47 results on '"Tumor desmóide"'
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2. DEFICIÊNCIA INTELECTUAL RELACIONADA À DELEÇÃO INTERSTICIAL DO BRAÇO LONGO DO CROMOSSOMO 5, ABRANGENDO LOCUS SUPRESSOR TUMORAL DO GENE APC (ADENOMATOUS POLYPOSIS COLI), RESULTANDO EM POLIPOSE ADENOMATOSA FAMILIAR ASSOCIADA A TUMOR DESMÓIDE
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Nadia Sclearuc de Siqueira, Simone Reges Perales, and Carla Manzoni Salgado
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APC ,Cromossomo 5 ,Polipose adenomatosa familiar ,Retardo mental ,Tumor desmóide ,Diseases of the blood and blood-forming organs ,RC633-647.5 - Abstract
Introdução/Justificativa: A polipose adenomatosa familiar (PAF) é uma síndrome hereditária com padrão autossômica dominante com penetrância de quase 100%, caracterizada por múltiplos pólipos no trato gastrointestinal, predispondo ao desenvolvimento de câncer colorretal. Neste relato de caso, discutimos o diagnóstico raro relacionado à deleção intersticial do braço longo do cromossomo 5, abrangendo locus supressor tumoral do gene APC (adenomatous polyposis coli) com os achados consequentes de PAF (polipose adenomatosa familiar), tumor desmóide e retardo mental. Abordamos a relevância do diagnóstico precoce e do aconselhamento genético em casos complexos como este. Relato: Um homem de 20 anos, com história de atraso de desenvolvimento mental e cariótipo alterado mostrando deleção em parte do braço longo do cromossomo 5. Durante seus 20 anos de vida, permaneceu em atendimentos pediátricos e neurológicos de rotina, além de terapias de apoio em função do retardo mental. Foi diagnosticado incidentalmente com um tumor desmóide durante herniorrafia inguinal esquerda eletiva,sendo inicialmente optado por observação. Cerca de sete meses após, apresentou desconforto abdominal progressivo por crescimento da massa abdominal. Foi submetido à ressecção cirúrgica completa sem complicações. O exame anátomo-patológico revelou tumor desmóide medindo 27 × 24 × 19 cm infiltrando tecido mesentérico e camada muscular entérica. Foi referenciado para acompanhamento oncológico por tumor desmóide abdominal O Interrogatório complementar identificou o histórico de dentes supranumerários. Diante do quadro relatado foi considerada a hipótese de Polipose Adenomatosa Familiar (PAF) e solicitados exames complementares. A colonoscopia revelou polipose difusa no íleo terminal, alças colônicas e reto. O painel de sequenciamento genético revelou uma variante patogênica com deleção nos éxons 1 a 16 do gene APC, confirmando o diagnóstico de PAF. O paciente foi encaminhado para avaliação quanto à realização de proctocolectomia profilática, investigação complementar por endoscopia digestiva alta e avaliação quanto à predisposição para neoplasia de tireóide. A testagem em cascata familiar também foi recomendada. Conclusão: Este caso ilustra a complexidade da PAF, especialmente quando associada a condições clínicas adicionais, como retardo mental e tumor desmóide. A identificação precoce desses pacientes de risco é crucial para o manejo adequado, incluindo o rastreamento precoce de tumores e o aconselhamento genético. A investigação genética revelou uma variante patogênica no gene APC. Possivelmente, o presente caso envolve a origem de novo da mutação, o que ocorre em cerca de 25% dos casos de PAF, tendo em vista que não há um histórico familiar típico para PAF. O acompanhamento a longo prazo desses pacientes e de suas famílias é essencial para o manejo adequado e para reduzir o risco de complicações relacionadas à PAF.
