Abstract
Desmoid tumor represents a rare monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Although histologically benign, desmoids are locally invasive and associated with a high local recurrence rate, but lack of metastatic potential. Many issues regarding the optimal treatment of patients with desmoids remain controversial. Surgical resection and radiotherapy are standard treatment options for these patients. Due to heterogeneity of the biological behavior of desmoids, including long periods of stable disease or even spontaneous regression, treatment needs to be individualized to optimize local tumor control and preserve patient's quality of life. Therapeutic approaches to the treatment of recurrent or unresectable desmoid tumors comprise anti-hormonal therapy, non-steroidal anti-inflammatory drugs, classic chemotherapy regimens and tyrosine kinase inhibitor, with highly variable results. It has not yet been possible to establish an optimal therapy protocol for this disease. In this case report we present our experience with the treatment of recurrent extra-abdominal desmoid tumor.
Highlights
IntroductionDesmoid tumors (DT), known as aggressive and to emphasize the importance of multidisciplinary fibromatosis or musculoaponeurotic fibromatosis, are approach in the treatment of such patients
Desmoid tumors (DT), known as aggressive and to emphasize the importance of multidisciplinary fibromatosis or musculoaponeurotic fibromatosis, are approach in the treatment of such patients.fibroblastic tumors of intermediate malignant potential.They are characterized by slow growth, tendency for local recurrence and absence of metastatic potential
Radiotherapy has been incorporated into the management of extra-abdominal DT, either as an adjuvant treatment after surgical resection in cases of positive surgical margins or as a primary treatment when surgical resection is not feasible or may result in significant loss of function[4]
Summary
Desmoid tumors (DT), known as aggressive and to emphasize the importance of multidisciplinary fibromatosis or musculoaponeurotic fibromatosis, are approach in the treatment of such patients. They are characterized by slow growth, tendency for local recurrence and absence of metastatic potential. The term “desmoid”, coined by Muller in 1838, is derived from the Greek word “desmos”, meaning tendon-like. DTs are extremely rare and account for less than 0.03% of all tumors[1]. Surgical resection and radiotherapy are standard treatment options for these patients[2,3,4]. Recurrence rate is high during DT management. Systemic treatment with Imatinib for unresectable or recurrent DTs could be acceptable therapeutic option[5]
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