Desmoid tumor: From surgical extirpation to molecular dissection

Alexander J.F. Lazar, Shohrae Hajibashi, Dina Lev

Research output: Contribution to journalReview articlepeer-review

Abstract

Purpose of review: Desmoid tumors are associated with a variable and unpredictable clinical course. Surgery is the therapeutic mainstay, but there has been much discussion of late regarding its proper application. Little is known regarding the molecular determinates of desmoid tumor behavior. Some recent work has focused on the role of p-catenin in desmoid tumor biology. Recent findings: Given the variable clinical course of desmoid tumors, the interpretation of factors classically associated with recurrence such as microscopic status of margins appears more nuanced that previously thought. The application of multidisciplinary assessment with multimodality treatment, including surgery, radiation and systemic therapies may underlie these changes and now form the basis of care for this tumor. The precise CTNNB1 mutation present appears to be strongly predictive of recurrence after initial resection in one large, retrospective, multivariate analysis. Summary: Establishing the population benefiting most from various treatment modalities and combinations is critical for progress in this disease. Assessment and treatment of individual patients in a multidisciplinary setting is critical to achieve the most favorable outcome. Additional study of the molecular determinates of desmoid behavior is needed to guide therapeutic selection.

Original languageEnglish
Pages (from-to)352-359
Number of pages8
JournalCurrent Opinion in Oncology
Volume21
Issue number4
DOIs
StatePublished - Jul 2009
Externally publishedYes

Keywords

  • CTNN
  • Desmoid fibromatosis
  • Mutations
  • P-catenin
  • Prognosis

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