Desmoid-type fibromatosis in children. Clinical features, treatment response, and long-term follow-up / Fibromatosis tipo desmoide en niños. Características, respuesta al tratamiento y seguimiento a largo plazo

Abstract Desmoid-type fibromatosis (DF) is a tumor with high local recurrence rate. Sixteen patients (18 desmoid tumors) were retrospectively evaluated. Initial surgery was performed in 13/18 tumors, with complete resection in 6 (one with free margin and five with microscopic residual disease); 10/13 had local relapse. Eleven patients with 13 tumors underwent treatment with methotrexate-vinblastine. The response rate to chemotherapy was 54%, and up to 81% if stable disease cases were included. The best response was partial remission. Only 2 had grade 4 toxicity. Twelve of 15 patients had sequelae. In 8 cases sequelae were directly related to the surgical intervention and 3 of them were severe. The 5-year progression-free survival and overall survival were 30% and 93.3%, respectively. DF has a high local relapse rate, regardless of surgical margin involvement. Low dose chemotherapy achieved stable disease and even remission of the lesions with low toxicity. The high rate of sequelae is probably related to the initial surgery performed in the majority of patients and may be avoided by the use of neoadjuvant low dose chemotherapy.

Resumen La fibromatosis tipo desmoide (FD) es un tumor con alta tasa de recurrencia local. Dieciséis pacientes (18 tumores desmoides) fueron evaluados retrospectivamente. La cirugía inicial se realizó en 13/18 tumores, con resección completa en 6 (uno con margen libre y cinco con margen microscópicamente comprometido); 10/13 tuvieron recaída local. Once pacientes con 13 tumores recibieron tratamiento con metotrexato/vinblastina. La tasa de respuesta a la quimioterapia fue del 54% y de hasta el 81% si se incluyen los casos que lograron enfermedad estable. La mejor respuesta fue remisión parcial. Solo 2 tuvieron toxicidad grado 4. Doce de 15 pacientes tuvieron secuelas. En 8 casos, las secuelas estuvieron directamente relacionadas con la intervención quirúrgica y 3 de ellas fueron graves. La sobrevida libre de progresión a 5 años y la supervivencia global fueron del 30% y del 93.3%, respectivamente. La FD tiene una alta tasa de recaída local, independientemente del margen quirúrgico. Dosis bajas de quimioterapia lograron una enfermedad estable e incluso la remisión de las lesiones, con baja toxicidad. La alta tasa de secuelas probablemente esté relacionada con la cirugía inicial realizada en la mayoría de los pacientes y podría evitarse mediante el uso de quimioterapia neoadyuvante en dosis bajas, como sugieren las estrategias actuales de tratamiento.

The Analysis of Treatment of Aggressive Fibromatosis Using Oral Methotrexate Chemotherapy

BACKGROUND: Aggressive fibromatosis is a rare but invasive tumor infiltrating widely between fascia and muscle fibers. It has a high tendency to be locally recurrent despite complete resection. Effectiveness of adjuvant treatment for aggressive fibromatosis including radiotherapy, pharmacological agents, hormonal treatments, and chemotherapy have been previously reported. The purpose of this article was to collect and analyze all information regarding the effectiveness and side effects of oral methotrexate in aggressive fibromatosis. METHODS: From 2005 to 2011, eleven patients with aggressive fibromatosis treated with oral methotrexate at our institution were analyzed in this study. Oral methotrexate was administered once per week at 10 mg per week. Authors collected information about effectiveness concerning cases of local recurrence and metastasis. RESULTS: Eleven patients had remission, two patients had local recurrence. Fatal complications or toxicity were not observed. CONCLUSIONS: Oral methotrexate given at this dose and schedule was considered as a useful treatment in primary inoperable fibromatosis and recurrent fibromatosis.

Treatment of a Recurrent Chest Wall Desmoid Tumor Using a CT-Guided Steroid Injection

We report on a 41-year-old woman with a chest wall desmoid tumour who was successfully treated with a computed tomography (CT)-guided steroid injection. She presented with a palpable mass in the right upper chest wall and was treated by surgical excision and postoperative radiation therapy due to recurrence of the mass at the surgical site. At 20 months after the second operation, a recurrent mass was again detected in the anterosuperior portion of the previous surgical site on CT. We performed a CT-guided steroid injection weekly for 4 weeks by applying a mixture of 3 mL of triamcinolone acetonide (40 mg/mL) and 3 mL of 1% Lidocaine, administering 4-6 mL of the mixture, to the lesion. Six months later, CT showed a marked decrease in the size of the mass.

Tumor desmóide / Desmoid tumor

O tumor desmóide e uma neoplasia benigna que se origina de diferentes estruturas fasciais ou músculo- aponeuroticas, resultado de uma proliferação fibroblastica . O termo “desmóide” vem do grego desmos, e foi primeiramente utilizado em 1800 para descrever os tumores com consistência de tendão . Pode receber denominações diferentes como fibromatose agressiva , fibroma desmóide e fibromatose músculo-aponeurótica. Está presente em 4 a 13% dos pacientes com polipose adenomatosa familiar (PAF). Apesar de histologicamente benignos, os tumores desmóides tem comportamento maligno , sendo localmente invasivos e com elevada recorrência após a ressecção.

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.