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- 2024
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3. Desmoid Tumor after Sleeve Gastrectomy: Case Report and Literature Review
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Renato Medas, Rosa Coelho, Renato Bessa-Melo, Pedro Pereira, and Guilherme Macedo
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desmoid tumor ,mesenteric tumor ,sleeve gastrectomy ,bariatric surgery ,tumor desmóide ,tumor mesentérico ,gastrectomia vertical ,cirurgia bariátrica ,Diseases of the digestive system. Gastroenterology ,RC799-869 - Abstract
Desmoid tumor is a rare mesenchymal neoplasm of unknown etiology. Despite rare, the diagnosis of desmoid tumors after bariatric surgery is increased over the last few years. We report a case of a 26-year-old male with complains of abdominal pain and postprandial fullness, diagnosed with a locally advanced large intra-abdominal mass (40 × 21 × 11.7 cm) centered in the mesentery, developed 3 years after sleeve gastrectomy. Percutaneous biopsy was suggestive of a mesenquimatous tumor and the patient underwent surgery. R0 surgical resection was achieved, despite intimal contact and common vascularization with a jejunal loop. Histopathology examination of the surgical specimen revealed fusiform to stellate cells with mild atypia, thin-walled vessels, and diffuse beta-catenin expression (negative for DOG-1, CD117, CD34, S100, desmin, and alpha-actin). The diagnosis of a desmoid tumor was made. The patient remained asymptomatic, and no recurrence occurred over a 4-year follow-up. With the increasing number of bariatric surgeries, owing to the alarming growing incidence of obesity and related conditions, it is expected that desmoid tumors reports will gradually increase over the next few years. Thus, both gastroenterologists and surgeons should be aware of the potential for desmoid tumor development shortly after surgery, to offer a prompt diagnosis and treatment.
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- 2023
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4. Fibromatosis subescapular como causa de escápula alada. Presentación de un caso y revisión bibliográfica
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Miguel González López, Renato Alessandro Delfino Carrillo, Pablo César Arviza-Lorenzo, Cristina Madrid de la Serna, and Lydia Cecilia Escribano Rueda
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escápula alada ,tumor desmoide ,tratamiento ,Orthopedic surgery ,RD701-811 - Abstract
La escápula alada suele producirse por lesiones neurológicas tanto del nervio espinal como del nervio torácico largo. La aparición a causa de un tumor ventral de la escápula dificulta su diagnóstico inicial. Presentamos el caso de una mujer joven, con limitación para la rotación externa del hombro, sin antecedentes traumáticos conocidos, evolución progresiva e imagen compatible con una lesión de partes blandas dependiente de la aponeurosis del músculo subescapular confirmada por biopsia como un tumor desmoide. Aunque se trata de un tumor benigno y autolimitado, tiene una inquietante alta tasa de recidivas después de la resección, por lo que se dispone de numerosos tratamientos y muchos grupos optan por hacer un seguimiento médico estrecho de los factores pronósticos y las limitaciones funcionales del paciente, con lo que obtienen resultados satisfactorios y, en algunas series, superiores a los del tratamiento quirúrgico.
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- 2023
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5. Tumor desmóide intraperitoneal
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José Pio Furtado, Fernando A. Pitrez, João Regis Oliveira, João G. Scholl, Enelruy Farias, and Zygmunt Wojcicki Filho
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Tumor Desmóide ,Fibromatose ,Neoplasms. Tumors. Oncology. Including cancer and carcinogens ,RC254-282 - Abstract
Os autores, a propósito de um caso de tumor desmóide intraperitoneal operado no Hospital Santa Rita/ISCMPA, apresentam os aspectos patológicos e terapêuticos desta intrigante neoplasia classificada como pertencente ao grupo das fibromatoses. Tal neoplasia foi achado intra-operatório de laparotomia por volumosa massa abdominal, que redundou na exérese ampla de um grande tumor de aspecto homogeneamente maciço e nacarado, apenas laxamente aderido às vísceras circunjacentes. Na formulação do diagnóstico diferencial os autores sugerem que sua eventual ocorrência deva ser lembrada, marcadamente na vigência de situações clínicas similares.
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- 2023
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6. Fibromatosis mesentérica solitaria
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Patricia López Penza, María Noel Pereira, Carlos Costa Paz, and María Oreggia Carrau
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TUMOR DESMOIDE ,TUMOR PELVIANO ,FIBROMATOSIS INTESTINAL ,MASA PELVIANA ,INTESTINO DELGADO ,Medicine ,Medicine (General) ,R5-920 - Abstract
La fibromatosis mesentérica es un subtipo profundo de tumor desmoide (TD), un tumor benigno de origen fibroblástico localmente agresivo por su tendencia a infiltrar los tejidos adyacentes. Son raros, esporádicos y pueden asociarse con el síndrome de Gardner. El tratamiento de elección es la resección completa, evitando la recurrencia local. Comunicamos el caso clínico de una paciente con fibromatosis intrabdominal mesentérica única, bien circunscripta, que simulaba por la imagenología una masa de origen pelviano.
