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Selected Publication:
Fraissler, L.
Therapy of infantile digital fibromatoses and desmoid tumors inchildren and adolescents.
[ Diplomarbeit/Master Thesis ] Graz Medical University; 2009. pp.50.
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- Authors Med Uni Graz:
- Advisor:
- Benesch Martin
- Leithner Andreas
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- Abstract:
- Background: Several types of fibromatoses can occur in children and adolescents. Because of their high recurrence rates of up to 60% and 88%, infantile digital fibromatosis (IDF) and desmoid-type fibromatosis (AF) especially pose a challenge to the attending physician. While some authors suggest a wait and see policy for IDF, others recommend surgical excision. First choice treatment of AF still remains wide or radical resection whereas little is known about non-surgical treatment. Despite non-cytotoxic and cytotoxic therapy theres still a tendency toward local recurrence. Other publications showed possible correlations between immunohistochemical markers and therapy of IDF and AF. Objective: To analyse clinical appearance and immunohistochemical patterns of infantile fibromatoses and to evaluate possible pharmacological treatment. Patients and Methods: Nineteen patients with AF and three patients with IDF were included. The age limit was set at 20 years. Mean age at diagnosis was 6.3 years (range 0 to 17 years). We evaluated margins at resection, medical treatment, time of recurrence, as well as immunohistochemical markers (estrogen receptor and , progesterone and androgen receptors, somatostatin, Ki-67, c-kit, platelet-derived growth factor and and -catenin). Results: The mean follow up was 112 months (range 2 to 251 months). Two of three patients with IDF and four of 19 patients with desmoid-type fibromatosis showed recurrences after a mean time of 21 and 55 months. There was no correlation between positive immunohistochemical markers and tumor recurrences detectable. Conclusion: Despite high primary recurrence rates and R1/2 resections, infantile digital fibromatoses showed no more recurrences. Type of resection (R0, R1/2) of desmoid tumors did not influence recurrence rate. In conclusion desmoids seem to behave in a different way in children and adolescents than in adult patients. The heterogeneity of treatment made it impossible to give a statement about therapy management. Therefore, we again state that only a large, prospective, randomized trial could really provide an answer for the efficacy of any adjuvant treatment. Nevertheless we recommend wide excision of primary desmoid tumors as treatment of choice. If an appropriate resection is not possible or associated with a high morbidity a wait-and-see policy might be indicated.