Diagnosis and treatment of intra-abdominal aggressive fibromatosis: a report of 8 cases_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

REN Xiangxiang ¹ ,  TIAN Yu ¹ , XIAO Gang ¹ , ZHANG Meng ²

1. Department of General Surgery, Shengjing Hospital of China Medical University, Shenyang 110004, P. R. China;
2. Department of Dermatology, The First Affiliated Hospital of China Medical University, Shenyang 110004, P. R. China;

Corresponding author：

TIAN Yu, Email: 18940256588@163.com

Keywords：

intra-abdominal aggressive fibromatosis; diagnosis; treatment; recurrence

DOI：

10.7507/1007-9424.201705090

Video：

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Objective The aim of this article is to analyze the clinical characteristics of intra-abdominal aggressive fibromatosis and discuss its treatment methods. Methods Retrospective analysis of the clinical data in 8 cases of intra-abdominal aggressive fibromatosis who were confirmed by surgery and pathological diagnosis between Feb. 2011 and Mar. 2017 in Shengjing hospital was performed. Results Of the 8 cases (3 males and 5 females), there were 4 cases of simple abdominal pain (2 cases of repeated intermittent abdominal pain and 2 cases of acute abdominal pain), 3 cases of abdominal mass, and 1 case of relapses after surgery in outside hospital (others 7 cases were the first visit). The course of disease was 4 to 720 d, with median of 130 d. All cases underwent radical surgery, and the operative time was 92 to 493 min, with an average of 246 min. Intraoperative blood loss was 20 to 1 000 mL, with an average of 321 mL. The drainage tube placement time in all patients was 5 to 9 d, with an average of 6 d. The hospital stay was 11 to 75 d, with an average of 25 d. Two cases suffered from postoperatively appeared abdominal pain symptoms. All cases were followed-up for 6 to 40 months, with median of 23 months. During the follow-up period, 1 case relapsed on 212 dafter surgery, and the remaining 7 cases had no recurrence. Conclusion For intra-abdominal aggressive fibromatosis, it is difficult to make clear diagnosis before operation and surgery is the primary choice of treatment when symptom appeared.

Citation： REN Xiangxiang, TIAN Yu, XIAO Gang, ZHANG Meng. Diagnosis and treatment of intra-abdominal aggressive fibromatosis: a report of 8 cases. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2017, 24(11): 1361-1364. doi: 10.7507/1007-9424.201705090 Copy