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- 2022
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7. Reconstrucción compleja de gran defecto de pared torácica derecha tras resección de tumor desmoide. A propósito de un caso.
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PLAZA SUÁREZ, Oger Gabriel, CENTENO, Jorge, ACURERO, Gleriset, and FINOL, Adrian
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Desmoid tumor is an uncommon form of fibromatosis of extremely rare musculoaponeurotic origin. Most are located in the abdomen, and its location in the chest wall is very rare. Due to its inability to produce metastases it has been considered benign, however, its rapid and aggressive local growth makes the surgical treatment equally aggressive and complete. We present a case of a thoracic wall desmoid tumor in a 40-year-old patient, one year evolution, with rapid and aggressive growth which deforms the anatomy of the thoracic wall. Excision of the tumor and 5 partially costal arches were performed, with subsequent thoracic reconstruction with prosthetic material and dermo-fat flap. Desmoid tumors, in advanced stages, requires multidisciplinary participation. [ABSTRACT FROM AUTHOR]
- Published
- 2021
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8. Fibromatosis musculoaponeurótica agresiva: presentación de un caso
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Cristian Pulido-Medina, Bibiana Matilde Bernal-Gomez, Daniela Jimenez-Peña, and Leonel Vega-Useche
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fibromatosis agresiva ,tumor desmoide ,biopsia ,radioterapia ,Medicine ,Nursing ,RT1-120 ,Public aspects of medicine ,RA1-1270 - Abstract
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.
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- 2018
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9. Fibromatosis gástrica: un tumor raro en una localización infrecuente. Reporte de un caso.
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Pacheco-Molina, Carlos, Baeza-Zapata, Armando A., García-De León, Óscar R., Delgado-Cortés, Héctor M., Salazar-Islas, Tania L., and Navarro-Bahena, Eduardo
- Abstract
Copyright of Cirugía y Cirujanos is the property of Publicidad Permanyer SLU and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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- 2020
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10. Tumor desmoide intraabdominal. Presentación de caso clínico y revisión de la literatura.
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Palacios-Fuenmayor, Luis J., Naranjo-Isaza, Ana M., Fuentes, Ossian, Palacio, María F., Martelo, Alfredo, Gómez, Luis R., and García, Héctor
- Abstract
Copyright of Cirugía y Cirujanos is the property of Publicidad Permanyer SLU and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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- 2020
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11. AWAKE XIFOPUBIC LAPAROTOMY FOR DESMOID TUMOR DURING PREGNANCY – A CASE REPORT
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Silva, Mafalda
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Anestesia obstétrica ,Obstetrical anaesthesia ,Desmoid tumor ,Regional anaesthesia ,Tumor desmóide ,Anestesia regional - Abstract
Non obstetric surgery during pregnancy is rare with maternal malignancy being one of the most common causes. Sporadic pregnancy related desmoid tumours are even more uncommon and optimal management of this tumour is complex. Complete surgical excision is the most commonly accepted treatment. Surgery during pregnancy is challenging as it demands taking into consideration requirements for both the mother and the foetus. The choice of anaesthetic plan must take into account surgical and patient specificities, and the effects of anaesthesia on the foetus. We report the perioperative management of a pregnant woman in the second trimester of pregnancy who underwent complete excision of a desmoid tumour under regional anaesthesia. Intraoperative change of surgical plan posted an additional challenge. , A cirurgia não obstétrica durante a gravidez é rara, sendo uma das causas mais comuns a excisão de tumores maternos. Tumores desmóides esporádicos na gravidez, constituem uma causa ainda mais rara, sendo o tratamento ideal complexo. A excisão cirúrgica completa é atualmente o tratamento mais aceite. A cirurgia durante a gravidez é um desafio uma vez que requer ter em consideração ambos a mãe e o feto. A escolha de um plano anestésico requer ter em linha de conta especificidades cirúrgicas e da paciente, bem como os efeitos da própria anestesia no feto. Este caso clínico relata o manuseio perioperatório de uma grávida no segundo trimestre de gravidez que foi submetida a uma excisão completa de um tumor desmóide sobre anestesia loco regional. A alteração intraoperatória do plano cirúrgico constituiu um desafio adicional.
