Aims and objectives
Desmoid tumours (DT) are rare mesenchymal tumours with an incidence of 5-6/1,000,000/year.
They usually affect young women between 15 and 60 years-old,
with a peak age of about 30-40 years-old.
Most cases are sporadic although approximately 5-10 % arises in the context of familial adenomatous polyposis (FAP) and Gardner’s syndrome,
suggesting a link with mutations of the APC gene on chromosome 5q22.
They may classified in three types according to their location: intraabdominal,
abdominal wall and extraabdominal.
These last ones are located deep in the...
Methods and materials
45 patients with biopsy-proven DT were diagnosed in a tertiary-level healthcare hospital (Vall d'Hebron,
Spain) from 2002 to 2017.
After excluding 3 patients with intraabdominal DT,
42 patients with extraabdominal and abdominal wall DT were included in our descriptive review.
MR was performed at diagnosis and during follow-up.
10 MR performed at diagnosis (23.8%) included functional sequences,
so semiquantitative analysis of tumour perfusion with SI/T curves (signal intensity/time) could be performed.
Patient age and sex,
treatment and outcome were recorded.
42 patients with biopsy-proven deep DT were included in the study.
4 patients were referred for recurrent disease.
They were predominantly women (75 %) with a mean age of 36 ± 18.8 years-old,
range from 12 to 86 years-old.
DT were categorized by location in extraabdominal (35) and abdominal wall (7) The extraabdominal group was subdivided in those located at the shoulder girdle (5),
the pelvic girdle (3),
the trunk and thoracic wall (18) and extremities (8),
including one in the stump of a subcondylar...
− MR is important in management of DT: diagnosis,
response to treatment and detection of recurrences.
− Signal intensity on T2WI correlates with distribution of fibroblast and collagen in anatomopathological samples.
− Diffusion sequences helps in differentiating DT from other soft-tissue tumours,
− DCE sequences are useful in better characterization of DT and selection those with higher risk of progression.
− Management of DT has evolved towards a more conservative approach (“watch and wait”),
so importance of MR increases.
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