Clinical Characteristics and Adequate Treatment of Familial Adenomatous Polyposis Combined with Desmoid Tumors
2
조회
0
다운로드
2
인용
0
참고문헌
키워드
초록
PurposeThe objective of this study was to examine the clincopathologic characteristics andoutcomes of familial adenomatous polyposis (FAP) patients with and without desmoidtumors (DTs), including the risk factors for progression of FAP-related DTs. Materials and MethodsWe reviewed the medical records and database of all patients with FAP who were treatedbetween January 1993 and December 2011. ResultsOf 75 FAP patients, 18 (24%) were FAP with DTs. Seventeen of these had intra-abdominalDTs and one had intra- and extra-abdominal DTs. We divided the patients into two groupsaccording to type of resection; the R0 or R1 resection group, referred to as the curativeresection group (eight patients), and the R2 resection/palliative operation/medicaltreatment group, referred to as the palliative resection group (10 patients). Two patients inthe curative resection group and two patients in the palliative group had progressed totumor growth by the follow-up (p=0.800). In univariate analysis, DT diagnosis before orsimultaneously with FAP diagnosis (DTs unrelated to surgical trauma) was a significant riskfactor for tumor progression at final follow-up. ConclusionA multidisciplinary approach to DT treatment is needed, including nonsteroidal antiinflammatorydrugs, anti-estrogens, cytotoxic agents, and surgery. However, the role ofsurgery in resectable and complicated tumors may be limited. DT unrelated to surgicaltrauma has a relatively poor prognosis.
Abstract
PurposeThe objective of this study was to examine the clincopathologic characteristics andoutcomes of familial adenomatous polyposis (FAP) patients with and without desmoidtumors (DTs), including the risk factors for progression of FAP-related DTs. Materials and MethodsWe reviewed the medical records and database of all patients with FAP who were treatedbetween January 1993 and December 2011. ResultsOf 75 FAP patients, 18 (24%) were FAP with DTs. Seventeen of these had intra-abdominalDTs and one had intra- and extra-abdominal DTs. We divided the patients into two groupsaccording to type of resection; the R0 or R1 resection group, referred to as the curativeresection group (eight patients), and the R2 resection/palliative operation/medicaltreatment group, referred to as the palliative resection group (10 patients). Two patients inthe curative resection group and two patients in the palliative group had progressed totumor growth by the follow-up (p=0.800). In univariate analysis, DT diagnosis before orsimultaneously with FAP diagnosis (DTs unrelated to surgical trauma) was a significant riskfactor for tumor progression at final follow-up. ConclusionA multidisciplinary approach to DT treatment is needed, including nonsteroidal antiinflammatorydrugs, anti-estrogens, cytotoxic agents, and surgery. However, the role ofsurgery in resectable and complicated tumors may be limited. DT unrelated to surgicaltrauma has a relatively poor prognosis.
참고문헌 (25)
- Lynch HT, "Hereditary colorectal cancer syndromes: molecular genetics, genetic counseling, diagnosis and management", Fam Cancer, 7, pp.27~39 (2008)
- Vasen HF, "Guidelines for the clinical management of familial adenomatous polyposis (FAP)", Gut, 57, pp.704~713 (2008)
- Sinha A, "Risk factors predicting desmoid occurrence in patients with familial adenomatous polyposis: a meta-analysis", Colorectal Dis, 13, pp.1222~1229 (2011)
- Arvanitis ML, "Mortality in patients with familial adenomatous polyposis", Dis Colon Rectum, 33, pp.639~642 (1990)
- Sturt NJ, "Current ideas in desmoid tumours", Fam Cancer, 5, pp.275~285 (2006)
- Gurbuz AK, "Desmoid tumours in familial adenomatous polyposis", Gut, 35, pp.377~381 (1994)
- Rodriguez-Bigas MA, "Desmoid tumors in patients with familial adenomatous polyposis", Cancer, 74, pp.1270~1274 (1994)
- Clark SK, "Desmoid tumours complicating familial adenomatous polyposis", Br J Surg, 86, pp.1185~1189 (1999)
- Speake D, "Desmoid tumours in patients with familial adenomatous polyposis and desmoid region adenomatous polyposis coli mutations", Br J Surg, 94, pp.1009~1013 (2007)
- Scott RJ, "Familial infiltrative fibromatosis (desmoid tumours) (MIM135290) caused by a recurrent 3' APC gene mutation", Hum Mol Genet, 5, pp.1921~1924 (1996)
- Latchford AR, "A 10-year review of surgery for desmoid disease associated with familial adenomatous polyposis", Br J Surg, 93, pp.1258~1264 (2006)
- Nieuwenhuis MH, "A nation-wide study comparing sporadic and familial adenomatous polyposisrelated desmoid-type fibromatoses", Int J Cancer, 129, pp.256~261 (2011)
- 김대동, "가족성 용종증에서 유건종의 발생양상", Annals of Coloproctolgy, 24(1), pp.20~26 (2008), 대한대장항문학회
- Heiskanen I, "Occurrence of desmoid tumours in familial adenomatous polyposis and results of treatment", Int J Colorectal Dis, 11, pp.157~162 (1996)
- Nieuwenhuis MH, "Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients", Br J Cancer, 104, pp.37~42 (2011)
- Janinis J, "The pharmacological treatment of aggressive fibromatosis: a systematic review", Ann Oncol, 14, pp.181~190 (2003)
- Hansmann A, "High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors", Cancer, 100, pp.612~620 (2004)
- Heinrich MC, "Phase II, open-label study evaluating the activity of imatinib in treating life-threatening malignancies known to be associated with imatinib-sensitive tyrosine kinases", Clin Cancer Res, 14, pp.2717~2725 (2008)
- Heinrich MC, "Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor)", J Clin Oncol, 24, pp.1195~1203 (2006)
- Gega M, "Successful chemotherapeutic modality of doxorubicin plus dacarbazine for the treatment of desmoid tumors in association with familial adenomatous polyposis", J Clin Oncol, 24, pp.102~105 (2006)
- Constantinidou A, "Pegylated liposomal doxorubicin, an effective, well-tolerated treatment for refractory aggressive fibromatosis", Eur J Cancer, 45, pp.2930~2934 (2009)
- Constantinidou A, "Advanced aggressive fibromatosis: Effective palliation with chemotherapy", Acta Oncol, 50, pp.455~461 (2011)
- O'Dea FJ, "Preoperative radiotherapy is effective in the treatment of fibromatosis", Clin Orthop Relat Res, (415), pp.19~24 (2003)
- Durno C, "Does early colectomy increase desmoid risk in familial adenomatous polyposis?", Clin Gastroenterol Hepatol, 5, pp.1190~1194 (2007)
- Quintini C, "Mortality of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis: a single center review of 154 patients", Ann Surg, 255, pp.511~516 (2012)