Type:
Educational Exhibit
Keywords:
Musculoskeletal soft tissue, Oncology, MR, Imaging sequences, Neoplasia
Authors:
K. Motupally, K. Singh, P. Jeremic
DOI:
10.26044/ranzcr2022/R-0232
Background
Desmoid type fibromatosis or desmoid tumours are rare neoplasms classified by the World Health Organization as an intermediate lesion that is locally aggressive[1]. It is a non-metastasizing, monoclonal fibroblastic proliferative disease[2]. Desmoid tumours usually occur in the abdomen but are rarely seen in the axillae where their relationship to surrounding neurovascular structures including the brachial plexus can cause significant morbidity, posing a challenge to surgeons[3].
The cause of desmoid tumours remains unknown and most desmoid tumours occur sporadically. Desmoid tumours constitute 0.03% of all neoplasms and have an incidence of 2-4 per million population with a female:male ratio of 2:1. The peak age of occurrence is between 30-40 years. They may present sporadically or as a manifestation of familial adenomatous polyposis and its variants such as Gardner’s syndrome. [4,5].
With a multifactorial pathogenesis, they are commonly associated with pregnancy, oral contraceptive pills, previous surgical procedures or trauma. Physical examination findings are generally benign and desmoid tumours are usually painless; however, they can cause neurological symptoms such as numbness, pain, tingling and weakness along with decreased mobility when adjacent to the axilla [3].
Common differentials include fibrosarcoma and reactive fibrosis due to similarities in imaging findings and therefore require a biopsy to confirm the diagnosis[6].
Although considered rare, desmoid tumours require the input of multidisciplinary teams to preserve functional status as they show propensity for recurrence despite complete surgical excision. Function-sparing excision is considered the ideal treatment for extra abdominal desmoid tumours with radiotherapy being used as either the primary or adjuvant therapy. For advanced desmoid tumours not amenable to surgery or radiotherapy other treatments including cryotherapy, antihormonal therapies, targeted drug therapy, chemotherapy and NSAIDs have been used [7, 8].