Innovative treatment for huge nuchal desmoid tumour: A case report with a 2-year follow-up

Summary 

Background

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 dire consequences such as disfigurement, functional impairment or even mortality. Surgical resection, radiotherapy, chemotherapy, hormonal therapy, non-steroidal anti-inflammatory drugs and even a wait-and-see policy, either alone or in combination, were advocated as treatment modalities.

We experienced an extremely difficult case who had a huge nuchal desmoid tumour measuring 45×35×20cm in dimension with extension to the anterior neck and thoracic paraspinal area. Its intimacy with the carotid artery, jugular vein and brachial plexus made margin-free resection infeasible. Moreover, the tumour burden was so immense that the patient was plunged into profound hypoproteinaemic, septic and anaemic status, with severe pain, bleeding and odour 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 exhibited no evidences of tumour relapse at 2 years' follow-up. She has resumed daily activity independently without noticeable functional deficit.

Conclusion

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

Keywords: Aggressive fibromatosis, Desmoid tumour, Embolisation, Nuchal tumour, Tourniquet