Desmoid Tumours: 3 cases involving the root of neck and literature review

Abstract

Desmoid tumours are a group of rare soft tissue tumours of mesenchymal origin, which arise following the monoclonal proliferation of well-differentiated fibroblasts. They are highly infiltrative and can be locally recurrent, but do not metastasize and may stabilise with time. This article describes 3 cases of desmoid tumours in a junctional area, and a literature review in the clinical management of this entity is also presented.

Three patients with junctional desmoid tumors were identified from the institutional soft tissue database comprising of 94 cases between 1994 and 2007. The case files were reviewed and the patient histories were summarized.

All 3 were female, and all presented with arm symptoms. Resection margins were either microscopically positive or narrowly cleared at the primary surgery of all 3 patients. Two patients suffered from recurrence, but all were able to achieve long-term survival of at least 12 years.

The management of desmoid tumours is evolving with increasing knowledge of the natural history of this disease. Watchful waiting is increasingly utilized for asymptomatic or stable cases. For symptomatic or progressive disease, treatment options include surgical resection, radiotherapy and systemic therapy. Both radiotherapy and systemic therapy have been used as primary treatment modalities, neoadjuvant therapy or adjuvant therapy with varying degrees of success. Due to the complexity of this disease, and the unpredictable natural history, it is recommended that all patients with desmoid tumors be managed in a multidisciplinary setting.