Role of surgery in the treatment of radiation-induced sarcomas of the head and neck

Introduction: Radiation-induced sarcoma (RIS) is a well-known complication of radiotherapy. It is an aggressive tumour and the prognosis is often poor, despite radical treatment. We aim to investigate the role of surgery in the treatment of patients with RIS of the head and neck region. Method: We conducted a retrospective review of records of patients whom were treated for radiation-induced sarcoma at Queen Mary Hospital, Hong Kong, between the period 1999 to 2010. Data collected included patient age, gender, latency period for the development of RIS, site of RIS, symptoms, treatment given, pathology and survival. Results: Nineteen patients were included, (M=11, F=8). Eighteen received radiotherapy for treatment of primary nasopharyngeal carcinoma. One received radiotherapy for carcinoma of the parotid. The medial latency period for the development of sarcoma was 11.1 years (range 5.3-25.1). The most common site for development of RIS was the neck (n=8), followed by the oropharynx/oral cavity (n=4), nasopharynx (n=3) nasal cavity (n=2), maxilla (n=1) and mandible (n=1). The most common histology was undifferentiated sarcoma (n=6). Nine patients underwent surgical resection of the RIS with curative intent, three and six patients achieved R0 and R1 resection respectively. Chemotherapy was used in eleven instances after surgery. Radiotherapy was used in seven instances, three through brachytherapy, four through external beam irradiation. Overall medial survival from diagnosis of RIS was 1.74 years (95% CI 0.60-2.87), which improves to 2.47 years (95% CI 0.97-3.97) when only calculating those whom surgery was performed. Conclusions: Patients suffering from radiation-induced sarcomas have a poor prognosis, in keeping with existing literature. Treatment is mainly by surgery aiming at resection with a curative intent. If curative resection is impossible, surgery may still have a role in palliating the patients’ symptoms such as bleeding, fungation, or painpublished_or_final_versio

Prophylactic thyroidectomy in ethnic Chinese patients with multiple endocrine neoplasia type 2A syndrome after the introduction of genetic testing

Objective: To evaluate the impact of genetic testing in the management of familial multiple endocrine neoplasia 2A patients. Design: Retrospective study. Setting: University teaching hospital, Hong Kong. Patients: Twenty-two patients from eight multiple endocrine neoplasia 2A families underwent prophylactic total thyroidectomy based on a positive RET mutation genetic testing. All mutations were located at codon 634 of exon 11. Nineteen patients had preoperative basal serum calcitonin measured, and the 12 with normal levels had pentagastrin stimulation tests. Preoperative thyroid ultrasound examination was performed for 17 patients. Results: There were 13 females and 9 males with a median age of 25.1 (range, 6.1-71.9) years. Histopathology revealed medullary thyroid carcinoma in 17 (77%), C-cell hyperplasia in four (18%), and normal pathology in one (5%) of the patients. Five patients with either C-cell hyperplasia or normal pathology were among the youngest (age range, 6-9 years). The youngest patient with medullary thyroid carcinoma was nearly 9 years old. The median size of medullary thyroid carcinomas was 8.3 (range, 0.1-18) mm, but there were no lymph node metastases. Of 15 patients with normal basal calcitonin levels, 10 had medullary thyroid carcinoma, though two tested negative with the pentagastrinstimulated calcitonin assay. Five of six patients with normal preoperative ultrasonographic examinations had medullary thyroid carcinoma. Three (14%) of the patients were prescribed long-term calcium and vitamin D supplementation. After a median follow-up of 49 (range, 13-128) months, no patient had recurrence of medullary thyroid carcinoma. Conclusions: Genetic testing has replaced conventional biochemical and radiological modalities to identifying multiple endocrine neoplasia 2A carriers, in order to offer them prophylactic thyroidectomy. Chinese multiple endocrine neoplasia 2A patients with codon 634 mutation seem to have less aggressive forms of medullary thyroid carcinoma, for whom prophylactic thyroidectomy can be considered at the age of 8 years.published_or_final_versio

Radiation induced sarcomas of the head and neck: a ten-year experience

Conference Theme: Shifting Paradigms in Head and Neck Oncolog

Longitudinal changes in retinal nerve fibre layer thickness after an isolated unilateral retrobulbar optic neuritis: 1-Year results

The objective of this study was to investigate the longitudinal changes in retinal nerve fibre layer (RNFL) thickness 1 year after an episode of unilateral acute optic neuritis. This prospective cohort study recruited consecutive patients with a first episode of isolated, unilateral acute optic neuritis from October 2010 to June 2013. RNFL thickness of the attack and normal fellow eyes was measured by optical coherence tomography on presentation and 3, 6, and 12 months post attack in both the treatment and non-treatment groups. The treatment group consisted of subjects that opted for systemic steroids to hasten recovery time. In 20 subjects, 11 received systemic steroids and 9 were treated conservatively. The baseline RNFL thickness was similar in the attack and fellow eyes (p≥0.4). Progressive RNFL thinning was seen in the attack eye over the 12-month period, with significant differences for baseline versus 3 months; baseline versus 12 months; and 3 versus 12 months (all p<0.0001). At 12 months, the attack eye had a thinner average RNFL than the fellow eye (100.9±6.1 versus 107.3±5.5 μm; p=0.002). The 12-month RNFL was similar between the treatment and non-treatment groups (p≥0.6). A single episode of optic neuritis triggered an accelerated, progressive RNFL thinning up to 6 months post attack. Initial treatment with systemic steroids did not seem to alter the degree of RNFL loss at 12 months