ABSTRACT
BACKGROUND: Primary malignant chest-wall tumors (PMCWTs) are a heterogeneous group of tumors. They require a special experience in designing resection and reconstruction. They account for less than 1% of all primary malignant tumors. This study is designed to clarify different factors contributing to the outcome of patients with PMCWTs in our institution. METHODS: A retrospective study included 98 patients with pathology proven PMCWTs, treated at the National Cancer Institute (NCI), Cairo University, Egypt, during the past 10 years. Used variables were: age, sex, forced expiratory volume in the 1st second (FEV1), site, size, multiplicity, pathologic subtype, tumor grade, safety margin (SM), excised ribs, complications, estimated blood loss (EBL), neo-adjuvant and adjuvant treatments, Overall and disease free survival (DFS) were obtained using Kaplan-Meier method and compared using Log rank test. Cox regression was used to identify DFS predictors. RESULTS: PMCWTs represented 10.5% of all thoracic malignancies in our institution. There were 51 females (52%). The median age was 39 years [interquartile range (IQR) =25-52.3)] years. Chondrosarcoma was the commonest tumor histology (20.4%). The median tumor size was 8 cm (IQR =5-14). Tumor multiplicity was found in 18.4% of patients. Bone resection was performed in 76 patients (78.3%), ribs resection was performed in 59 patients and the median number of resected ribs per patient was 3 (IQR =1-3) ribs. Sternal resection was done in 7 (7.1%) cases. R0 resection was achieved in 62.2% of patients. There was one operative related mortality (1.02%) and 17.3% patients suffered procedure related complications. Local recurrence developed in 35 (35.7%) patients. The overall survival (OS) at 1, 3 and 5 years was 73.9%, 45.6% and 34.6% respectively and the median OS was 33 months (95% CI, 21.8-44.2), while median DFS was 24 months (95% CI, 19.6-28.4). Predictors of better DFS were -ve SM (P<0.001), tumors <5 cm (P=0.039), low grade (P=0.033), lower EBL (P=0.003) and absence of adjuvant therapy (P=0.007); however, on multivariate analysis, only -ve SM was the only predictor (HR =0.54; 95% CI, 0.29-0.97, P=0.041). CONCLUSIONS: In primary malignant CWTs (PMCWTs) achievement of wide resection margins is of great importance to minimize the local tumor recurrence that will have an adverse impact on long-term survival.
ABSTRACT
BACKGROUND: Ewing's sarcoma accounts for 4-6% of primary malignant bone tumors and it affects the head and neck in only 1-4% of cases. The purpose of this study was to review the NCI experience with Ewing's sarcoma of the head and neck in children. PATIENTS AND METHODS: A retrospective analysis of patient files with head and neck Ewing's sarcoma treated at the National Cancer Institute, Cairo University, Egypt, during the period from 1997 to 2008 was done. Files were reviewed and data for patients, tumor and treatment profile were extracted. RESULTS: Twenty patients out of 280 with Ewing's sarcoma were identified during an 11-year period. Patients had a median age of 11.5 years (range 5 months - 22 years) with a male to female ratio of 1:1. The most common tumor site was in the mandible (9/20, 45%) followed by a neck mass (4/20, 20%) and a clavicular mass (3/20, 15%). Six patients (30%) were metastatic at presentation. Most of the patients (19/20, 95%) received chemotherapy. Local therapy was in the form of radical radiotherapy for 8 patients (40%), 2 patients (10%) had surgery alone, while five patients (25%) had surgical resection and postoperative radiotherapy. Overall survival ranged from 1 to 128 months, with a median of 36 months. At the end of the study, 9 patients (45%) were alive in CR, 6 (30%) were lost to FU in disease progression, while 5 patients died from disease progression. CONCLUSION: Ewing's sarcoma of the head and neck is a disease of a rare incidence with debate about the optimum local therapy. Small non-metastatic tumors with good response to chemotherapy have abetter outcome. KEY WORDS: Ewing's sarcoma - Head and neck - Management.
