MANAGEMENT OUTCOMES OF EXTRA ABDOMINAL FIBROMATOSIS AMONG ADULT PATIENTS TREATED AT A TERTIARY CARE HOSPITAL

Authors

  • Masood Umer Aga khan university hospital
  • Javeria Saeed Aga khan university hospital
  • Irfan Anwer Aga khan university hospital
  • Nida Zahid Aga khan university hospital
  • Younus Durrani Jinnah post graduate medical college

Abstract

Background: Aggressive fibromatosis or desmoid fibromatosis is a soft tissue neoplasm which is non-metastatic in nature. Among all soft tissue tumours, it comprises of 3% cases and is more common in females as compared to males. Objective of our study was to evaluate the treatment outcomes of extra abdominal fibromatosis in patients who were treated in our setup and determine the recurrence patterns. Methods: It is retrospective cohort of 15 patients that were treated in section of Orthopaedics, department of surgery, Aga Khan University hospital, Karachi from January 1990 to December 2015. It included all adult patients of age >18 years with biopsy proven extra abdominal aggressive fibromatosis. Data was analysed on SPSS 22 version. Results: Out of 15 patients, there were 7 males (46.7%) and 8 females (53.3%). Median age was 22 years. Majority of patients [10 (66.6%)] had upper limb lesion. On initial biopsy we had 11 (73.3%) cases of primary fibromatosis while 2 (13.3%) were recurrent and 2 (13.3%) were spindle cell carcinoma. The median (IQR) follow-up time of the participants was 3 (2–3) months. Complications occurred in 8 (53.3%) patients. A significant difference was observed in the haemoglobin levels before and after surgery with a mean difference of 2.74 (p-value<0.001). Recurrence of disease occurred in 4 (26.7%) patients and all of these patients who had recurrence underwent 2nd surgery versus 1 of the participants who had second surgery without recurrence and this was a significant difference (p value <0.004). Conclusion: Extra abdominal fibromatosis is commonly found among younger age groups, affecting females more as compared to males. Less than half of the patients had recurrence of disease in our study and intra-operative and post-operative complications are common surgical outcomes and our study results are compatible with previously reported literature.Keywords: Aggressive fibromatosis; Desmoid fibromatosis; adult patients; extra-abdominal

Author Biographies

Masood Umer, Aga khan university hospital

Associate professor in dept. of Surgery

Javeria Saeed, Aga khan university hospital

research associate in dept .of surgery

Irfan Anwer, Aga khan university hospital

Senior medical officer  in dept. of surgery

Nida Zahid, Aga khan university hospital

Senior instructor research in dept ofsurgery

Younus Durrani, Jinnah post graduate medical college

medical officer in dept of surgery

References

Otero S, Moskovic E, Strauss D, Benson C, Miah A, Thway K, et al. Desmoid-type fibromatosis. Clin Radiol 2015;70(9):1038–45.

Eastley N, McCulloch T, Esler C, Hennig I, Fairbairn J, Gronchi A, et al. Extra-abdominal desmoid fibromatosis: a review of management, current guidance and unanswered questions. Eur J Surg Oncol 2016;42(7):1071–83.

Eastley NC, Hennig IM, Esler CP, Ashford RU. Nationwide trends in the current management of desmoid (aggressive) fibromatosis. Clin Oncol (R Coll Radiol) 2015;27(6):362–8.

Kasper B, Baumgarten C, Bonvalot S, Haas R, Haller F, Hohenberger P, et al. Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients’ and professionals’ expertise–a sarcoma patients EuroNet and European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group initiative. Eur J Cancer 2015;51(2):127–36.

Cates JM, Stricker TP. Surgical resection margins in desmoid-type fibromatosis: a critical reassessment. Am J Surg Pathol 2014;38(12):1707–14.

Sri-Ram K, Haddo O, Dannawi Z, Tirabosco R, Cannon S, Briggs T, et al. The outcome of extra-abdominal fibromatosis treated at a tertiary referral centre. Eur J Surg Oncol 2012;38(8):700–5.

Walker EA, Petscavage JM, Brian PL, Logie CI, Montini KM, Murphey MD. Imaging features of superficial and deep fibromatoses in the adult population. Sarcoma 2012;2012:215810.

Bonvalot S, Ternès N, Fiore M, Bitsakou G, Colombo C, Honoré C, et al. Spontaneous regression of primary abdominal wall desmoid tumors: more common than previously thought. Ann Surg Oncol 2013;20(13):4096–102.

Murphey MD, Ruble CM, Tyszko SM, Zbojniewicz AM, Potter BK, Miettinen M. From the archives of the AFIP: Musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics 2009;29(7):2143–83.

Kransdorf MJ, Murphey MD. Imaging of soft tissue tumors: Lippincott williams & wilkins; 2006.

Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. 5TH ed. Elsevier; Philadelphia, USA: Perivascular tumors, 2008; p.751–67.

Abbas AE, Deschamps C, Cassivi SD, Nichols FC, Allen MS, Schleck CD, et al. Chest wall desmoid tumors: results of surgical intervention. Ann Thorac Surg 2004;78(4):1219–23.

Mankin HJ, Hornicek FJ, Springfield DS. Extra‐abdominal desmoid tumors: a report of 234 cases. J Surg Oncol 2010;102(5):380–4.

Gronchi A, Casali PG, Mariani L, Lo Vullo S, Colecchia M, Lozza L, et al. Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. J Clin Oncol 2003;21(7):1390–7.

Published

2019-11-30