Fibromatosis agresiva en la infancia en el servicio de oncopediatría / Aggresive fibromatosis in childhood at the oncologic pediatric service

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.

Fibromatosis agresiva de la pared torácica asociada a implante mamario: a propósito de un caso / Aggressive fibromatosis of the chest wall associated to mammary implant: a case report

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

Tumor desmóide de parede torácica recidivado / Recurrent desmoid tumor of the chest wall

Tumor desmóide (TD) ou fibromatose agressiva é um tipo raro de neoplasia de partes moles que não causa metástases, mas apresenta alto poder de infiltração por estruturas músculo-aponeuróticas adjacentes. O tratamento é agressivo, consistindo principalmente em ressecção cirúrgica com margens livres, também contando com a radioterapia, utilização de antiinflamatório não esteroidal (AINE´s), agentes hormonais e quimioterapia. A incidência de recidiva local após tratamento primário é alta. O presente trabalho relata o caso de um paciente que apresentou tumor desmóide recidivado em dorso, sendo submetido à cirurgia, que consistiu em toracotomia, apofisectomia, osteotomia, biópsia de parênquima pulmonar de lobo inferior esquerdo, drenagem fechada de pleura, reconstrução imediata com rotação de retalhos musculares e colocação de tela. Após procedimento cirúrgico o paciente foi submetido a 23 ciclos de radioterapia associados a doses diárias de Tamoxifeno (40mg/dia). Trinta meses após cirurgia, o paciente encontra-se livre de doença, assintomático e em acompanhamento.

Desmoid tumor (DT) or aggressive fibromatosis is a rare type of soft tissue neoplastic disorder that does not metastasize, but is highly infiltrative on the adjacent muscle and aponeurosis. Treatment must be aggressive, and consists mainly of surgical resection with free margins, although radiotherapy, non-steroidal anti-inflammatory drugs, hormones and chemotherapy may be used. Local relapse rate after primary treatment is high. We report a patient who had a relapse of desmoid tumor on the dorsum, and who underwent thoracotomy, apophysectomy, osteotomy, biopsy of the left lower lobe of the lung, closed pleural space drainage, immediate reconstruction with rotation of muscle flaps and mesh repair. After surgery, the patient received 23 cycles of radiotherapy associated with tamoxifen (40 mg/day). Thirty months after surgery, the patient shows no signs of the disease, no symptons at all and is currently being observed.

The role of radiotherapy in the treatment of aggressive fibromatosis

Aggressive fibromatosis is a rare benign soft tissue tumor that is difficult to cure because of its infiltrative nature and high tendency to recur locally. The authors retrospectively analyzed 20 patients with histologically-confirmed fibromatosis. All patients underwent surgery with a wide or marginal margin. Five (25%) cases with histologically-negative margins had recurred. External beam radiotherapy was administered to patients whose margins were positive or who had local recurrence. However, out of concern for safety, radiotherapy was not given to two babies and a reproductive-aged woman. The average dose was 5,020 cGy. During the follow-up (mean 32.6 months), all the patients undergoing radiotherapy showed no evidence of local recurrence. A wide local excision has traditionally been the treatment of choice. However, postoperative radiotherapy could be an effective measure for preventing local recurrence in patients with a histologically-positive surgical margin and recurrence independent of any signs of relapse.

Tumor desmóide / Desmoid tumor

Esse artigo relata a experiência de um caso de tumor desmóide, uma neoplasia rara, no Hospital da Baleia. O objetivo deste trabalho é expor sua definição mais freqüente e suas várias e complexas formas de tratamento (medicamentosa, radioterápica e cirúrgica) com suas indicações precisas.

This article reports a case experience of the dismoid tumor, a rare tumor, in the Hospital da Baleia. The objetive of this paper is explain its definition and clinical findings, tumor's localization and many forms of treatment (drugs, radioterapy and surgery) with correct indication.