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.

Fibromatose agressiva (tumor desmoide) associada ao implante mamário: revisão da literatura e apresentação de três novos casos / Aggressive fibromatosis (desmoid tumor) associated with breast implant: literature review and presentation of three new cases

INTRODUÇÃO: As fibromatoses consistem na proliferação de tecido fibroso, na forma de faixas ou nódulos, associadas às fáscias e aponeuroses. Sua variedade agressiva, denominada previamente tumor desmoide, se comporta de forma semelhante a uma neoplasia maligna, com destruição local de tecidos, sem, entretanto, originar metástases. MÉTODOS: Fez-se revisão da literatura de1979 a janeiro de 2017. Dados de três pacientes, operados entre maio de 2010 e agosto de 2015, foram revistos, com proservação até março de 2017. Observaram-se idade, características do implante, via de introdução do mesmo, tempo decorrido entre o implante e o aparecimento da fibromatose, tratamento cirúrgico instituído, acompanhamento clínico e resultados. RESULTADOS: Foram encontrados 24 trabalhos na literatura disponível, versando sobre fibromatose agressiva da parede torácica associada ao implante mamário de silicone, nos quais foram relatados 34 casos. São apresentados três casos de fibromatose agressiva associados ao implante mamário de silicone. Os casos foram tratados com sucesso por ressecção alargada da parede torácica, incluindo pele, musculatura, costelas, fáscia endotorácica e pleura parietal. A reconstrução foi bem-sucedida, realizada com tela aloplástica (Prolene & reg;) recoberta por retalho muscular em dois casos e retalho cutâneo local em um caso. CONCLUSÃO: A associação de fibromatose agressiva e implante mamário é rara. O tratamento deve consistir em cirurgia alargada, removendo-se o implante e toda a área da cápsula adjacente, em conjunto com parte da mama, costelas subjacentes, musculatura intercostal, fáscia endotorácica e pleura parietal. A reconstrução deve ser feita com tela aloplástica associada a retalho muscular ou retalho tegumentar local.

INTRODUCTION: Fibromatoses consists of the proliferation of fibrous tissue, in the form of bands or nodules, associated with fasciae and aponeuroses. The aggressive variety, previously denominated desmoid tumor, behaves similarly to malignant neoplasm, with local destruction of tissues, without, however, producing metastases. METHODS: A literature review was carried out from 1979 to January 2017. Data from three patients, operated between May 2010 and August 2015, were reviewed. Age, implant characteristics, route of introduction, time elapsed between implantation and fibromatosis, surgical treatment, clinical follow-up and results were observed. RESULTS: Twenty-four papers were found in the literature reporting aggressive fibromatosis of the chest wall associated with silicone breast implant. In these studies, 34 cases were reported. Three new cases of aggressive fibromatosis associated with silicone breast implant are now presented. These cases were successfully treated by extensive resection of the chest wall, including skin, musculature, ribs, endothoracic fascia, and parietal pleura. The reconstruction was successful, performed with alloplastic mesh (Prolene & reg;) covered by muscular flap in two cases and local skin flap in one case. CONCLUSION: The association of aggressive fibromatosis and breast implant is rare. The treatment should consist of extensive surgery, removing the breast implant and the entire area of the capsule around it, part of breast, together with the underlying ribs, intercostal muscles, endothoracic fascia and parietal pleura. The reconstruction should be made with an alloplastic mesh, covered by muscular flaps or local skin flap.

Fibromatosis Desmoide en Pediatría. Presentación de un caso clínico / DESMOID FIBROMATOSIS IN PEDIATRICS ; CASE REPORT AND REVISION

Se conoce como fibromatosis desmoide a la proliferación fibroblástica clonal que surge de partes blandas y se caracteriza por un crecimiento infiltrativo, localmente invasivo y con tendencia a la recurrencia local, pero con baja capacidad de metastatizar. Es un patología infrecuente en la edad padiatrica, sobre todo en la localización de cabeza y cuello. su tratamiento esta en discusión entre la cirugía radical, la conducta expectante y la radio o quimioterapia. Se presenta el caso clínico de un varón de 4 años de edad con diagnostico de fibromatosis desmoide laterocervical de cuello, adyacente a paquete vascular yugulocarotideo, en el cual se practico con éxito la resección total.

