Progress and hot topics in clinical management of desmoid tumor
- VernacularTitle:韧带样瘤的诊治进展及热点问题
- Author:
Kai HUANG
;
Hong FU
- Publication Type:Journal Article
- Keywords:
desmoid tumor;
surgery;
radiation therapy;
drug therapy
- From:China Oncology
2010;20(3):227-231
- CountryChina
- Language:Chinese
-
Abstract:
Desmoid tumor also known as aggressive fibromatosis,are rare fibroblastic tumors which are derived from deep fascia planes or muscuofascia structures.Resectable extra-abdominal and abdominal desmoid tumor should always be treated with the aim of achieving a negative margin through wide radical resection with margins above 2 cm.Ifthis is not feasible,function-sparing surgical resection is suggested.Adjuvant radiation should be considered for patients with positive margins,recirrent tumors or unresectable diseases.Intra-abdominal desmoid tumor is common in patients with familial adenomatous polyposis.It has a high recurring rate after surgical resection and therefore a high dosage of tamoxifen and sulindac are recommended as first-line treatment.Due to the variable nature of the outcome and vague reaction to drug therapy,individualized treatments,including the wait and see policy,has been widely investigated and thought to be a promising strategy for the future.
