Desmoid tumor: from surgical extirpation to molecular dissection.
OBJECTIVE
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 beta-catenin in desmoid tumor biology.


RESULTS
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.


CONCLUSIONS
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.
Lazar
Alexander J F
AJ
Department of Pathology, University of Texas M. D. Anderson Cancer Center, Houston, Texas 77030-5009, USA. alazar@mdanderson.org
Hajibashi
Shohrae
S
Lev
Dina
D
D003131:Combined Modality Therapy; D018222:Desmoid Tumors / Q000235:genetics / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D006801:Humans; D008637:Mesenchymoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D051153:Wnt Proteins / Q000378:metabolism; D051176:beta Catenin / Q000235:genetics / Q000378:metabolism
C495270:CTNNB1 protein, human; D051153:Wnt Proteins; D051176:beta Catenin