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- 2023
12. Fibromatosis Intraabdominal Mesentérica de Identificación Postmortem. Reporte de caso
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Edwin Eduardo Velásquez Maldonado, Nicolás Sabillón, and Ana Molina
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Fibromatosis abdominal ,Neoplasia abdominal ,Fibromatosis profunda ,Tumor desmoide ,Reporte de caso ,Criminal law and procedure ,K5000-5582 ,Medical legislation ,K3601-3611 ,Public aspects of medicine ,RA1-1270 ,Social pathology. Social and public welfare. Criminology ,HV1-9960 - Abstract
La Fibromatosis Intraabdominal es una entidad rara, de etiología desconocida, benigna, sin riesgo de metástasis, pero de comportamiento clínico muy agresivo. Se presenta el caso de femenina de 47 años de edad con paro de evacuaciones de un mes de evolución, marcada distensión abdominal e intolerancia a la vía oral, quien fue manejada en la emergencia de adultos de un hospital público, sin poder determinar su diagnóstico, quien falleció en su casa 30 días después de su última atención hospitalaria. El diagnostico post-mortem en la autopsia médico legal se estableció como Fibromatosis Intraabdominal. Se requiere un alto índice de sospecha ante esta enfermedad con el fin de proveer manejo adecuado a las complicaciones asociadas y disminuir la mortalidad atribuible a ellas
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- 2018
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13. Fibromatosis musculoaponeurótica agresiva: presentación de un caso.
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Pulido-Medina, Cristian, Bernal-Gomez, Bibiana Matilde, Jimenez-Peña, Daniela, and Vega-Useche, Leonel
- Abstract
Copyright of Salud Uninorte is the property of Fundacion Universidad del Norte and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
- Published
- 2018
14. Reporte de caso: tumor desmoides gigante de pared abdominal con rápido crecimiento durante el embarazo.
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Palacios-Zertuche, Jorge Tadeo, Cardona-Huerta, Servando, Juárez-García, María Luisa, Valdés-Flores, Everardo, and Muñoz-Maldonado, Gerardo Enrique
- Abstract
Copyright of Cirugía y Cirujanos is the property of Publicidad Permanyer SLU and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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- 2017
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15. Risk factors for local recurrence of fibromatosis.
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Machado, V., Troncoso, S., Mejías, L., Idoate, M.Á., and San-Julián, M.
- Abstract
Copyright of Revista Española de Cirugía Ortopédica y Traumatologia (English Edition) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
- Published
- 2017
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16. Pediatric fibromatosis involving mandible: case reportand a five-year post-operative follow-up
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Vilson Lacerda Brasileiro Junior, Laura Priscila Barboza de Carvalho, Cláudia Roberta Leite Vieira de Figueiredo, and Marcos Antônio Farias de Paiva
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fibroma desmoplásico ,fibromatose extra-abdominal ,tumor desmoide ,Pathology ,RB1-214 - Abstract
Extra-abdominal fibromatosis is a benign fibrous neoplasm of locally aggressive behavior. Surgical excision with a wide margin is the treatment of choice. The aim of the present work is to report the case of fibromatosis in an 11-year-old melanodermic patient, who showed swelling in the area of the right mandible. Conservative surgery was performed based on the initial histopathological diagnosis of benign lesion suggestive of neural origin. The new anatomopathological examination of the surgical specimen was compatible with fibromatosis. The patient is still under periodic observation as part of the five-year surgical follow-up, showing no signs of recurrence.
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- 2013
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17. Management of desmoid-type fibromatosis involving peripheral nerves
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Mario G. Siqueira, Paulo L. Tavares, Roberto S. Martins, Carlos O. Heise, Luciano H.L. Foroni, Marcelo Bordalo, and Roberto Falzoni
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fibromatose tipo desmoide ,tumor desmoide ,fibroma ,nervos periféricos ,Neurosciences. Biological psychiatry. Neuropsychiatry ,RC321-571 - Abstract
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.
