages. Group I stage 12 cases, Group II stage 13 cases, Group III stage 35 cases, and Group IV stage 6 cases. Nine factors such as age, gender, histological type, tumor size, pre-treatment staging, surgical pathological staging, whether or not extensive resection, and chemotherapy regimen were introduced for statistical analysis.  RESULTS: Tumor size, whether or not extensive resection, surgical pathological staging, and choice of chemotherapy regimen were independent clinical factors affecting the survival rate of rhabdomyosarcoma. of 66 cases, 37 cases survived for more than 2 years, 27 cases had event-free survival, OS 56.06% (37/66) and EFS 40.91% (27/66); OS for tumors ≤5 cm and >5 cm were 77.78% (28/ 36) and 30.00% (9/30), EFS was 63.89% (23/36) and 13.33% (4/30); 2-year OS was 77.14% (27/35) and 32.26% (10/31), EFS was 68.57% (24/35) and 9.68% (3/31) for final extensive resection and non-extensive resection of tumor; stage I to IV OS was 100% (12/12), 78.57% (11/14), 35.29% (12/34), and 33.33% (2/6); EFS was 91.67% (11/12), 64.43% (9/14), 17.65% (6/34), and 16.67% (1/6), respectively; OS for irregular or no chemotherapy, VAC regimen, and platinum-based iso OS was 23.08% (6/26), 57.14% (4/7), and 81.82% (27/33), and EFS was 3.58% (1/26), 28.57% (2/7), and 72.73% (24/33) for irregular or no chemotherapy, VAC regimen, and platinum isophosphamide regimen, respectively. There was a statistical difference by test. Patients with tumors ≤5 cm, no residuals after surgery or the eventual ability to extensively resect the tumor with neoadjuvant therapy, and with appropriate strong chemotherapy regimens had higher survival rates.  Conclusion: The treatment of rhabdomyosarcoma in children and adolescents is comprehensive, and surgery should aim to achieve no tumor sarcoid and microscopic residue, but the preservation of postoperative function should be considered. For tumors that cannot be resected because they are large or the surgery may seriously affect the function or those with clear residue after the initial surgery can be extensively resected in the second surgery, or the second stage surgery can be performed after adopting appropriate neoadjuvant chemotherapy, and some children with stage III and IV disease can achieve extensive resection through effective Some children with stage III and IV disease can achieve extensive resection and long-term survival through effective neoadjuvant chemotherapy. Most pediatric rhabdomyosarcomas are very sensitive to chemotherapy, so chemotherapy is extremely important in the treatment of pediatric rhabdomyosarcoma, including neoadjuvant chemotherapy and postoperative adjuvant chemotherapy, and choosing the appropriate strong regimen of regular chemotherapy obviously helps to improve the surgical resection rate and long-term survival. In pediatric patients, routine radiotherapy is not advocated due to the development of distant complications such as delayed development and second carcinoma, and can be considered in certain cases where the primary site cannot be removed surgically and the primary site or metastases are poorly controlled locally after chemotherapy.