Desmoid tumors are benign soft tissue tumors (known as aggressive fibromatosis) that arise from musculoaponevrotic structures.
It was first described in 1832 by McFarlane and it accounts for 0.03% of all neoplasms.
Desmoid tumors are rarely encountered in children,
but they are more aggressive at pediatric age.
These tumors often develop on postsurgical or posttraumatic scars and can also be related to pregnancy and oral contraceptive use.
It has been established the association with Gardner syndrome and familial adenomatous polyposis.
Desmoid tumors have a very low rate of malignant transformation,
a high rate of recurrence (19-77%) and are very invasive locally.
The tumor has firm consistency.
(Fig.
1 and 2)
Microscopically,
these tumors contain fascicles of spindle cells,
fibroblasts and dense collagen stroma.
The histopathological confirmation of diagnostic is necessary before treatment.
It is difficult to choose the best treatment method for desmoid tumors because of the low incidence and the lack of randomized and prospective trials.
The asymptomatic,
stable desmoid tumors can be observed (watchful waiting).
For symptomatic desmoids (those that produce mass effect on important structures) treatment is necessary.
Surgical treatment
If feasible,
the conventional treatment is surgical resection with wide margins.
The standard surgical goal is complete resection with negative microscopic margins.
If the resection is not complete,
the risk for local recurrence is higher,
although the reccurence can occur in complete resections,
too.
Nonsurgical treatment options
If surgical approach is impossible,
the patients have others options of treatment,
like radiation therapy or systemic treatment.
Radiotherapy is primary therapeutic option for the patients to which surgical excision is not possible.
Systemic treatment options:
§ Molecular targeted agents (imatinib –selective tyrosine kinase inhibitor);
§ Interferon;
§ Antiestrogen hormonal therapy (tamoxifen);
§ Cytotoxic agents (anthracyclines) are recommended for rapidly growing desmoids or for highly symptomatic patients;
§ Nonsteroidal anti-inflammatory drugs;