Author/Authors :
Pablo Zumárraga, Juan Orthopedic Oncology Group - Instituto de Ortopedia e Traumatologia - Hospital das Clinicas HCFMUSP - Faculdade de Medicina - Universidade de São Paulo, São Paulo, SP, Brazil , guilherme monteiro marta Coimbra, brian Instituto de Ortopedia - Hospital das Clinicas HCFMUSP- Faculdade de Medicina - Universidade de São Paulo, São Paulo, SP, Brazil , gonçalves dos santos, FeliPe Orthopedic Oncology Group - Instituto de Ortopedia e Traumatologia - Hospital das Clinicas HCFMUSP - Faculdade de Medicina - Universidade de São Paulo, São Paulo, SP, Brazil , mathias baPtista, andré Orthopedic Oncology Group - Instituto de Ortopedia e Traumatologia - Hospital das Clinicas HCFMUSP - Faculdade de Medicina - Universidade de São Paulo, São Paulo, SP, Brazil , tomio Kohara, marCelo Orthopedic Oncology Group - Instituto de Ortopedia e Traumatologia - Hospital das Clinicas HCFMUSP - Faculdade de Medicina - Universidade de São Paulo, São Paulo, SP, Brazil , Pires de Camargo, olavo Orthopedic Oncology Group - Instituto de Ortopedia e Traumatologia - Hospital das Clinicas HCFMUSP - Faculdade de Medicina - Universidade de São Paulo, São Paulo, SP, Brazil
Abstract :
Objective: Extra-abdominal desmoid tumor (EDT) is a rare con-
dition, caused by proliferation of fibroblasts. Despite being a
benign tumor, it is locally aggressive and has unpredictable clinical
behavior. The objective of this study is to present the clinical
outcomes of patients with EDT treated surgically between 1995
and 2016. Methods: This is a retrospective series of 23 patients
with histopathological diagnosis of EDT that underwent surgery at
the orthopedic oncology service of our hospital. The information
was obtained from the institute’s clinical and pathology reports.
Results: A total of 223 medical records with histopathological
reports were evaluated. Only 23 cases of EDT were included in
the present study. The mean age was 22.5 years. Twelve (52.2%)
patients had the tumor on the lower limbs, seven (30.4%) on the
upper limbs and four (17.4%) cases were reported on the back.
Five (21.7%) patients had tumors measuring less than 5 cm, while
eighteen (78.3%) patients had tumors measuring more than 5
cm. All patients underwent surgery as the definitive treatment in
our institute. Twelve (52.2%) cases presented negative margins
(NM) and eleven (47.8%) cases had positive margins (PM). Local
recurrence (LR) occurred in eleven (47.8%) patients. Conclusion:
Impairment of the surgical margin was the only prognostic factor
found for LR of EDT. Level of Evidence IV, Case Series.
Keywords :
Fibromatosis , aggressive , Surgical oncology , Local neoplasm recurrence , Margins of excision , Prognosis