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To analyse the presentation, diagnosis and patterns of care of extraosseous Ewing sarcoma treated at our institution between 2008 and 2018.
Electronic medical records of extraosseous Ewing sarcoma patients treated at our institution between January 2008 and April 2018 were reviewed. Kaplan–Meier curves were plotted to assess the overall and disease-free survival with 95% confidence intervals. A univariate analysis was carried out to assess the impact of variables such as surgical excision, completeness of surgery, completeness of chemotherapy and addition of radiation therapy on the survivorship.
The records of 65 patients treated at our institution were available for review. The mean age was 26·4 years. The most frequent sites of extraosseous Ewing tumour were kidney—9/65 (13·8%) and brain—10/65 (15·4%). Sixteen (24·6%) patients presented with inoperable/metastatic disease at diagnosis. The other 49 (75·4%) had localised disease at presentation. The median overall survival of the 49 non-metastatic patients was 46 months, and the disease-free survival was 45 months.
Extraosseous Ewing sarcoma is a rare and aggressive tumour diagnosed by molecular techniques. Multi-modality treatment including surgical resection with wide margins, adjuvant radiation when indicated and completion of systemic chemotherapy results in optimum outcomes.
Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive intermediate malignancy.
The purpose of this retrospective analysis is to determine the efficacy of radiation therapy (RT) in local control of DFSP.
Patients and methods
The recurrence-free survival (RFS) for 45 patients treated for DFSP at our institution was estimated and compared between surgery alone and postoperative RT groups.
Age range of the patients were in the third and fourth decades; males:females=2:1; most common site: anterior abdominal wall; tumours >5 cm in size in 75%; low grade in 77·8%; margins positive in 31·8% and <5 mm margins in 45·5%. Two-thirds of patients had at least one recurrence before presentation to our institution. RT dose was >50 Gy in 88% of patients. The patients treated with postoperative RT had poorer prognostic factors compared with surgery alone: they were males (17 versus 13 patients), and presented with high-grade tumours (5 versus 1 patients), multiple recurrences prior to presentation (25 versus 20 patients) and positive or <5 mm margins (22 versus 12 patients). Median follow-up for surgery alone group was 17 (1–152) months and for postoperative RT group, this was 54 (5–121) months. RFS at 5 years was 77·1% for surgery alone and 87·9% for postoperative RT group but was not statistically significant. The median time to recurrence was 4 years.
RT delays the time to recurrence in DFSP. RT improves the outcome of DFSP for recurrent tumours and with positive margins.
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