RESUMEN
BACKGROUND: Head and neck soft tissue sarcomas (HNSTS) constitute a rare and heterogeneous cancer entity. Management remains a challenge due to the rarity and varied biological behaviour among various subtypes. This systematic review examines the characteristics of tumours and patients with HNSTS. MATERIALS AND METHODS: A systematic literature review and meta-analysis were performed using the electronic databases PubMed and Embase. Eight eligible studies were identified, and 13 variables were extracted from each study including 5-year overall survival (OS) rate and 5-year disease-free survival (DFS) rate. RESULTS: We identified eight studies (n = 1,120 patients; 739 males (66%)) from six different countries). In total, 24 histological subtypes were found, and 20% of the sarcomas (n = 215) could not be subclassified. 607 sarcomas (57%) were <5 cm in diameter, and 945 sarcomas (84%) were grade 3. 1,059 patients (90%) underwent surgery. Estimated 5-year OS was 74% (95% CI; 0.63-0.84%) and 5-year DFS was 56% (95% CI; 38-74%). CONCLUSION: HNSTS holds a relative poor prognosis possibly explained by the heterogeneity of the disease. Treatment of HNSTS has shown to be highly diverse, underlining the importance of uniformed treatment guidelines in order to achieve improved survival outcomes.
RESUMEN
The aim of this study was to report incidence and patient characteristics of olfactory neuroblastoma (ONB) at a tertiary cancer institution during a 16-year period. A retrospective review was conducted on patients with ONB treated at Rigshospitalet, Copenhagen from 2000 to 2016 covering Eastern Denmark. Patient demographics, symptoms, stage, pathology-reports, treatment, and outcome were extracted from the patient records and the Danish pathology register. The tumours were graded both histologically and clinically using Hyam's and Kadish classifications, respectively. We identified a total of 14 patients (ten males, four females, median age 57 years, range 17-81 years). Four patients were in Kadish stage A, one stage B, and seven stage C. According to Hyam's classification, two tumours were grade I, nine grade II, and three grade III. All patients were treated with surgery, eight in combination with radiotherapy, where one received proton therapy, and one a combination with chemotherapy. At a median follow-up time of 58 months, the 5-year overall survival was 90% (95% CI 61; 99). ONB is a rare disease; complete radical surgery alone or combined with radiotherapy offered good oncologic control and outcome. Long-term follow-up of ONB should be mandatory.