1.	Dalén BP, Bergh PM, Gunterberg BU. Desmoid tumors: a clinical review of 30 patients with more than 20 years’ follow-up. Acta Orthop Scand, 2003, 74(4): 455-459.
2.	Mueller J. Ueber den feineren bau und die formender krankhaften geschwulste. Berlin: G. Reimer, 1838: 60.
3.	Jo VY, Fletcher CD. WHO classification of soft tissue tumours: an update based on the 2013 (4^th) edition. Pathology, 2014, 46(2): 95-104.
4.	van Broekhoven DL, Grünhagen DJ, den Bakker MA, et al. Time trends in the incidence and treatment of extra-abdominal and abdominal aggressive fibromatosis: a population-based study. Ann Surg Oncol, 2015, 22(9): 2817-2823.
5.	Rampone B, Pedrazzani C, Marrelli D, et al. Updates on abdominal desmoid tumors. World J Gastroenterol, 2007, 13(45): 5985-5988.
6.	Ballo MT, Zagars GK, Pollack A, et al. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol, 1999, 17(1): 158-167.
7.	Zlotecki RA, Scarborough MT, Morris CG, et al. External beam radiotherapy for primary and adjuvant management of aggressive fibromatosis. Int J Radiat Oncol Biol Phys, 2002, 54(1): 177-181.
8.	Posner MC, Shiu MH, Newsome JL, et al. The desmoid tumor. Not a benign disease. Arch Surg, 1989, 124(2): 191-196.
9.	Hatzimarkou A, Filippou D, Papadopoulos V, et al. Desmoid tumor in Gardner’s Syndrome presented as acute abdomen. World J Surg Oncol, 2006, 4: 18.
10.	Kasper B, Ströbel P, Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist, 2011, 16(5): 682-693.
11.	Clark SK, Phillips RK. Desmoids in familial adenomatous polyposis. Br J Surg, 1996, 83(11): 1494-1504.
12.	Ihalainen HR, Koljonen V, Böhling TO, et al. The desmoid tumour: local control after surgical treatment. J Plast Surg Hand Surg, 2015, 49(1): 19-24.
13.	Tolan S, Shanks JH, Loh MY, et al. Fibromatosis: benign by name but not necessarily by nature. Clin Oncol (R Coll Radiol), 2007, 19(5): 319-326.
14.	Faria SC, Iyer RB, Rashid A, et al. Desmoid tumor of the small bowel and the mesentery. AJR Am J Roentgenol, 2004, 183(1): 118.
15.	Nakayama T, Tsuboyama T, Toguchida J, et al. Natural course of desmoid-type fibromatosis. J Orthop Sci, 2008, 13(1): 51-55.
16.	Lazar AJ, Hajibashi S, Lev D. Desmoid tumor: from surgical extirpation to molecular dissection. Curr Opin Oncol, 2009, 21(4): 352-359.
17.	刘啸峰, 丁玉芹, 何德明, 等. 腹内型侵袭性纤维瘤病多排螺旋 CT 和 MRI 诊断价值. 蚌埠医学院学报, 2015, 40(2): 238-241.
18.	Kamali F, Wang WL, Guadagnolo BA, et al. MRI may be used as a prognostic indicator in patients with extra-abdominal desmoid tumours. Br J Radiol, 2016, 89(1058): 20150308.
19.	He XD, Zhang YB, Wang L, et al. Prognostic factors for the recurrence of sporadic desmoid-type fibromatosis after macroscopically complete resection: analysis of 114 patients at a single institution. Eur J Surg Oncol, 2015, 41(8): 1013-1019.
20.	Fiore M, Rimareix F, Mariani L, et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol, 2009, 16(9): 2587-2593.
21.	Mullen JT, Delaney TF, Kobayashi WK, et al. Desmoid tumor: analysis of prognostic factors and outcomes in a surgical series. Ann Surg Oncol, 2012, 19(13): 4028-4035.
22.	Crago AM, Denton B, Salas S, et al. A prognostic nomogram for prediction of recurrence in desmoid fibromatosis. Ann Surg, 2013, 258(2): 347-353.
23.	Ergen ŞA, Tiken EE, Öksüz DÇ, et al. The role of radiotherapy in the treatment of primary or recurrent desmoid tumors and long-term results. Balkan Med J, 2016, 33(3): 316-321.
24.	Lev D, Kotilingam D, Wei C, et al. Optimizing treatment of desmoid tumors. J Clin Oncol, 2007, 25(13): 1785-1791.
25.	Ballo MT, Zagars GK, Pollack A. Radiation therapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys, 1998, 42(5): 1007-1014.
26.	Park KH, Choi YJ, Kim KW, et al. Combination chemotherapy with methotrexate and vinblastine for surgically unresectable, aggressive fibromatosis. Jpn J Clin Oncol, 2016, 46(9): 845-849.

