Tratamiento quirúrgico de la fibromatosis agresiva en relación con cesárea previa / Surgical treatment of aggressive fibromatosis in relation to previous cesarean section

Introducción. Los tumores desmoides son lesiones de los tejidos blandos, histológicamente benignas, poco frecuentes y con gran agresividad local y carencia de potencial metastásico. Se relacionan estrechamente con antecedentes traumáticos o quirúrgicos, como la cesárea, y su tratamiento generalmente es quirúrgico. Métodos. Presentamos una serie de tres pacientes intervenidas en nuestro centro durante el año 2020. Se revisan sus antecedentes y se describe su tratamiento. Resultados. En todas nuestras pacientes se encontró algún antecedente quirúrgico, dos cesáreas y una resección de un disgerminoma. El tratamiento empleado fue la resección quirúrgica con márgenes libres y reparación del defecto mediante malla. Conclusiones. El tumor desmoide es una patología poco frecuente, su diagnóstico se realiza mediante exámenes imagenológicos y se confirma con el estudio histológico; es importante hacer el diagnóstico diferencial con el sarcoma. La cirugía radical sigue siendo el tratamiento de elección, aunque algunos autores proponen el tratamiento conservador.

Introduction. Desmoid tumors are soft tissue lesions, histologically benign, rare and with great local aggressiveness and lack of metastatic potential. They are closely related to traumatic or surgical history such as caesarean section. Their treatment is generally surgical. Methods. We present a case series of three patients operated on in our center during the year 2020. Their history is reviewed and their type of treatment is presented. Results. In all our patients, surgical history was found (two caesarean sections and one resection of a dysgerminoma). The treatment used was surgical resection with free margins and mesh repair of the defect. Conclusions. Desmoid tumor is a rare pathology; its diagnosis is made by imaging studies, and confirmed by histology. It is important to make a differential diagnosis with sarcoma. Radical surgery remains the treatment of choice, although some authors propose conservative treatment.

Tumor desmoide abdominal gigante en puerperio: caso clínico y revisión de la literatura / Giant abdominal desmoid tumor during the puerperium: Case report and review of literature

Introducción. Los tumores desmoides o fibromatosis agresiva corresponden a neoplasias mesenquimales poco frecuentes. Son tumores localmente agresivos que ocurren especialmente en jóvenes, no desarrollan metástasis a distancia, pero se asocian con invasión locorregional y alta tasa de recurrencia después de la resección. Su etiología es desconocida, pero se ha asociado al síndrome de Gardner, trauma, embarazo, estados hiperestrogénicos y puerperio. El objetivo de este artículo fue hacer una revisión sobre el tema a propósito de un caso clínico. Caso clínico. Se presenta el caso de una paciente puérpera con progresivo y rápido aumento del volumen abdominal. Se realizó una tomografía computarizada de abdomen y pelvis que confirmó la presencia de una masa intraperitoneal bien definida. La paciente fue operada con escisión de la masa y confirmación histológica de tumor desmoide a partir de la muestra de patología. Discusión. Los tumores desmoides tienen una incidencia de 2 a 4 casos por millón de habitantes por año, con leve predominio en el sexo femenino y representan menos del 3 % de los tumores de partes blandas. Aunque el tumor se puede ubicar a nivel intraabdominal o en la pared, la ubicación más común es en las extremidades. Conclusiones. La sospecha y detección del tumor desmoide es fundamental, así como su adecuado estudio, para determinar el tratamiento quirúrgico como fue realizado en este caso