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- 2012
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18. Tumor desmoide intra abdominal esporádico
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Pablo Santiago, Adriana Cedrés, Carlos Ferreira, and Mónica Yapur
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fibromatosis agresiva ,tumor desmoide ,Surgery ,RD1-811 - Abstract
Se analiza el caso de un paciente de 44 años que consultó por una tumoración intra abdominal, que correspondió a un tumor desmoide. Se comentan los pasos diagnósticos y terapéuticos. De la revisión bibliográfica y su análisis surgen varios hechos interesantes a destacar. En primer lugar lo difícil del diagnóstico diferencial con los GIST, para lo cual es necesario recurrir a la inmunohistoquímica. Los desmoides intra abdominales solo corresponden al 10% de todos los desmoides. Por otro lado, y dada la poca frecuencia de esta afección, señalamos la poca evidencia que existe en cuanto a la más correcta modalidad terapéutica. Se analizan factores clínicos, paraclínicos, etiológicos y terapéuticos
- Published
- 2004
19. Fibromatosis mamaria. Reporte de dos casos clínicos y revisión bibliogáfica
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Covadonga Martí Álvarez, José Ignacio Sánchez Méndez, Laura Yébenes Gregorio, Alba Táboas Álvarez, Alicia Hernández Gutiérrez, and David Hardisson Hernáez
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nódulo mamario ,Obstetrics and Gynecology ,Fibromatosis ,tumor desmoide - Abstract
RESUMEN Introducción y objetivos: La fibromatosis produce tumores benignos pero localmente agresivos, que afectan a los tejidos blandos. A nivel mamario, representa tan sólo el 0.2% de las neoplasias de la mama. Nuestro objetivo con el presente artículo es profundizar en el conocimiento de la fibromatosis mamaria, a través del estudio de dos casos clínicos, mostrando sus características clínico-radiológicas e histológicas, e intentar establecer un protocolo de actuación adecuado. Métodos: Estudio retrospectivo de dos casos clínicos de fibromatosis mamaria diagnosticados en el Hospital Universitario La Paz entre los años 2018 y 2019. Resultados: Presentaremos dos pacientes con diagnóstico de fibromatosis mamaria, ambas debutaron con la autopalpación de un nódulo mamario. Al realizarles una ecografía, se visualizó un nódulo sólido, mal definido y axila ecográficamente negativa, que precisó de biopsia-aspiración con aguja gruesa. En los dos casos, se decidió resección quirúrgica de la lesión. Seguimiento mediante exploración mamaria y pruebas de imagen periódicas. Conclusiones: Aunque se trata de una entidad benigna, la fibromatosis mamaria puede simular un proceso maligno, tanto clínica como radiológicamente, por lo que precisa de un estudio histológico. A pesar de que la diseminación metastásica es muy poco frecuente, no se debe olvidar el carácter agresivo a nivel local de esta patología, y sus altas tasas de recurrencia. Como tratamiento, se debe realizar una resección quirúrgica, aunque recientemente se ha contemplado la opción de vigilancia estrecha sin tratamiento. No existe evidencia científica que justifique la utilización de otros tratamientos como la radioterapia o el tratamiento hormonal.
- Published
- 2019
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20. Tumor desmoides: Reporte de caso.
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Seijas-Jerónimo, R., Guzmán-Hidalgo, M., Guerra-GonzálezGail, L., Fuentes-Seijas, M., and Díaz-Izarra, A. J.
