Desmoid Tumors
Background
What are desmoid tumors (DT)?
DTs are deep-seated, benign, slow-growing −fibroblastic neoplasms that arise from musculoaponeurotic stromal elements.
What is another commonly used name for DT?
DT is also known as aggressive fibromatosis.
Do DTs have metastatic potential?
No. DTs do not have metastatic potential but are locally aggressive with a predilection for LR.
Approximately how many cases of DT are diagnosed annually in the U.S.?
~9,000 cases/yr of DT in the U.S.
Is there a gender or racial/ethnic predilection for DT?
Yes, with regard to gender. Women are more −commonly affected than men (2:1). There is no −significant racial or ethnic predilection in DT.
What is the avg decade of life in which pts present with DT?
On avg, DT pts present in the 3rd–4th decade of life.
What genetic abnormality is associated with DT?
2% of DTs are associated with mutations to the APC gene, resulting in familial adenomatous polyposis (FAP).
What is the clinical syndrome associated with DT?
Gardner syndrome is associated with DT, and 4%–20% of pts with this syndrome will develop DT.
Sebaceous cysts
Osteomas
Desmoid tumors
(Mnemonic: gardener SOD)
What % of DTs are intra-abdominal, and with what clinical syndrome are intra-abdominal DTs associated?
10%–30% of DTs are intra-abdominal, and they are associated with Gardner syndrome.
DTs are a significant source of morbidity and mortality in what group of pts?
Pts with Gardner syndrome experience significant morbidity and mortality from their intra-abdominal tumors.
What 2 environmental conditions are associated with DT?
Retrospective data suggests an association between DT and (1) antecedent trauma and (2) high estrogen states (such as pregnancy).
DT appears histologically similar to what tumor?
DT appears histologically similar to well-differentiated (grade 1) fibrosarcoma.
Name 3 general anatomic sites in which DT develops.
DT develops in the (1) trunk/extremity, (2) abdominal wall, and (3) intra-abdominal compartment.
What is the typical presentation of an extremity DT?
Most DTs of the extremity present as a deep-seated painless mass with a Hx of slow growth.
What is the typical presentation of an intra-abdominal DT?
An intra-abdominal DT can present with bowel −ischemia or obstruction.
What is the natural Hx of untreated DTs?
Although DTs can regress spontaneously, they usually continue with slow growth and local Sx associated with tumor invasion into surrounding structures.
Related posts:
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