Introduction. Desmoid tumors or aggressive fibromatosis correspond to rare mesenchymal neoplasms. They are locally aggressive tumors that occur especially in young people, they do not develop distant metastases, but are associated with locoregional invasion and a high recurrence rate after resection. Its etiology is unknown, but it has been associated with Gardner syndrome, trauma, pregnancy, hyperestrogenic states, and puerperium. The objective of this article was to review the topic based on a clinical case. Clinical case. The case of a puerperal patient with progressive and rapid increase in abdominal volume is presented. An abdominal and pelvic CT scan was performed, which confirmed the presence of a well-defined intraperitoneal mass. The patient underwent surgery with excision of the mass and histological confirmation of a desmoid tumor from the pathology sample. Discussion. Desmoid tumors have an incidence of 2 to 4 cases per million inhabitants per year, with a slight predominance in females, and represent less than 3% of soft tissue tumors. Although the tumor can be located intra-abdominal or in the wall, the most common location is in the extremities. Conclusions. The suspicion and detection of the desmoid tumor is essential, as well as its adequate study to determine the surgical treatment as it was done in this case

Ultrasound image and pathological features analysis of invasive fibromatosis / 肿瘤研究与临床

Objective:To investigate the ultrasound image and pathological features of invasive fibromatosis, and to provide a basis for the diagnosis of invasive fibromatosis.Methods:The clinicopathological data of 22 patients pathologically diagnosed with invasive fibromatosis from January 2016 to March 2019 in Shanxi Provincial Cancer Hospital were retrospectively analyzed. The clinical, ultrasound and pathological data were also summarized.Results:Ultrasound images of invasive fibromatosis showed irregular morphology, unclear boundaries, uneven echo, spot-like or strip-shaped blood flow signals. The coincidence rate of ultrasound diagnosis was 59.1% (13/22), 3 cases were misdiagnosed as fibrous, fat and other sarcomas, 4 cases were misdiagnosed as nerve-derived tumors, 1 case was misdiagnosed as nodular fasciitis, and 1 case was misdiagnosed as gastrointestinal stromal tumor. The pathological characteristics of invasive fibromatosis were more typical, and the positive expression rate of vimentin and β-catenin in immunohistochemistry was 100.0% (22/22); the coincidence rate of preoperative pathological diagnosis of puncture was 78.6% (11/14), 1 case was misdiagnosed as nerve fiber tumor, 1 case was misdiagnosed as low-grade fibromyxoid sarcoma, and 1 case was misdiagnosed as nodular fasciitis.Conclusion:Invasive fibromatosis has a certain specificity in ultrasound and pathological diagnosis, which can be diagnosed and differentially diagnosed according to the ultrasound image and pathological characteristics.

A case report of ureteral stenosis secondary to pelvic aggressive fibromatosis / 中华泌尿外科杂志

Aggressive fibromatosis(AF)is a rare soft tissue tumor clinically that presents with local aggressive growth, are prone to relapse, but rarely metastasize to distant sites. It occurs in the limbs, trunk, mesentery, etc., but is rare in the pelvis or around the urinary system. This article reported a case of ureteral stenosis secondary to pelvic AF. The surgical treatment was effective.

Tumores desmoides: consideraciones diagnóstico-terapéuticas / Desmoid tumors: diagnostic and therapeutic considerations

Resumen Se realizó una revisión bibliográfica de los tumores desmoides, lo cuales afectan los tejidos blandos con un comportamiento localmente agresivo sin capacidad de producir metástasis. Los casos esporádicos se localizan en extremidades y pared torácica; los casos hereditarios tienen predilección intraabdominal y los asociados con el embarazo en la pared abdominal. Las técnicas de imagen evalúan la extensión de la enfermedad. La biopsia con aguja trucut es el estudio de elección para el diagnóstico. Las mutaciones en el gen CTNNB1 o en el gen de APC provocan acumulación anormal de betacatenina en la célula. En esta revisión se hace énfasis en la evolución y cambio de las estrategias terapéuticas y se analizan las actuales herramientas para la toma de decisiones, así como los resultados clínicos. La radioterapia puede tener un papel terapéutico o adyuvante. Los avances en la comprensión de la enfermedad han permitido establecer tratamientos mejor dirigidos y con menor morbilidad; sin embargo, aún existen interrogantes en cuanto a la elección del candidato ideal para la vigilancia o el tratamiento precoz. También se presentan datos relacionados con la calidad de vida y la incertidumbre que genera el diagnóstico en el médico y el paciente.

