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A patient with Down’s syndrome, with dermatofibrosarcoma protuberans, was intended for adjuvant radiotherapy. The lesion was on the parietal region of the head of the patient. Given the proximity of the lesion to the brain, the curvature of the lesion, and potential complications of anaesthesia for a Down’s syndrome patient, brachytherapy was the appropriate treatment. Anaesthesia complications for patients with Down’s syndrome are airway infections, atlanto-occipital dislocation and bradycardia.
Method:
Instead of sedating the patient in order to prepare a mould applicator, a 3D-printed model of the patient’s head was used. This allowed us greater time to prepare the applicator in a more relaxed environment.
Result:
The fit of the mould applicator on the patient was satisfactory. Minimum air gaps were observed. The treatment could be completed with sedation only.
Conclusion:
We were able to achieve an equivalent dose of 44·69 Gy in 5 sessions of brachytherapy, significantly reducing the anaesthesia sessions and the associated risks. A drawback of 3D printing is that it takes several hours to print the model.
Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive intermediate malignancy.
Objective
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.
Results
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.
Conclusion
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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