Desmoid fibromatosis is a fibroblastic monoclonal proliferation of soft tissues, and is characterized by an infiltrative growth, locally invasive and with tendency to local recurrence, but with a low metastatic capacity . It is an uncommon condition in pediatric patients, especially in head and neck locations. The treatment is under discussion between radical surgery, expectant management and radio or chemotherapy. We report the clinical case of a 4 years old male with a diagnosis of laterocervical desmoid fibromatosis of the neck, adjacent to a vascular jugulocarotide package, in which the total resection was successfully performed.

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.

Tumor desmoide extraabdominal: descripción de un caso y revisión de la bibliografía / Extraabdominal desmoid tumor: case description and review of the literature

Objetivo: presentar un caso clínico de tumor desmoide de maxilar inferior en un niño de 4 años, analizar las características histológicas y el comportamiento clínico e informar el diagnóstico y tratamiento. Caso clínico: un varón de 4 años de edad, sin antecedentes patológicos, fue atendido por presentar tumoración en región mandibular izquierda de 7 meses de evolución. Se realizaron ecografía, tomografía axial computarizada y resonancia magnética, las cuales describieron masa ocupante de espacio sólida, de límites escasamente definidos con resorción perióstica. La biopsia informó neurofibroma submandibular. Se efectuó la extirpación quirúrgica que confirmó el diagnóstico de tumor desmoide extraabdominal. Conclusiones: el tumor desmoide es de histología benigna pero infiltrante, con una tasa de recurrencia alta, por lo que el tratamiento indicado es la resección quirúrgica amplia. La radioterapia puede controlar las lesiones irresecables.

Reinserção psicossocial de paciente jovem através de desarticulação interescápulo torácica e hemitoracectomia / Psychosocial rehabilitation of a young patient through interscapular thoracic disarticulation and hemithoracectomy

Na era das cirurgias minimamente invasivas, parece controverso advogar grandes operações. Entretanto, ainda há espaço para grandes intervenções oncológicas. É relatado caso de paciente de 23 anos de idade portador de tumor desmoide em hemitórax direito, com deformidade e erosão de arcos costais, atelectasia parcial do pulmão ipsilateral, compressão e desvio contralateral do mediastino, invasão de nervos do plexo braquial, vasos subclávios e pleura apical direita. Realizada toracectomia, ressecção de tumor em mediastino, amputação do membro superior direito, escapulectomia e reconstrução da parede torácica com tela de márlex e metilmetacrilato e confecção de retalho fasciocutâneo. O paciente encontra-se em seguimento ambulatorial com suas funções diárias quase recuperadas, limitado apenas nas atividades que envolviam o membro desarticulado. Em casos selecionados devem ser consideras grandes intervenções com o objetivo de ressecção R0 (ressecção com margens cirúrgicas livres) e melhora na qualidade de vida, atentando-se sempre para a ressocialização.

In the era of minimally invasive surgery, advocating large operations seems controversial. However, there is still room for major oncologic interventions. This is report of a 23 yearold patient with desmoid tumor in the right hemithorax, deformed and eroded ribs, ipsilateral lung partial atelectasis, contralateral mediastinal compression and shift, invasion ofnerves in the brachial plexus, subclavian vessels and right apical pleura. Thoracectomy was performed with resection of the tumor in the mediastinum, right upper limb amputation, scapulectomy, and chest wall reconstruction with methylmethacrylate and marlex screen, and use of a fasciocutaneous flap. The subject is in follow up as an outpatient andhas almost recovered his daily functions, which are now limited only for activities involving the inarticulate member. Selected cases should be considered for major interventions with aiming at R0 resection (resection with free surgical margins) and improved quality of life, always bearing in mind the need for rehabilitation and socialization.