Abstract A literature review on desmoid tumors was carried out, which are tumors that affect soft tissues with a locally aggressive behavior and are unable to metastasize. Sporadic cases are located on the extremities and chest wall; hereditary cases have an intra-abdominal predilection, and those associated with pregnancy occur on the abdominal wall. Imaging techniques assess disease extension. Trucut biopsy is the study of choice for diagnosis. Mutations in the CTNNB1 or APC genes cause an abnormal accumulation of b-catenin within the cell. In this review, an emphasis is made on therapeutic strategies’ evolution and change, and current tools for decision making are analyzed, as well as clinical outcomes. Radiation therapy can play a therapeutic or adjuvant role. Advances in the understanding of the disease have allowed establishing better targeted treatments with lower morbidity; however, there are still unanswered questions regarding the choice of the ideal candidate for surveillance and/or early treatment. Data related to quality of life are also presented, as well as the uncertainty generated by this diagnosis for both doctor and patient.

Extra-abdominal aggressive fibromatosis treated with meloxicam and sorafenib: An encouraging option

Objective: Aggressive fibromatosis (AF), also called desmoid tumor, is an uncommon soft-tissue neoplasm. Characteristically, it expands locally without metastatic potential. However, its tendency of relapse after curative resections has been well documented. Effective treatment options have been limited and there is a clear need for novel treatment strategies. Methods: We used combination therapy including multikinase tyrosine kinase inhibitor for treating AF. Results: We presented a case of an extra-abdominal AF who was successfully treated with meloxicam and sorafenib combination in our clinic. She tolerated this therapy well with only mild side effects. To our knowledge, this is the first case report of an extra-abdominal AF with a major partial response to sorafenib and meloxicam combination. Conclusion: Due to the favorable toxicity profile of sorafenib and meloxicam, this combination might be an effective treatment option for patients with locally aggressive and inoperable AF.

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.

Clinical and prognostic characteristics of aggressive fibromatosis in children:A report of 27 cases / 中国实用儿科杂志

OBJECTIVE: To investigate the clinical characteristics,treatment strategies,and prognosis of aggressive fibromatosis in children. METHODS: A retrospective analysis was performed in 27 cases of aggressive fibromatosis from January 2007 to January 2018 in Beijing Children's Hospital,Capital Medical University. RESULTS: There were 16 males and 11 females in 27 cases of aggressive fibromatosis,with a median age of 65(35,96)months at diagnosis. Tumor locations were as follows: abdominal wall(n=3),abdominal cavity(n=5),and extra-abdominal(n=19). Preoperative imaging examination showed that the median maximum diameter of primary tumors was 9.8(5.3,12.1)cm. Upfront surgery was performed in all of the cases,and 10 cases received adjuvant chemotherapy combined with methotrexate and vinorelbine. Among the 27 cases,9 cases survived without disease,15 cases survived with disease,and 3 cases died of tumor recurrence or progression. The 5-year overall survival and event-free survival rates were 85.8% and 32.6%,respectively.Further analysis of survival showed that positive surgical margin was associated with tumor recurrence. CONCLUSION: Aggressive fibromatosis is a rare,intermediate tumor in children. It is easy to relapse after incomplete surgical resection.Chemotherapy plays a role in controlling the growth of tumors. Most of the children have good prognosis,but some of them may be disabled or even die because of persistent,fast-growing tumors.

