Progress on Diagnosis and Treatment of Abdominal Aggressive Fibromatosis_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

CAO feng ¹ ,  XIE Bin ² , LIU Min. ³

1.Department of General Surgery， Central Hospital of Zaozhuang Mining Group of Shandong， Zaozhuang 277000， Shandong Province， China;;
2.Department of Urology， Tengzhou Central People’s Hospital， Tengzhou 277500， Shandong Province， China;;
3.Department of Operating Theatre， Central Hospital of Zaozhuang Mining Group of Shandong， Zaozhuang 277000， Shandong Province， China;

Corresponding author：

XIE Bin, Email: xie_b@hotmail.com

Keywords：

Fibromatosis; Invasive; Abdomen; Surgical operation; Radiotherapy; Chemotherapy

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Objective 　To comprehend the concept， pathology， molecular mechanisms， diagnosis， and treatment
of aggressive fibromatosis （AF）， and to find a novel way to cure aggressive fibromatosis.
Method 　The literatures about the definition， molecular mechanisms， and clinical research of AF were reviewed and analized.
Results 　AF is rare and benign fibromatous lesion that is the result of abnormal proliferation of myofibroblasts. The pathologic features of AF is
a benign disease， but it has “malignant” biological behavior. The tumor often involved the surrounding organs and blood
vessels， and caused death of patients. For patients with clinical symptoms or complications， complete excision of the
tumor is the treatment of choice. Even if the operation to ensure the negative margin also has a higher recurrence rate， so
its treatment requires multidisciplinary treatment.
Conclusions 　The mechanism of AF is very complex， and it’s mecha-
nism is still unclear. Clinical management of patients with AF is difficult and controversial， at present， the most effective treatment for AF is operation resection. The effects of adjuvant radiotherapy， chemotherapy， and other treatment after operation for AF still need further study.

Citation： CAO feng,XIE Bin,LIU Min.. Progress on Diagnosis and Treatment of Abdominal Aggressive Fibromatosis. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2013, 20(10): 1193-1202. doi: Copy