Giant desmoid tumor of the abdominal wall in a patient with Gardner Syndrome

Gardner syndrome (GS) is a rare entity characterized by a triad of familial colonic polyposis, multiple osteomas and soft tissue tumors, including desmoid tumor (DT). This is a case report of a 30 year-old patient with GS who developed giant DT in the abdominal wall after undergoing several laparotomies. The patient has taken a long time to search for medical care, and at first he saw another team that refused to operate him by judging the lesion unresectable. The surgery in our department was performed in three steps. Initially, we resected the lesion with macroscopic margins, and as there were small bowel adhesions in the tumor, we performed enterectomy and closed using the "Bogotá" technique, with skin closure on the bag. On the fourth postoperative day (POD), we reoperated the abdomen without identifying any signs of fistula. On the seventh POD there was another surgical intervention, this time to insert a double-sided mesh. The patient recovered well, and had no debilitating motor deficit, despite the extensive resection of the abdominal muscles. Curative treatment of DT is based on surgical resection and only sequential surveillance allows us an early diagnosis, when the lesion is still resectable. (AU)

Tumor desmoide gigante de parede abdominal em paciente portador da Síndrome de Gardner. A Síndrome de Gardner (SG) é uma entidade rara caracterizada pela tríade polipose colônica familial, múltiplos osteomas e tumores de tecidos moles, dentre eles o tumor desmoide (TD). Tratou-se de um relato de caso de um paciente de 30 anos, com SG que evoluiu com TD gigante em parede abdominal, após ser submetido a diversas laparotomias prévias. O paciente levou longo tempo para procurar o serviço de cirurgia, passando por outra equipe que se negou a abordá-lo por julgar a lesão irressecável. A cirurgia no nosso serviço se deu em três tempos. Inicialmente, foi feita a ressecção da lesão com margens macroscópicas e, por haver aderências de alças no tumor, realizamos enterectomia e fechamos a Bogotá com síntese da pele sobre a bolsa. No quarto dia pós-operatório (DPO), reabordamos o abdômen sem identificar sinal de fístula. No sétimo DPO houve nova abordagem, agora para colocar tela dupla face. O paciente evoluiu bem, sem déficit motor debilitante, apesar da extensa área de ressecção muscular abdominal. O tratamento curativo dos TD é baseado na sua ressecção cirúrgica e somente a vigilância sequencial nos permite seu diagnóstico precoce e a abordagem enquanto a lesão é ressecável. (AU)

Management of desmoid-type fibromatosis involving peripheral nerves / Tratamento da fibromatose tipo desmoide envolvendo nervos periféricos

Desmoid-type fibromatosis is an uncommon and aggressive neoplasia, associated with a high rate of recurrence. It is characterized by an infiltrative but benign fibroblastic proliferation occurring within the deep soft tissues. There is no consensus about the treatment of those tumors. We present a surgical series of four cases, involving the brachial plexus (two cases), the median nerve and the medial brachial cutaneous nerve. Except for the last case, they were submitted to multiple surgical procedures and showed repeated recurrences. The diagnosis, the different ways of treatment and the prognosis of these tumoral lesions are discussed. Our results support the indication of radical surgery followed by radiotherapy as probably one of the best ways to treat those controversial lesions.

A fibromatose do tipo desmoide é uma lesão tumoral agressiva e rara, associada a alto índice de recorrência. É caracterizada pela fibroblástica infiltrativa, porém benigna, que ocorre no interior de tecidos moles profundos. Não existe consenso com relação ao tratamento desses tumores. Apresentamos uma série cirúrgica de quatro casos comprometendo o plexo braquial (dois casos), o nervo mediano e o nervo cutâneo medial do braço. Com exceção do último caso, todos foram submetidos a múltiplos procedimentos cirúrgicos e apresentaram recorrências repetidas. São discutidos o diagnóstico, as diferentes formas de tratamento e o prognóstico dessas lesões tumorais. Nossos resultados apoiam o conceito de que cirurgia radical seguida por radioterapia é uma das melhores formas de se tratar essas controvertidas lesões.