- Abstract
The desmoid tumor also known as muscle-aponeurotic fibromatosis, aggressive fibromatosis, deep fibromatosis, no metastatic fibrosarcoma and fibrosarcoma grade I, is an aggressive fibromatosis of slow and gradual installation .There are discrepancies regarding optimal treatment; because of its benign nature surgery is justified, but in some cases, chemotherapy has been administered, when evolution is not the usual and has existed response to this medication. Desmoid tumors can be considered as sarcomas with very low virulence. Conventional wisdom would indicate that such tumors would not have any response to chemotherapy, but the opposite happens. It has an incidence of 3-4 cases per million, with a peak between 25 and 35 years of age, affecting mainly females. Presents high probability of recurrence. It usually occurs in women in the postpartum period, the rectus abdominis and the scars of abdominal surgery. But can appear in any muscle. We present the case of a female aged 27 with APP health until November 2010 when she was diagnosed with left renal calculi and subjected to partial nephrectomy lumbotomy. One year and nine months after, having eight weeks of pregnancy, in the area of the scar a tumor appears. The tumor had rapid growth hard consistency without being painful, after diagnostic procedures, it was concluded as a desmoid tumor, expressing their diagnosis and treatment, the case was diagnosed in the Camilo Cienfuegos Hospital of Sancti Spiritus Cuba. [ABSTRACT FROM AUTHOR]
- Published
- 2015
21. Reconstrucción compleja de gran defecto de pared torácica derecha tras resección de tumor desmoide. A propósito de un caso
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Plaza Suárez,Oger Gabriel, Centeno,Jorge, Acurero,Gleriset, and Finol,Adrian
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Tumor desmoide ,Pared torácica ,Colgajo dermograso ,Reconstrucción pared torácica - Abstract
Resumen El tumor desmoide es una forma infrecuente de fibromatosis de origen musculoaponeurótico extremadamente raro. En su mayoría, se localiza en abdomen y es muy raro en pared torácica. Dada su incapacidad para metastatizar se ha considerado benigno, sin embargo, su crecimiento local rápido y agresivo hace que el tratamiento quirúrgico sea igualmente agresivo y completo. Presentamos un caso de tumor desmoide de pared torácica intervenido en paciente de 40 años, de 1 año de evolución y crecimiento rápido y agresivo con deformidad de pared torácica. Realizamos exéresis del tumor y de 5 arcos costales parcialmente, más reconstrucción con material protésico y colgajo dermograso. La resolución quirúrgica de este tipo de tumores en etapas avanzadas requiere participación interdisciplinaria.
- Published
- 2021
22. Fibromatosis mamaria: entidad histopatológica que simula cáncer. Reporte de un caso.
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Dávila-Zablah, Yesika, Garza-García, Nancy, Guerrero-Martínez, Marianela, and Garza-Montemayor, Margarita
- Subjects
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BREAST cancer surgery , *MYOFIBROBLASTS , *METASTASIS , *ETIOLOGY of cancer , *CANCER relapse - Abstract
Mammary fibromatosis or desmoid tumor is a rare lesion which accounts for less than 0.2% of all mammary tumors. It represents a proliferation of fibroblasts, myofibroblasts, or both, which infiltrate the stroma of the breast locally and aggressively but without potential to cause metastasis. The causes are unknown; however, there is a clear association with a history of traumatism and breast surgery (e.g. tumorectomy, reductive surgery, or placement of implants). The clinical and image presentations imitate, almost undistinguishably, an invasive carcinoma. The diagnosis is histological and the treatment of choice is full surgical resection, due to the tumor's high rate of local recurrence, even with free surgical margins. [ABSTRACT FROM AUTHOR]
