Learning objectives
To give an overview of the clinical background of desmoid type fibromatosis.
To highlight importance of MRI characteristics in pre and post-treatment imaging with respect to its clinical implication and clinicopathological correlate.
To provide a summary of the available options and novel treatment alternatives for desmoid type fibromatosis.
To briefly comment upon the complications associated with fibromatosis and its treatment.
Background
Desmoid type fibromatosis represents spectrum of benign soft tissue tumors with fibroblastic and myofibroblastic cell proliferation. These are characterized by tendency to arise from deep soft tissue, with high risk of recurrence owing to its infiltrative growth pattern. Commonly occurring in young females, these are associated with significant morbidity. Distant metastases are rare. Hence these can be characterized as locally aggressive malignancies1. While majority of them are sporadic, syndromic association, such as with hereditary familial adenomatous polyposis (Gardner’s syndrome) is well documented in literature2.
Patients...
Findings and procedure details
The role of imaging is to accurately tell us about the classical features which usually help in distinguishing a low grade fibrosarcoma vs traditional fibromatosis, describing nature of the tumour, defining its extent, margins, relations with surrounding neurovascular structures, extracompartmental extension and complications (figure 1) to help formulate a reasonable management plan for the patient. Additional information such as likely histopathological features and immediate post treatment changes may also be obtained by a radiologist with a keen eye.
MRI is the primary imaging modality to...
Conclusion
By understanding MRI imaging characteristics of desmoid-type fibromatosis, we can predict its pathological stage. A synoptic reporting format might help the treating physician create a thorough management plan for the patient by giving them crucial information about the anatomical characteristics of the tumour. Newer treatment modalities not only reduce the morbidities associated with surgery, but are also associated with fewer complications.
Personal information and conflict of interest
P. Rai:
Nothing to disclose
S. Suryavanshi:
Nothing to disclose
A. Janu:
Nothing to disclose
K. B. Gala:
Nothing to disclose
N. Shetty:
Nothing to disclose
S. Kulkarni:
Nothing to disclose
References
Fletcher CDM, World Health Organization, International Agency for Research on Cancer (2013) WHO classification of tumours of soft tissue and bone, 4th edn. IARC Press, Lyon
Nieuwenhuis MH, Casparie M, Mathus-Vliegen LM, Dekkers OM, Hogendoorn PC, Vasen HF. A nation-wide study comparing sporadic and familial adenomatous polyposis-related desmoid-type fibromatoses. Int J Cancer 2011;129(1):256–261.
Ganeshan D, Amini B, Nikolaidis P, Assing M, Vikram R. Current Update on Desmoid Fibromatosis. Journal of Computer Assisted Tomography. 2019 Jan-Feb;43(1):29-38. DOI: 10.1097/rct.0000000000000790. PMID: 30211798; PMCID: PMC6331223.
Murphey MD, Ruble CM,...