[Radiation therapy in extra abdominal desmoid tumours]

Desmoid tumours are rare benign tumours that have local 'malignity'. They can be locally aggressive by infiltrating adjacent vascular, nervous and bony structures. To define the role of radiotherapy in the management of desmoid tumours. Retrospective study of six extra-abdominal desmoid tumours treated with surgery and radiation therapy. Median age was 26.8 years [16-35 years]. The tumor was located in the arm [1 case], in the legs [2 cases] and in the body [3 cases]. Median tumoral sizes were 86.6 mm [60-100 min]. Five patients had local recurrence after surgical resection. Radiation therapy included Cobalt 60 in five cases and photons associated to linear acceleration in one case. Total dose was between 54 and 62 Gy. At the end of radiation therapy, tumoral control was obtained in five cases after a median follow-up of 26 months [1-83 months]. Surgery is the main treatment of desmoids tumours but local recurrence rates remain high even after complete resection. The role of adjuvant radiation therapy is controversial

Desmoid tumor in patients with familial adenomatous polyposis / Tumor desmóide em pacientes com polipose adenomatosa familiar

CONTEXT: Desmoid tumors constitute one of the most important extraintestinal manifestations of familial adenomatous polyposis. The development of desmoids is responsible for increasing morbidity and mortality rates in cases of familial adenomatous polyposis. OBJECTIVES: To evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to present patient outcome. METHODS: Between 1984 and 2008, 68 patients underwent colectomy for familial adenomatous polyposis at the School of Medical Sciences Teaching Hospital, University of Campinas, SP, Brazil. Desmoid tumors were found in nine (13.2 percent) of these patients, who were studied retrospectively by consulting their medical charts with respect to clinical and surgical data. RESULTS: Of nine patients, seven (77.8 percent) were submitted to laparotomy for tumor resection. Median age at the time of surgery was 33.9 years (range 22-51 years). Desmoid tumors were found in the abdominal wall in 3/9 cases (33.3 percent) and in an intra-abdominal site in the remaining six cases (66.7 percent). Median time elapsed between ileal pouch-anal anastomosis and diagnosis of desmoid tumor was 37.5 months (range 14-60 months), while the median time between colectomy with ileorectal anastomosis and diagnosis was 63.7 months (range 25-116 months). In 6/9 (66.7 percent) patients with desmoid tumors, the disease was either under control or there was no evidence of tumor recurrence at a follow-up visit made a mean of 63.1 months later (range 12-240 months). CONCLUSIONS: Desmoid tumors were found in 13.2 percent of cases of familial adenomatous polyposis following colectomy; therefore, familial adenomatous polyposis patients should be followed-up and surveillance should include abdominal examination to detect signs and symptoms. Treatment options include surgery and clinical management with antiestrogens, antiinflammatory drugs or chemotherapy.

CONTEXTO: Os tumores desmóides representam uma das manifestações extraintestinais mais importantes na síndrome da polipose adenomatosa familiar. O aparecimento desta neoplasia está relacionada ao aumento da morbimortalidade nos doentes com polipose adenomatosa familiar. OBJETIVOS: Avaliar a ocorrência dos tumores desmóides nos casos de polipose adenomatosa familiar submetidos a colectomia profilática e avaliar o seguimento em ambulatório. MÉTODOS: Entre 1984 e 2008, 68 pacientes foram submetidos a colectomia por polipose adenomatosa familiar no Hospital das Clínicas da Faculdade de Ciências Médicas da Universidade de Campinas, SP. Os tumores desmóides ocorreram em nove pacientes (13.2 por cento), que foram estudados retrospectivamente, por meio da análise de prontuários, buscando dados clínicos e cirúrgicos. RESULTADOS: Dos nove pacientes, sete (77,8 por cento) foram submetidos a laparotomia para ressecção do tumor. A média de idade no momento da cirurgia foi de 33,9 anos (variando 22-51 anos). Os tumores desmóides da parede abdominal ocorreram em 3/9 casos (33.3 por cento) e os intra-abdominais em seis casos (66,7 por cento). O tempo médio entre a cirurgia do reservatório ileal e o diagnóstico do tumor desmóide foi de 37,5 meses (variando 14-60 meses), enquanto o tempo médio entre a cirurgia de colectomia com anastomose íleorretal e o diagnóstico foi de 63,7 meses (variando 25-116 meses). Em 6/9 (66,7 por cento) pacientes com tumor desmóide, a doença estava controlada ou não havia evidência de recidiva do tumor em 63,1 meses (variando de 12 a 240 meses) de tempo médio de seguimento. CONCLUSÃO: Os tumores desmóides ocorreram em 13,2 por cento dos casos de polipose adenomatosa familiar após a cirurgia do cólon; desta maneira, os pacientes com polipose adenomatosa familiar devem manter seguimento em ambulatório e o rastreamento deve incluir o exame abdominal minucioso a fim de identificar sinais e sintomas que possam conduzir ao diagnóstico de tumor desmóide. As opções de tratamento incluem cirurgia e manejo clínico com antiestrogênios, anti-inflamatórios ou quimioterapia.