- Published
- 2013
23. Fibromatosis mamaria en el varón. A propósito de un caso.
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Muñoz-Atienza, Virginia, Manzanares-Campillo, María del Carmen, Sánchez-García, Susana, Pardo-García, Ricardo, and Martín-Fernández, Jesús
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BREAST tumor treatment ,MALES ,HISTOLOGY ,MASTECTOMY ,ADJUVANT treatment of cancer ,SURGICAL excision ,TREATMENT effectiveness ,DISEASES - Abstract
Copyright of Cirugía y Cirujanos is the property of Publicidad Permanyer SLU and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
- Published
- 2013
24. Fibromatosis mamaria. Reporte de dos casos clínicos y revisión bibliogáfica
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Táboas Álvarez, Alba, Martí Álvarez, Covadonga, Sánchez Méndez, José Ignacio, Yébenes Gregorio, Laura, Hardisson Hernáez, David, and Hernández Gutiérrez, Alicia
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nódulo mamario ,desmoid tumor ,Fibromatosis ,mammary nodule ,tumor desmoide - Abstract
RESUMEN Introducción y objetivos: La fibromatosis produce tumores benignos pero localmente agresivos, que afectan a los tejidos blandos. A nivel mamario, representa tan sólo el 0.2% de las neoplasias de la mama. Nuestro objetivo con el presente artículo es profundizar en el conocimiento de la fibromatosis mamaria, a través del estudio de dos casos clínicos, mostrando sus características clínico-radiológicas e histológicas, e intentar establecer un protocolo de actuación adecuado. Métodos: Estudio retrospectivo de dos casos clínicos de fibromatosis mamaria diagnosticados en el Hospital Universitario La Paz entre los años 2018 y 2019. Resultados: Presentaremos dos pacientes con diagnóstico de fibromatosis mamaria, ambas debutaron con la autopalpación de un nódulo mamario. Al realizarles una ecografía, se visualizó un nódulo sólido, mal definido y axila ecográficamente negativa, que precisó de biopsia-aspiración con aguja gruesa. En los dos casos, se decidió resección quirúrgica de la lesión. Seguimiento mediante exploración mamaria y pruebas de imagen periódicas. Conclusiones: Aunque se trata de una entidad benigna, la fibromatosis mamaria puede simular un proceso maligno, tanto clínica como radiológicamente, por lo que precisa de un estudio histológico. A pesar de que la diseminación metastásica es muy poco frecuente, no se debe olvidar el carácter agresivo a nivel local de esta patología, y sus altas tasas de recurrencia. Como tratamiento, se debe realizar una resección quirúrgica, aunque recientemente se ha contemplado la opción de vigilancia estrecha sin tratamiento. No existe evidencia científica que justifique la utilización de otros tratamientos como la radioterapia o el tratamiento hormonal. ABSTRACT Introduction and objectives: Fibromatosis produces benign but locally aggressive tumours that affect soft tissues. At breast level, it represents only 0.2% of breast neoplasms. Our goal with this article is to increase knowledge on breast fibromatosis, through the study of two clinical cases; explaining their clinical-radiologic and histological characteristics. Additionally, try to establish an adequate protocol, for the management of the disease and for its subsequent monitoring. Methods: A retrospective study about two clinical cases of breast fibromatosis diagnosed in La Paz Hospital between 2018-2019. Results: both patients presented with clinical manifestations, autopalpation of a breast nodule. A breast ultrasound was performed and a solid nodule was visualized, with poorly defined edges and ecographically negative armpit. A core needle biopsy was performed to confirm the histological diagnosis. In both clinical cases, the treatment was surgical resection of the lesion. Periodic revisions are being performed in order to exclude recurrence. Conclusions: Although it is a benign disease, breast fibromatosis can simulate a malignancy, both in a clinical and radiological way, so histological study is mandatory in order to achieve an accurate diagnosis. Even metastatic dissemination is extremely rare, the local aggressive nature and high rates of recurrence for fibromatosis makes surgical excision, with wide free margins, the most important tool in treatment, although the possibility of close surveillance without treatment is recently being contemplated. There is no scientific evidence to justify the use of other treatments such as radiotherapy or hormonal treatment.
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- 2019
25. TUMOR DESMOIDE INTRA-ABDOMINAL COM ORIGEM RARA NA PAREDE INTESTINAL: RELATO DE CASO
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Josep Lluis Molinero Polo, Tomas Stickar, Yuhami Mitsahid Curbelo Peña, Manel Guixa Gener, Francesc Xavier Quer Vall, Jordi de Cozar Duch, Juan Andrés Dárdano Berriel, Tonia Palau Figueroa, Helena Valverdu Cartie, and Julia Gardenyes Martinez
