Desmoid tumor are mesenchymal neoplasms which are invasive but do not metatstize. They are commonly referred to as musculoaponeurotic fibromatosis, deep fibromatosis, and aggressive fibromatosis.
Although desmoid tumors do not undergo metastasis, they are capable of causing severe morbidity and death.
A treatment plan which includes surgery, sometimes in conjunction with radiation therapy is generally suggested. Th
e local recurrence rate for desmoid tumors is high so the WHO has labeled them as, “intermediate locally aggressive tumors.”
The WHO describes a desmoid tumor as a “clonal fibroblastic proliferation that arises in the deep soft tissues and is characterized by infiltrative growth and a tendency toward local recurrence but an inability to metastasize,” Chemotherapy is a suggested option for DT cases which do not respond well to RT and surgery, or when both these options are not possible.
Epidemiology
Desmoid tumors are rare, with a meagre incidence of 2-4 individuals per million. They account only for 0.03% of all tumors.
Ages most affected by DT range between 30-40. Men between the ages of 15-60 are more affected than women.
Desmoid tumors develop on abdominal, intra-abdominal, and extra-abdominal sites.
Anatomy
Pathophysiology
Surgery, Radiation therapy, or a combination of both is suggested to patients with desmoid tumors.
In a study conducted in 1999, where 189 individuals were treated with surgery, resection and radiation therapy or only radiation, the overall relapse rates for patients were 30% and 33% for 5 and 10 years respectively.
Survival rates for 5, 10, and 15 years were 96%, 92%, and 87% respectively.
Tumors in the trunk have a better prognosis than tumors found in the extremities. The study concluded that for the majority of desmoid tumours, wide local excision with negative pathologic margins is the the best treatment.
Positive margins can be neutralised by adjuvant radiation therapy, therefore function-sparing resection is reasonable. Unresectable illness requires radiation therapy.
R
Etiology
Most cases of Desmoid tumors are idiopathic, and around 85% cases present a mutation in the CTNNB1 encoding beta-catenin pathway.
The three identified unique mutations are 41A, 45F, and 45. The mutation 45F is related with an elevated recurrence risk. The 5-year survival rate without recurrence was 23% for the 45F gene, 57% for the 41A gene, and 68% for no mutation.
In familial adenomatous polyposis (FAP), particularly abdominal FAP induced by a mutation in the APC gene, desmoid tumours are more prevalent. Patients
FAP-related DT shows a preference for the prior surgical location, and past surgery can increase risk of incidence. DT is a more significant source of morbidity and death in people treated with preventive colectomy than colon cancer.
DT occurs more frequently in women during or after pregnancy, and subjective data shows that abdominal wall damage and elevated oestrogen levels may be the cause. In general, outcomes are better for DT coupled with pregnancy.
Desmoid tumor are mesenchymal neoplasms which are invasive but do not metatstize. They are commonly referred to as musculoaponeurotic fibromatosis, deep fibromatosis, and aggressive fibromatosis.
Although desmoid tumors do not undergo metastasis, they are capable of causing severe morbidity and death.
A treatment plan which includes surgery, sometimes in conjunction with radiation therapy is generally suggested. Th
e local recurrence rate for desmoid tumors is high so the WHO has labeled them as, “intermediate locally aggressive tumors.”
The WHO describes a desmoid tumor as a “clonal fibroblastic proliferation that arises in the deep soft tissues and is characterized by infiltrative growth and a tendency toward local recurrence but an inability to metastasize,” Chemotherapy is a suggested option for DT cases which do not respond well to RT and surgery, or when both these options are not possible.
Desmoid tumors are rare, with a meagre incidence of 2-4 individuals per million. They account only for 0.03% of all tumors.
Ages most affected by DT range between 30-40. Men between the ages of 15-60 are more affected than women.
Desmoid tumors develop on abdominal, intra-abdominal, and extra-abdominal sites.
Surgery, Radiation therapy, or a combination of both is suggested to patients with desmoid tumors.
In a study conducted in 1999, where 189 individuals were treated with surgery, resection and radiation therapy or only radiation, the overall relapse rates for patients were 30% and 33% for 5 and 10 years respectively.
Survival rates for 5, 10, and 15 years were 96%, 92%, and 87% respectively.
Tumors in the trunk have a better prognosis than tumors found in the extremities. The study concluded that for the majority of desmoid tumours, wide local excision with negative pathologic margins is the the best treatment.
Positive margins can be neutralised by adjuvant radiation therapy, therefore function-sparing resection is reasonable. Unresectable illness requires radiation therapy.