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.

Tumor desmoide de la pared torácica: presentación de un caso y revisión de la literatura / Desmoid tumor of the chest wall: case report and literature review

Los tumores desmoides originados en la pared torácica son infrecuentes, en nuestro medio y a nivel mundial. Aquellos de dimensiones gigantes son aún más raros, requieren abordajes quirúrgicos adecuados, siendo la única alternativa de tratamiento, razón por la cual presentamos el presente caso.

Desmoid tumors originating in the chest wall are infrequent in our institution as in the rest of the world. Those of huge dimentios are even more unusual and require an adequate surgical approach, the only choice of treatment, reason for presenting this clinical case

Tumor desmoide: reporte de un caso / Desmoide tumor: case report

El tumor desmoide es una neoplasia rara de tejidos blandos que se desarrolla a partir de músculo, tejido conectivo, fascia y aponeurois. Se presenta esporádicamente y más frecuentemente en mujeres. El caso que se presenta concierne a paciente femenina de 27 años que fue sometida a escisión radical de una gran masa que infiltraba músculo recto abdominal, diagnosticada como tumor desmoide. Aunque tiene características benignas, es de naturaleza infiltrativa y se comporta como una masa localmente agresiva, la cual puede invadir estructuras adyacentes haciendo que la resección quirúrgica sea difícil. El único tratamiento viable es la cirugía amplia dejando bordes sanos, esto causa gran defecto de pared y por tanto serios problemas en la reconstrucción. Además, la tasa de recurrencias locales varía y depende de la edad del paciente, localización y los márgenes de resección.

Desmoid tumor is quite rare soft tissues neoplasm that develops from muscle connective tissue, fasciae and aponeuroses. This neoplasm occurs in sporadic and more frequent in women than men. The presented case report refers to young female (27 years old), who underwent the radical excision of a large desmoid tumour infiltrating the right rectus muscle of the abdomen. Although desmoid is classified pathologically as a benign tumour, its infiltrative nature leads to a locally aggressive mass, which can invade surrounding structures and organs making surgical resection difficult. The only radical treatment for her was the surgical resection carried out far from the tumour borders into the healthy tissues. This resection causes wide muscle-fascial defects determining serious reconstructive problems. Futhermore, overall local recurrence rates vary and depend on patient’s age, tumour location and margins at resection.

[Abdominal desmoid tumor pathologic and therapeutic concepts]

Abdominal desmoid tumors arise in young adults between 20 and 40 years. They are character/i2ed by a local recurrence in 20 to 30% of eases. Review the clinical and pathologic features of this entity, look for the expression of immunohistochemical markers [actine, desmine, CD34, vimentin] and also to estimate the expression of two potential targets therapy; the hormonal receptors and the CD117. From 1990 to 2004, three cases of abdominal desmoid tumors were diagnosed in the Pathologic laboratory of the Charles Nicole Hospital of Tunis. The study concerned three women 62, 35 and 30 year-old. All the patients were operated and the site of tumours varied to 5 to 7cm. The CD117 and the hormonal receptors were negative in every case. None of the patients presented local recurrence. Abdominal desmoid tumors are locally aggressive but never give metastasis. The negativity of these lesions to the CD117 and in the hormonal receptors does not imply resistance in the hormonal treatment by anti-estrogens