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ISSN 1561-3119 (Electronic)

2015, Number 4

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Rev Cubana Pediatr 2015; 87 (4)

Aggresive fibromatosis in childhood at the oncologic pediatric service

Pérez TM, Forteza SM, Renó CJS, García SD, Quintero VD, Curbelo HI

Language: Spanish
References: 19
Page: 468-476
PDF size: 172.44 Kb.


ABSTRACT

Introduction: fibromatosis covers a wide spectrum of proliferative fiber lesions with similar microscopic appearance that affect various anatomical locations. These lesions are grouped into the benign fiber tumors in children and have an intermediate potential between the benign and the malignant lesions.
Objective: to describe the clinical characteristics of and the treatment prescribed for patients with diagnosis of aggressive fibromatosis, who were treated at the oncologic pediatrics service of the National Institute of Oncology and Radiobiology.
Methods: retrospective, longitudinal and descriptive study conducted from January 1st, 2003 through December 31st 2013 based on demographic, clinical and therapeutic variables. The patients were identified according to databases from the hospital register of the National Institute of Oncology and Radiobiology. All the patients with histological diagnosis for the disease participated in the study.
Results: nine patients were detected with predominance of males (56 %), age ranging from 0 to 9 years and the most common location were head and neck. The treatment modalities included surgery in 100 % of cases and concurrent chemotherapy and radiotherapy (33 %). Currently, the survival rate is 100 %.
Conclusions: aggressive fibromatosis are benign lesions that are very unusual, locally aggressive and with no metastatic potential. The main treatment is surgery; but other therapeutic variants should be included to achieve the local management of disease.


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