R
Most cases of Desmoid tumors are idiopathic, and around 85% cases present a mutation in the CTNNB1 encoding beta-catenin pathway.
The three identified unique mutations are 41A, 45F, and 45. The mutation 45F is related with an elevated recurrence risk. The 5-year survival rate without recurrence was 23% for the 45F gene, 57% for the 41A gene, and 68% for no mutation.
In familial adenomatous polyposis (FAP), particularly abdominal FAP induced by a mutation in the APC gene, desmoid tumours are more prevalent. Patients
FAP-related DT shows a preference for the prior surgical location, and past surgery can increase risk of incidence. DT is a more significant source of morbidity and death in people treated with preventive colectomy than colon cancer.
DT occurs more frequently in women during or after pregnancy, and subjective data shows that abdominal wall damage and elevated oestrogen levels may be the cause. In general, outcomes are better for DT coupled with pregnancy.
Desmoid tumor are mesenchymal neoplasms which are invasive but do not metatstize. They are commonly referred to as musculoaponeurotic fibromatosis, deep fibromatosis, and aggressive fibromatosis.
Although desmoid tumors do not undergo metastasis, they are capable of causing severe morbidity and death.
A treatment plan which includes surgery, sometimes in conjunction with radiation therapy is generally suggested. Th
e local recurrence rate for desmoid tumors is high so the WHO has labeled them as, “intermediate locally aggressive tumors.”
The WHO describes a desmoid tumor as a “clonal fibroblastic proliferation that arises in the deep soft tissues and is characterized by infiltrative growth and a tendency toward local recurrence but an inability to metastasize,” Chemotherapy is a suggested option for DT cases which do not respond well to RT and surgery, or when both these options are not possible.
Desmoid tumors are rare, with a meagre incidence of 2-4 individuals per million. They account only for 0.03% of all tumors.
Ages most affected by DT range between 30-40. Men between the ages of 15-60 are more affected than women.
Desmoid tumors develop on abdominal, intra-abdominal, and extra-abdominal sites.
Surgery, Radiation therapy, or a combination of both is suggested to patients with desmoid tumors.
In a study conducted in 1999, where 189 individuals were treated with surgery, resection and radiation therapy or only radiation, the overall relapse rates for patients were 30% and 33% for 5 and 10 years respectively.
Survival rates for 5, 10, and 15 years were 96%, 92%, and 87% respectively.
Tumors in the trunk have a better prognosis than tumors found in the extremities. The study concluded that for the majority of desmoid tumours, wide local excision with negative pathologic margins is the the best treatment.
Positive margins can be neutralised by adjuvant radiation therapy, therefore function-sparing resection is reasonable. Unresectable illness requires radiation therapy.
R
Most cases of Desmoid tumors are idiopathic, and around 85% cases present a mutation in the CTNNB1 encoding beta-catenin pathway.
The three identified unique mutations are 41A, 45F, and 45. The mutation 45F is related with an elevated recurrence risk. The 5-year survival rate without recurrence was 23% for the 45F gene, 57% for the 41A gene, and 68% for no mutation.
In familial adenomatous polyposis (FAP), particularly abdominal FAP induced by a mutation in the APC gene, desmoid tumours are more prevalent. Patients
FAP-related DT shows a preference for the prior surgical location, and past surgery can increase risk of incidence. DT is a more significant source of morbidity and death in people treated with preventive colectomy than colon cancer.
DT occurs more frequently in women during or after pregnancy, and subjective data shows that abdominal wall damage and elevated oestrogen levels may be the cause. In general, outcomes are better for DT coupled with pregnancy.
Both our subscription plans include Free CME/CPD AMA PRA Category 1 credits.
Digital Certificate PDF
On course completion, you will receive a full-sized presentation quality digital certificate.
medtigo Simulation
A dynamic medical simulation platform designed to train healthcare professionals and students to effectively run code situations through an immersive hands-on experience in a live, interactive 3D environment.
medtigo Points
medtigo points is our unique point redemption system created to award users for interacting on our site. These points can be redeemed for special discounts on the medtigo marketplace as well as towards the membership cost itself.
Community Forum post/reply = 5 points
*Redemption of points can occur only through the medtigo marketplace, courses, or simulation system. Money will not be credited to your bank account. 10 points = $1.
All Your Certificates in One Place
When you have your licenses, certificates and CMEs in one place, it's easier to track your career growth. You can easily share these with hospitals as well, using your medtigo app.
