Free jejunal transfer for a 15-year-old girl with synovial sarcoma of the hypopharynx.

INTRODUCTION

Synovial sarcomas are rare, aggressive malignant soft-tissue tumours. Approximately 85-90% of synovial sarcomas occur in the extremities, with only 3% arising in the head and neck region. The hypopharynx as a site of occurrence has only rarely been reported. We describe herein our experience of free jejunal transfer for an adolescent patient with synovial sarcoma of the hypopharynx.

CASE REPORT

A 15-year-old girl presented with a 2-month history of swallowing difficulties and hoarseness. Laryngoscopy revealed a mass filling the hypopharynx, almost occluding the larynx. Histological diagnosis obtained by biopsy was synovial sarcoma. The patient underwent total pharyngo-laryngo-oesophagectomy and reconstruction with a free jejunal graft. Postoperative chemotherapy and radiotherapy was performed. She has been free of recurrence or metastases for 2 years and 6 months. The patient can take a normal diet without dysphagia.

DISCUSSION

Synovial sarcomas are rare, malignant, soft-tissue tumours primarily affecting the extremities, with only 3% arising in the head and neck region. The cornerstone of treatment for synovial sarcomas is complete surgical resection. Recently, total pharyngo-laryngo-oesophagectomy and free jejunal transfer have become the standard technique for patients with malignancies confined to the hypopharynx because of the minimal morbidity and mortality, favourable functional outcome and low incidence of complications associated with the donor site.

CONCLUSION

We report the case of a 15-year-old girl with synovial sarcoma of the hypopharynx. We performed a total pharyngo-laryngo-oesophagectomy and reconstruction with a free jejunal graft. The postoperative course and the functional outcome were satisfactory.