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Woltsche, N; Gilg, MM; Fraissler, L; Liegl-Atzwanger, B; Beham, A; Lackner, H; Benesch, M; Leithner, A.
Is wide resection obsolete for desmoid tumors in children and adolescents? Evaluation of histological margins, immunohistochemical markers, and review of literature.
Pediatr Hematol Oncol. 2015; 32(1):60-69
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Autor/innen der Med Uni Graz:
Beham Alfred
Benesch Martin
Gilg Magdalena Maria
Lackner Herwig
Leithner Andreas
Liegl-Atzwanger Bernadette
Woltsche Nora

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Desmoid fibromatosis is a benign fibroblastic neoplasm with high recurrence rates predominantly observed in pediatric and adolescent patients. The use of wide resection margins has been discussed controversially in literature. In addition, data on non-surgical treatment is limited as phase III studies are still missing. Nineteen patients under the age of 18 years were identified. Tumor location, surgical treatment for primary or recurrent tumors, resection margins, medical neo-/adjuvant treatment, time to recurrence as well as immunohistochemical markers (estrogen receptor, ER α and β, progesterone and androgen receptors, somatostatin, Ki-67, c-kit, platelet-derived growth factor receptors, PDGFRs, α and β, β-catenin) were evaluated. The mean age at diagnosis was 6.6 years, with a mean follow-up of 114 months. Recurrences were detected in four out of nineteen patients. Surprisingly, the recurrence rate was not influenced by type of resection used (R0, R1/2). All samples were tested negative for ER α, somatostatin, and progesterone receptor. In contrast, a majority of tumors showed positive results for PDGFR α and β and β-catenin. No correlation between positive immunohistochemical markers and tumor recurrences was detectable. In conclusion, recurrence rates are not depending on resection type and immunohistochemical markers seem to behave differently in children and adolescents in contrast to adult patients.
Find related publications in this database (using NLM MeSH Indexing)
Abdominal Neoplasms - metabolism
Abdominal Neoplasms - pathology
Abdominal Neoplasms - surgery
Adenomatous Polyposis Coli - metabolism
Adenomatous Polyposis Coli - pathology
Adenomatous Polyposis Coli - surgery
Adolescent -
Adult -
Biomarkers, Tumor - metabolism
Child -
Child, Preschool -
Female -
Fibromatosis, Aggressive - metabolism
Fibromatosis, Aggressive - pathology
Fibromatosis, Aggressive - surgery
Follow-Up Studies -
Humans -
Infant -
Infant, Newborn -
Male -
Neoplasm Recurrence, Local - metabolism
Neoplasm Recurrence, Local - pathology
Neoplasm Recurrence, Local - surgery
Retrospective Studies -

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aggressive fibromatosis in children and adolescents
type of resection
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