ABSTRACT
Desmoid tumors are rare slow growing, monoclonal, fibroblastic proliferation characterized by variable and often unpredictable clinical outcome, sometimes with aggressive infiltration of adjacent tissue but with no metastatic potential. Desmoids are histologically benign. On a molecular study level, they have been characterized by mutations in the β-catenin gene, APC (Adenomatous Polyposis Coli) gene or the CTNNB1. Gene studies are useful when the pathological diagnosis is difficult. Application of various multidisciplinary assessments along with multimodality treatment forms the basis of management for these patients. In selected asymptomatic patients, watchful waiting might be the most appropriate management. But for patients with desmoid located in other sites like the mesentery or at the head and neck region which might present with serious complications, thus requiring a more aggressive approach of treatment. In this article, we reported a 28-year-old female with a desmoid tumor of the anterior abdominal wall who underwent surgical resection. Preoperative evaluation was done using abdominal ultrasound, computed tomography, and magnetic resonance imaging. The histopathological examination of the resected specimen revealed a desmoid tumor. Because of the heterogeneity of the desmoid tumors, treatment needs to be individualized. However, complete surgical resection with negative margins seems to be the treatment of choice for this tumor entity and postoperative radiotherapy can help reduces the local recurrence rate.
ABSTRACT
INTRODUCCIÓN: la fibromatosis abarca un amplio espectro de lesiones fibrosas proliferativas con apariencia microscópica similar, que afectan a diferentes localizaciones anatómicas. Se agrupan dentro de los tumores fibrosos benignos en niños, y poseen un potencial intermedio entre las lesiones benignas y malignas. OBJETIVO: describir las características clínicas y el tratamiento de los pacientes con diagnóstico de fibromatosis agresiva tratados en el servicio de Oncopediatría en el Instituto Nacional de Oncología y Radiobiología. MÉTODOS: se realizó un estudio descriptivo, longitudinal y retrospectivo desde el 1º de enero de 2003 al 31 de diciembre de 2013, según variables demográficas, clínicas y terapéuticas. Se identificaron los pacientes a partir de las bases de datos del registro hospitalario del Instituto Nacional de Oncología y Radiobiología. Se seleccionaron todos los pacientes con diagnóstico histológico de esta enfermedad. RESULTADOS: se identificaron 9 pacientes con predominio del sexo masculino (56 %), con un rango de edades entre 0 y 9 años; y la localización más frecuente fue cabeza y cuello. Las modalidades de tratamiento utilizadas fueron: cirugía en 100 % de los casos, y quimioterapia y radioterapia concurrente (33 %). En estos momentos se cuenta con 100 % de supervivencia. CONCLUSIONES: la fibromatosis agresiva son lesiones benignas muy raras, agresivas localmente y sin potencial metastásico. Su tratamiento fundamental es la cirugía, sin embargo, deben incluirse otras modalidades terapéuticas para lograr el control local de la enfermedad.
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.
Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Child , Pediatrics , Oncology Service, Hospital , Fibromatosis, Aggressive , Fibromatosis, Aggressive/surgery , Fibromatosis, Aggressive/radiotherapy , Epidemiology, Descriptive , Retrospective Studies , Longitudinal StudiesABSTRACT
La fibromatosis agresiva es una lesión benigna, que comprende el 0,3% de todos los tumores sólidos. A pesar que la pared torácica es un lugar común, este tipo de tumor raramente se ha asociado con los implantes o tejido mamarios. Pocos casos se han descrito en conjunción con un implante de seno. Se presenta el caso de una paciente femenina de 27 años, quien le aparece esta patología dos años después de la colocación de implante mamario
The aggressive fibromatoses is a benign lesion, is not frequent, represent the 0.3% of the all solid tumors. The chest wall was considered a common place for tumors, but this type is rarely associated with the mammary implants. There are few cases reported in conjunction with a mammary implant. We presented a rare clinical case of a feminine patient of 27 years old, to whom apparition of this pathology two years after the collocation of mammary implant