Survival outcomes after adjuvant radiotherapy for aggressive fibromatosis depend on time frame and nuclear β-catenin

PURPOSE: To identify prognostic factors influencing progression-free survival (PFS) of aggressive fibromatosis (AF) after postoperative radiotherapy (PORT) and assess correlations between immunohistochemistry (IHC) features of β-catenin/smooth muscle actin (SMA) and PFS. MATERIALS AND METHODS: Records of 37 patients with AF treated by PORT from 1984 to 2015 were retrospectively reviewed. Fifteen patients underwent wide excision for AF and 22 patients received debulking operation. The median total dose of PORT was 59.4 Gy. IHC staining results of β-catenin and SMA were available for 11 and 12 patients, respectively. RESULTS: The median follow-up duration was 105.9 months. Five-year PFS rate was 70.9%. Tumor size or margin status was not related to PFS in univariate analysis (p = 0.197 and p = 0.716, respectively). Multivariate analysis showed that increased interval from surgery to PORT (>5.7 weeks) was a marginal risk factor for PFS (p = 0.054). Administration of PORT at the initial diagnosis resulted in significantly improved PFS compared to deferring PORT after recurrence (p = 0.045). Patient with both risk factors of deferring PORT after recurrence and interval from surgery to PORT >5.7 weeks had significantly lower 5-year PFS than patients without risk factor (34.1% vs. 100.0%; p = 0.012). Nuclear β-catenin intensity tended to inversely correlate with 5-year PFS, although it did not reach statistical significance (62.5% at low vs. 100.0% at high; p = 0.260). SMA intensity was not related to PFS (p = 0.700). CONCLUSION: PORT should be performed immediately after surgery irrespective of margin status or tumor size especially in recurrent case. Nuclear β-catenin staining intensity of IHC might correlate with local recurrence.

Fibromatosis musculoaponeurótica agresiva: presentación de un caso / Muscleneuropatic agressive fibromatosis: a case presentation

Resumen Paciente masculino de 40 años, consulta por dolor en región glútea izquierda, asociado a masa de crecimiento progresivo; inicialmente valorado por ortopedia, donde le realizan radiografía y gammagrafía sin diagnóstico definitivo; revalorado por ortopedia y traumatología, luego de resonancia magnética y biopsia del glúteo comprometido se diagnostica fibromatosis glútea, la cual fue tratada con una resección del tejido invasivo y posteriormente radioterapia complementaria con acelerador lineal. Conclusión: Los tumores desmoides son infrecuentes y representan 0,03 % de todas las neoplasias y < 3 % de todos los tumores de tejidos; la cirugía ha sido tradicionalmente el pilar terapéutico debido a la variabilidad en el curso clínico y la importancia del sitio involucrado se asocia al tratamiento y aplicación de radioterapia.

Abstract 40 year old male patient complains about pain in left gluteal region, associated with progressive growing mass; initially valued by orthopedics, which performed x-ray scan and gammagraphy without a definitive diagnosis. He was reassessed by orthopedics and traumatology, through magnetic resonance imaging and biopsy of the committed gluteal, and was diagnosed with gluteal fibromatosis, which was treated with invasive tissue resection and subsequently additional accelerator radiotherapy. Conclusions: Desmoid tumors are rare and account for 0.03 % of all malignancies and <3 % of all tumors of tissues. Surgery has traditionally been the therapeutic mainstay due to variability in the clinical course and importance of the site of involvement is associated with the treatment and application of radiation therapy.

Imaging manifestations of intra-abdominal aggressive fibromatosis and correlation with pathology / 实用放射学杂志

Objective To investigate the CT and MRI manifestations of intra-abdominal aggressive fibromatosis and correlation with pathology.Methods The CT and MRI manifestations of 26 cases with intra-abdominal aggressive fibromatosis confirmed by pathological examination were analyzed retrospectively.Results 26 cases showed single solid mass,13 cases showed well-circumscribed and round-like,9 cases wrapped around the common bile duct,intestine or ureter,4 cases were lobulated which had unclear margin with surrounding tissues in pelvic.All the lesions displayed isodensity or slightly low density non-enhanced CT appearance,heterogenous high intensity FS T2WI,some larger tumors showed mixed signal.CT/MRI enhanced scan showed gradual enhancement in 26 cases.Conclusion Intra-abdominal aggressive fibromatosis have some certain imaging features,MRI can offer the histo logical features of tumors and have some correlation with pathology.