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2. Möslein G， Dozois RR. Desmoid tumors associated with familial adenomatous polyposis[J]. Perspect Col Rect Surg， 1998， 10：109-126.
3. Papagelopoulos PJ， Mavrogcnis AF， Mitsiokapa EA， et al. Current trends in the management of extra-abdominal desmoid turnouts[J]. World J Surg Onc01， 2006， 4：2l-29.
4. Goldblum J， Fletcher JA. Desmoid-type fibromatosis//Fletcher CDM， Unni KK， Mertens F， eds. World Health Organization Classification of Tumors： Pathology and Genetics of Tumors of Soft Tissue and Bone[M]. 1st ed. Lyon：IARC Press， 2002：83-84.
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6. Muller J. Ueber den feineren Bau und die Formen der krankhaftenGeschwulste[M]. Berlin：G.Reimer， 1838：60.
7. Nuyttens JJ， Rust PF， Thomas CR Jr， et al. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors. A comparative review of 22 articles[J]. Cancer， 2000， 88(7)：1517-1523.
8. Papagelopoulos PJ， Mavrogenis AF， Mitsiokapa EA， et al. Current trends in the management of extra-abdominal desmoid tumours[J]. World J Surg Oncol， 2006， 4：21-28.
9. Lee JC， Thomas JM， Phillips S， et al. Aggressive fibromatosis：MRI features with pathologic correlation[J]. AJR Am J Roentgenol， 2006， 186(1)：247-254.
10. Ferenc T， Sygut J， Kopczyński J， et al. Aggressive fibromatosis (desmoid tumors)：definition， occurrence， pathology， diagnostic，problems， clinical behavior， genetic background[J]. Pol J Pathol， 2006， 57(1)：5-15.
11. Stuff NJ， Clark SK. Current ideas in desmoid tmours[J]. Fam Cancer， 2006， 5(3)：275-285.
12. Caspari R， Olschwang S， Friedl W， et al. Familial adenomatouspolyposis：desmoid tumours and lack of ophthalmic lesions (CHRPE)associated with APC mutations beyond codon 1444[J]. Hum Mol Genet， 1995， 4(3)：337-340.
13. Sturt NJ， Gallagher MC， Bassett P， et al. Evidence for genetic predisposition to desmoid tumours in familial adenomatous poly-posis independent of the germline APC mutation[J]. Gut， 2004，.
14. Alman BA， Li C， Pajemki ME， et al. Increased betacateninprotein and somatic APC mutations in sporadic aggressive fibrom-atoses (desmoid tumors)[J]. Am J Pathol， 1997， 151(2)：329-334.
15. Bertario L， Russe A， Sah P， et al. Genotype and phenotypefactors as determinants of desmoid tumors in patients with familialadenomatous polyposis[J]. Int J Cancer， 2001， 95(2)： 102-107.
16. Soravia C， Berk T， McLeod RS， et al. Desmoid disease in patients with familial adenomatous polyposis[J]. Dis Colon Rectmn， 2000， 43(3)：363-369.
17. Friedl W， Caspari R， Sengteller M， et al. Can APC mutation analysis contribute to therapeutic decisions in familial adenomatous polyposis? Experience from 680 FAP families[J]. Gut， 2001， 48(4)：515-521.
18. (1)：158-167.
19. Sakorafas GH， Nissetakis C， Peros G. Abdominal desmoid tumors[J]. Surg Oncol， 2007， 16(2)：131-142.
20. Church JM. Anatomy of a gene：functional correlations of APC mutation[J]. Semin Colon Rectum Surg， 1998， 9：49-52.
21. Hosalkar HS， Fox EJ， Delaney T， et al. Desmoid tumors and current status of management[J]. Orthop CIin North Am， 2006， 37(1)：53-63.
22. Brueckl WM， Ballhausen WG， Förtsch T， et al. Genetic testing for germline mutations of the APC gene in patients with apparentlysporadic desmoid tumors but a family history of colorectal carcinoma[J]. Dis Colon Rectum， 2005， 48(6)：1275-1281.
23. Latchford AR， Sturt NJH， Neale K， et al. A 10-year review of surgery for desmoid disease associated with familial adenomatous polyposis[J]. Br J Surg， 2006， 93(10)： 1258-1264.
24. Lopez R， Kemalyan N， Moseley HS， et al. Problems in diagnosis and management of desmoid tumors[J]. Am J Surg， 1990， 159(5)：450-453.
25. Stoeckle E， Coindre JM， Longy M， et al. A critical analysis of treatment strategies in desmoid turnouts：a review of a series 0f 106 cases[J]. Eur J Surg Oncol， 2009， 35(2)：129-134.
26. Clark SK， Phillips RK. Desmoids in familial adenomatous polyposis[J]. Br J Surg， 1996， 83(11)：1494-1504.
27. (5)：501-506.
28. Goy BW， Lee SP， Eilber F， et al. The role of adjuvant radiotherapy in the treatment of resectable desmoid tumors[J]. Int J Radiat Oncol Biol Phys， 1997， 39(3)：659-665.
29. Clark SK， Neale KF， Landgrebe JC， et al. Desmoid tumours complicating familial adenomatous polyposis[J]. Br J Surg， 1999， 86(9)：1185-1189.
30. Reitamo JJ， Scheinin TM， Häyry P. The desmoid syndrome：new aspects in the cause， pathogenesis and treatment of the desmoid tumour[J]. Am J Surg， 1986， 151(2)：230-237.
31. Lotfi AM， Dozois RR， Gordon H， et al. Mesenteric fibromatosiscomplicating familial adenomatous polyposis：predisposing factorsand results of treatment[J]. Int J Colorectal Dis， 1989， 4(1)：30-36.
32. Sturt JNH， Clark SK. Current ideas in desmoid tumors[J]. Fam Cancer， 2006， 5(3)：275-285.
33. Wilcken N， Tattersall MH. Endocrine therapy for desmoid tumours[J]. Cancer， 1991， 68(6)：1384-1388.
34. Dhingra K. Antiestrogens-tamoxifen， SERMs and beyond[J]. Invest New Drugs， 1999， 17(3)：285-311.
35. Church J， Berk T， Boman BM， et al. Staging intra-abdominal desmoid tumors in familial adenomatous polyposis：a search for a uniform approach to a troubling disease[J]. Dis Colon Rectum， 2005， 48(8)：1528-1534.
36. Middleton SB， Pack K， Phillips RK. Telomere Length in familialadenomatous polyposis-associated desmoids[J]. Dis Colon Rectum， 2000， 43(11)：1535-1539.
37. Bhattacharya B， Dilworth HP， Iacobuzio-Donahue C， et al. Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions[J]. Am J Surg Pathol， 2005， 29(5)：653-659.
38. Leithner A， Gapp M， Radl R， et al. Immunohistochemical analysis of desmoid tumours[J]. J Clin Pathol， 2005， 58(11)：1152-1156.
39. Lim CL， Walker MJ， Mehta RR， et al. Estrogen and antiestrogen binding sites in desmoid tumors[J]. Eur J Cancer Clin Oncol， 1986， 22(5)：583-587.
40. Sørensen A， Keller J， Nielsen OS， et al. Treatment of aggressivefibromatosis：a retrospective study of 72 patients followed for 1-27 years[J]. Acta Orthop Scand， 2002， 73(2)： 213-219.
41. Hartley JE， Church JM， Gupta S， et al. Significance of incidentaldesmoids identified during surgery for familial adenomatous polyposis[J]. Dis Colon Rectum， 2004， 47(3)：334-340.
42. Anthony T， Rodriguez-Bigas MA， Weber T， et al. Desmoidtumors[J]. J Am Coll Surg， 1996， 182(4)：369-377.
43. Dozois EJ， Dozois RR. Desmoid disease. //Kelly KA， Sarr MG， Hinder RA， eds. Mayo clinic gastrointestinal surgery[M]. Saunders Eds：Philadelphia， 2004：563-565.
44. Spear MA， Jennings LC， Mankin HJ， et al. Individualizing management of aggressive fibromatoses[J]. Int J Radiat Oncol Biol Phys， 1998， 40(3)：637-645.
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CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Progress on Diagnosis and Treatment of Abdominal Aggressive Fibromatosis

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