Aggressive fibromatosis in pediatric population—A case series

Masood Umer,Javeria Saeed,Nida Zahid

doi:10.1016/j.ijscr.2018.09.032

Masood Umer, Javeria Saeed + Show 1 more

Open Access

https://doi.org/10.1016/j.ijscr.2018.09.032

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Abstract

IntroductionAggressive fibromatosis or Desmoid fibromatosis is a benign tumor which originated form mesenchymal tissues of the body. This tumor has strong potential of recurrence and infiltration but it does not metastasize to other organs of the body. This case series is focused to determine the treatment outcomes for pediatric patients of aggressive fibromatosis. MethodologyIt is a retrospective case series conducted on 7 patients presented to section of Orthopedics, department of surgery of our institute in Karachi. We included all the cases of pediatric patients from 1 to 16 years, with biopsy proven fibromatosis from January 2000 to December 2015. Presentation of CasesOut of 7 pediatric patients, there were 6 (85.7%) males and 1 (14.3%) female patient. The median age was 6 years IQR (5–11) years. Gluteal region was the most common site of disease. Four patients (57.1%) had positive tumor margins while three (42.9%) had negative margins. Out of 7 patients, 4 patients (57.1%) had recurrent disease and they had positive margins. The median Disease Free survival time was 14 months and there was no expiry of patients. Conclusion & recommendationThe conclusion of our study was that aggressive fibromatosis is more prevalent in children below 15 years of age and disease burden is higher in male gender. Positive margins after surgery indicate a high risk for disease recurrence therefore; primary surgery with negative margins is the treatment of choice for children with AF. However, we recommend that multicenter trials should be conducted in the future to clarify the role of adjuvant treatment for patients with pediatric AF.

Highlights

Aggressive fibromatosis or Desmoid fibromatosis is a benign tumor which originated form mesenchymal tissues of the body
World health organization (WHO) defines fibromatosis as a tumor that originates from mesenchymal tissues which is non-metastatic but presented as locally belligerent lesion which accounts for 0.03% of neoplasms and 3% of every type of soft tissue lesion
The median age of the participants of study was 6 years which was consistent with the findings from other studies that indicates that fibromatosis can affect any age group but it is more evident among children

Summary

Introduction

Aggressive fibromatosis or Desmoid fibromatosis is a benign tumor which originated form mesenchymal tissues of the body. This tumor has strong potential of recurrence and infiltration but it does not metastasize to other organs of the body This case series is focused to determine the treatment outcomes for pediatric patients of aggressive fibromatosis. World health organization (WHO) defines fibromatosis as a tumor that originates from mesenchymal tissues which is non-metastatic but presented as locally belligerent lesion which accounts for 0.03% of neoplasms and 3% of every type of soft tissue lesion. It is nonmetastasizing tumor but it has high frequency of recurrence [1]. AF has a very high predisposition for local recurrence [2,3]

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