A Case of Desmoid-Type Fibromatosis Occurring Around the Transverse Process of Cervical Vertebra / 대한이비인후과학회지

Fibromatosis is generally a benign tumor that arises from the musculoaponeurotic tissues of the body, rarely occurring in the head and neck region. This can be treated with a good prognosis, but sometimes recurs as a local invasion. Preoperative core needle biopsy and MR images are necessary to diagnose preoperatively and outline the tumor extent. The mainstay of treatment is complete surgical excision. Nonetheless, an excision is often difficult because of the complex anatomy or proximity of the tumor to vital structures in the head and neck region. We report a rare case of desmoid-type fibromatosis that occurred in the neck, closely attached to the transverse process of the cervical vertebra. The present article covers an extensive analysis of the case with a review of the related literature.

Tumor desmoide abdominal / Abdominal desmoid tumor

Los tumores desmoides son infrecuentes, presentan crecimiento localmente agresivo, no suelen dar metástasis pero con frecuencia desarrollan un crecimiento infiltrante que amenaza la vida y puede conducir a una gran morbi-mortalidad. Representan el 0.03% de todos los tumores. En base a la experiencia, los expertos recomiendan la administración de dosis altas de tamoxifeno y sulindac como tratamiento primario para los pacientes con tumores desmoides asociados a poliposis adenomatosa familiar (PAF). Sin embargo, el mejor enfoque después de la intervención quirúrgica para pacientes con tumores desmoides esporádicos, aún no se ha determinado (AU)

Desmoid tumors are infrequent, locally aggressive growth, do not usually metastasize but often develop an infiltrating growth that threatens life and can lead to great morbidity and mortality. They represent 0.03% of all tumors. Based on experience, experts recommend the administration of high doses of tamoxifen and sulindac as a primary treatment for patients with desmoid tumors associated with familial adenomatous polyposis (FAP). However, the best approach after surgical intervention for patients with sporadic desmoid tumors has yet to be determined (AU)

Diagnosis and treatment of aggressive fibromatosis after thyroidectomy / 局解手术学杂志

Objective To investigate the diagnosis and treatment of aggressive fibromatosis after thyroidectomy.Methods One patient who were admitted into our hospital on January 16,2014 were discovered to have left cervical mass at the regular follow-up 8 months after opertaion.The ultrasonic examination showed that there were low echo area of 58 mm ×30 mm in the 3 ~5 muscular layer at the left neck. And it was diagnosed as aggressive fibromatosis through the puncture disease inspection.Whereafter,the patient were readmitted into hospital and recieved radical resection of the tumor on left side of the neck.The clinical,pathological and radiological data of the patient were collect-ed and analyzed so ao to discuss the diagnosis and treatment experience combined with related literatures.Results Postoperative pathologic examination confirmed that it was aggressive fibromatosis.The patient was actively cooperated with perioperative treatment,and the postoperative recovery was good with no local recurrence during the follow-up up to now.Conclusion Early diagnosis and surgical treatment are needed for patients with aggressive fibromatosis after thyroidectomy.

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.

Unilateral Gingival Fibromatosis: a case report / Fibromatose Gengival Unilateral: um relato de caso

The gingival fibromatosis is a slow and progressive benign proliferation, which affects the gingival tissues. It may present a genetic inheritance and association with some syndromes. There are conservative and radical treatments, ranging from hygiene care to bloc resection of the affected bone. This case scenario is a 07 year-old child, who presented a nodular unilateral hyperplastic lesion in the right mandible, with sessile base and approximately 5 cm in its largest diameter. The patient presented difficulty of lip closure and slight swelling in the right area of the face. The tomographic image showed infiltration in buccal and lingual cortical of right mandible and tooth displacement. After lesion removal, the histopathologic diagnosis of fibromatosis was confirmed, with no relapse after 20 months of follow-up.

