Innovative Treatment for Life-Threatening Nuchal Desmoid Tumour: A Case Report With a 5-Year Follow Up

The desmoid tumour is a monoclonal neoplasm originating from musculoaponeurotic tissues. It is benign in histological presentations and yet its locally invasive behaviour could lead to serious consequences such as disfigurement, functional impairment or even mortality.

We experienced an extremely difficult case who had a huge nuchal desmoid tumour measuring 45x35 x 20 cm in dimension with extension to the anterior neck and thoracic paraspinal area. Its wide based attachment and tenacious adherence to underlying musculoskeletal structures made margin free resection infeasible. Moreover, the tumour burden was so immense that the patient was entrenched into profound hypoproteinaemic, septic and anaemic status, with severe pain, bleeding and foul odoured discharges that mandated prompt and daring management.

Methods: In an effort to prevent uncontrollable tumour bleeding, we embarked on a series of strategic measures, including pre-surgical embolisation, innovative tourniquet technique, a novel method of ligature deployment, staged tumour excision and adjunct methods, such as ethanol injection and irradiation therapy.

Results: The huge nuchal desmoid tumour was successfully excised under the planned strategies. The patient went through a number of complications such as sepsis, acute respiratory distress and renal failure. Fortunately, she eventually survived and fared well at 5 years’ follow-up. She has resumed daily activity independently without noticeable functional deficit.

We thus suggested that multimodality strategies and innovative surgical techniques are the key to success in managing such a difficult case.