1. Dalén BP, Bergh PM, Gunterberg BU. Desmoid tumors: a clinical review of 30 patients with more than 20 years’ follow-up. Acta Orthop Scand, 2003, 74(4): 455-459.
2. Mueller J. Ueber den feineren bau und die formender krankhaften geschwulste. Berlin: G. Reimer, 1838: 60.
3. Jo VY, Fletcher CD. WHO classification of soft tissue tumours: an update based on the 2013 (4^th) edition. Pathology, 2014, 46(2): 95-104.
4. van Broekhoven DL, Grünhagen DJ, den Bakker MA, et al. Time trends in the incidence and treatment of extra-abdominal and abdominal aggressive fibromatosis: a population-based study. Ann Surg Oncol, 2015, 22(9): 2817-2823.
5. Rampone B, Pedrazzani C, Marrelli D, et al. Updates on abdominal desmoid tumors. World J Gastroenterol, 2007, 13(45): 5985-5988.
6. Ballo MT, Zagars GK, Pollack A, et al. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol, 1999, 17(1): 158-167.
7. Zlotecki RA, Scarborough MT, Morris CG, et al. External beam radiotherapy for primary and adjuvant management of aggressive fibromatosis. Int J Radiat Oncol Biol Phys, 2002, 54(1): 177-181.
8. Posner MC, Shiu MH, Newsome JL, et al. The desmoid tumor. Not a benign disease. Arch Surg, 1989, 124(2): 191-196.
9. Hatzimarkou A, Filippou D, Papadopoulos V, et al. Desmoid tumor in Gardner’s Syndrome presented as acute abdomen. World J Surg Oncol, 2006, 4: 18.
10. Kasper B, Ströbel P, Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist, 2011, 16(5): 682-693.
11. Clark SK, Phillips RK. Desmoids in familial adenomatous polyposis. Br J Surg, 1996, 83(11): 1494-1504.
12. Ihalainen HR, Koljonen V, Böhling TO, et al. The desmoid tumour: local control after surgical treatment. J Plast Surg Hand Surg, 2015, 49(1): 19-24.
13. Tolan S, Shanks JH, Loh MY, et al. Fibromatosis: benign by name but not necessarily by nature. Clin Oncol (R Coll Radiol), 2007, 19(5): 319-326.
14. Faria SC, Iyer RB, Rashid A, et al. Desmoid tumor of the small bowel and the mesentery. AJR Am J Roentgenol, 2004, 183(1): 118.
15. Nakayama T, Tsuboyama T, Toguchida J, et al. Natural course of desmoid-type fibromatosis. J Orthop Sci, 2008, 13(1): 51-55.
16. Lazar AJ, Hajibashi S, Lev D. Desmoid tumor: from surgical extirpation to molecular dissection. Curr Opin Oncol, 2009, 21(4): 352-359.
17. 刘啸峰, 丁玉芹, 何德明, 等. 腹内型侵袭性纤维瘤病多排螺旋 CT 和 MRI 诊断价值. 蚌埠医学院学报, 2015, 40(2): 238-241.
18. Kamali F, Wang WL, Guadagnolo BA, et al. MRI may be used as a prognostic indicator in patients with extra-abdominal desmoid tumours. Br J Radiol, 2016, 89(1058): 20150308.
19. He XD, Zhang YB, Wang L, et al. Prognostic factors for the recurrence of sporadic desmoid-type fibromatosis after macroscopically complete resection: analysis of 114 patients at a single institution. Eur J Surg Oncol, 2015, 41(8): 1013-1019.
20. Fiore M, Rimareix F, Mariani L, et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol, 2009, 16(9): 2587-2593.
21. Mullen JT, Delaney TF, Kobayashi WK, et al. Desmoid tumor: analysis of prognostic factors and outcomes in a surgical series. Ann Surg Oncol, 2012, 19(13): 4028-4035.
22. Crago AM, Denton B, Salas S, et al. A prognostic nomogram for prediction of recurrence in desmoid fibromatosis. Ann Surg, 2013, 258(2): 347-353.
23. Ergen ŞA, Tiken EE, Öksüz DÇ, et al. The role of radiotherapy in the treatment of primary or recurrent desmoid tumors and long-term results. Balkan Med J, 2016, 33(3): 316-321.
24. Lev D, Kotilingam D, Wei C, et al. Optimizing treatment of desmoid tumors. J Clin Oncol, 2007, 25(13): 1785-1791.
25. Ballo MT, Zagars GK, Pollack A. Radiation therapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys, 1998, 42(5): 1007-1014.
26. Park KH, Choi YJ, Kim KW, et al. Combination chemotherapy with methotrexate and vinblastine for surgically unresectable, aggressive fibromatosis. Jpn J Clin Oncol, 2016, 46(9): 845-849.

CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Diagnosis and treatment of intra-abdominal aggressive fibromatosis: a report of 8 cases

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