A Fibromatose Gengival é uma proliferação benigna, lenta e progressiva, que afeta os tecidos gengivais. Pode apresentar herança genética e associação à uma série de síndromes. Existem tratamentos conservadores e radicais, desde cuidados com higiene à ressecção em bloco do tecido ósseo afetado. Este é o caso clínico de uma criança, que apresentou lesão hiperplásica unilateral em mandíbula, com deslocamento dentário. Após remoção, o diagnóstico de hiperplasia gengival foi confirmado e não há recidiva da lesão até o momento.

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.

Clinical Characteristics and Adequate Treatment of Familial Adenomatous Polyposis Combined with Desmoid Tumors / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: The objective of this study was to examine the clincopathologic characteristics and outcomes of familial adenomatous polyposis (FAP) patients with and without desmoid tumors (DTs), including the risk factors for progression of FAP-related DTs. MATERIALS AND METHODS: We reviewed the medical records and database of all patients with FAP who were treated between January 1993 and December 2011. RESULTS: Of 75 FAP patients, 18 (24%) were FAP with DTs. Seventeen of these had intra-abdominal DTs and one had intra- and extra-abdominal DTs. We divided the patients into two groups according to type of resection; the R0 or R1 resection group, referred to as the curative resection group (eight patients), and the R2 resection/palliative operation/medical treatment group, referred to as the palliative resection group (10 patients). Two patients in the curative resection group and two patients in the palliative group had progressed to tumor growth by the follow-up (p=0.800). In univariate analysis, DT diagnosis before or simultaneously with FAP diagnosis (DTs unrelated to surgical trauma) was a significant risk factor for tumor progression at final follow-up. CONCLUSION: A multidisciplinary approach to DT treatment is needed, including nonsteroidal antiinflammatory drugs, anti-estrogens, cytotoxic agents, and surgery. However, the role of surgery in resectable and complicated tumors may be limited. DT unrelated to surgical trauma has a relatively poor prognosis.

Aggressive fibromatosis of the oral cavity.

Fibromatosis are proliferations of highly differentiated fibrous tissue. The deep or musculoaponeurotic types of fibromatosis are known as desmoid tumors, aggressive fibromatosis, or desmoid-type fibrosarcoma. It is commonly seen as abdominal tumors; the occurrence of these tumors in maxillofacial region is a rare entity. We report a case of aggressive fibromatosis in a 50-year-old female, who presented with a swelling of the left side of the face. The tumor was extending in to the maxillary antrum which was confirmed as aggressive fibromatosis on histopathological examination. The detailed discussion of etiology, incidence, management and prognosis of the same in corelation with the literature is presented in the article.

Fibroma desmoplásico del hueso / Bone desmoplastic fibroma

Se presentó un caso de un fibroma desmoplásico en una adolescente de 14 años de edad. Se realizó estudio radiográfico que reveló lesión osteolítica diafisometafisaria extensa en fémur derecho. El objetivo fue mostrar una tumoración con poca frecuencia estadística en la bibliografía nacional e internacional y mostrar el proceder y tratamiento definitivo. El estudio histológico corroboró presencia de abundantes fibras colágenas, que formaban bandas hialinizadas separadas por escasos fibroblastos de tamaño pequeño sin actividad mitótica. Se realizó curetaje de la lesión con inserción de cemento quirúrgico. La paciente tuvo una evolución posquirúrgica satisfactoria con incorporación inmediata al medio social

This is the case of a desmoplastic fibroma present in an adolescent aged 14. A radiographic study was conducted showing an extensive osteolytic diaphysis and metaphyseal lesion in right femur. The objective of present paper was to present a tumor with little statistic frequency in the national and international bibliography and also to show the procedure and definitive treatment. The histological study corroborates the presence of abundant collagen fibers forming hyalinization strips separated by scarce small fibroblasts without mitosis activity. Curettage of lesion was performed with insertion of surgical cement. Patient had a satisfactory postsurgical course with an immediate incorporation to social environment