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Pathology ,medicine.medical_specialty ,RD1-811 ,business.industry ,General Medicine ,RC799-869 ,Diseases of the digestive system. Gastroenterology ,Small bowel ,Text mining ,Tumor desmoide ,Intestino delgado ,medicine ,Abdominal desmoid tumor ,Surgery ,Desmoid tumor ,business ,Letter to the Editor - Published
- 2018
26. Tratamiento quirúrgico de los tumores de la pared abdominal
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Rafael Estevan, Fernando Carbonell, Jorge Campos, and Beatriz Llombart
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Gynecology ,Pared abdominal ,medicine.medical_specialty ,business.industry ,Gastroenterology ,Soft tissue sarcomas ,Dermatofibrosarcoma protuberans ,Desmoid tumour ,Abdominal wall ,Tumor desmoide ,medicine ,Sarcomas de partes blandas ,Surgery ,business - Abstract
ResumenLa extirpación quirúrgica es el tratamiento de elección en los tumores de la pared abdominal. La radioterapia puede resultar muy útil para reducir el volumen de los tumores y hacerlos accesibles a la cirugía, además de servir como tratamiento adyuvante en casos de tumores de alto riesgo de recidiva o cirugías subóptimas. El tipo de exéresis variará en función del tipo de tumor. Así, en tumores benignos, una exéresis simple será suficiente. En caso de tumores de malignidad intermedia (poca capacidad de metástasis pero con alto riesgo de recaídas locales), la cirugía se irá adaptando a esta, pasando de una cirugía amplia para el tumor desmoide a una cirugía micrográfica de Mohs para el dermatofibrosarcoma protuberans y a una cirugía radical compartimental con reconstrucción de la pared para el sarcoma de partes blandas. Las metástasis en la pared abdominal se tratarán de la misma forma que los sarcomas. En cualquier caso, para el éxito de cualquier intervención es fundamental conseguir unos márgenes quirúrgicos libres de tumor, junto a una estética y funcionalidad aceptables.AbstractSurgical removal is the cornerstone of treatment of tumours of the abdominal wall. Radiation therapy can be very helpful in reducing tumour volume and make them accessible to surgery and adjuvant treatment for tumours at high risk of recurrence or suboptimal surgery. Excision type vary depending on the type of tumour. Thus, a simple excision for benign tumours will suffice. In case of tumours of intermediate malignancy (metastasis but little capacity at high risk of local relapse) surgery will adapt to it; going from a wide desmoid tumour surgery for a Mohs micrographic surgery for dermatofibrosarcoma protuberans and one compartment radical surgery with reconstruction of the wall for soft tissue sarcoma. Metastases in the abdominal wall are treated in the same way as sarcomas. In any case for the success of any intervention it is critical to achieve tumour-free surgical margins, along with acceptable aesthetics and functionality.
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- 2015
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27. Tumor desmoide de la pared torácica. ¿Tratamiento quirúrgico o multimodal?
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Mier-Odriozola, José Manuel, López-Rodó, Laureano Molins, Fibla-Alfara, Juan José, and Vidal-López, Gonzalo
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SOFT tissue tumors ,DIAGNOSIS of diseases in women ,TREATMENT of diseases in women ,SURGICAL excision - Abstract
Copyright of Cirugía y Cirujanos is the property of Publicidad Permanyer SLU and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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- 2008
28. Fibromatosis de mama: una lesión simuladora de cáncer.
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Villarreal-Colin, Silvia Patricia, Soto-Dávalos, Baltazar Alberto, Bargalló-Rocha, Juan Enrique, Bandera-Delgado, Artfy, Zumaran-Cuéllar, Oscar, and Robles-Vidal, Carlos Daniel
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BREAST tumors ,ETIOLOGY of diseases ,TRAUMATISM ,GENETIC disorders ,NEEDLE biopsy ,SURGICAL excision ,THORACIC surgery - Abstract
Copyright of Cirugía y Cirujanos is the property of Publicidad Permanyer SLU and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
- Published
- 2008
29. Unusual finding after resection of rectal adenocarcinoma: Report of two cases
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Natalia Pranzetti Vieira, Cláudio Saddy Rodrigues Coy, Debora Helena Rossi, Priscilla de Sene Portel Oliveira, Maria de Lourdes Setsuko Ayrizono, Raquel Franco Leal, and João José Fagundes
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Rectal adenocarcinoma ,medicine.medical_specialty ,Recidiva pélvica ,RC799-869 ,Resection ,03 medical and health sciences ,0302 clinical medicine ,Biopsy ,medicine ,Rectal Adenocarcinoma ,Desmoid tumor ,Adenocarcinoma de reto ,medicine.diagnostic_test ,business.industry ,General surgery ,Gastroenterology ,Pelvic recurrence ,Diseases of the digestive system. Gastroenterology ,medicine.disease ,Tumor desmóide ,body regions ,030220 oncology & carcinogenesis ,Pelvic tumor ,030211 gastroenterology & hepatology ,Radiology ,Differential diagnosis ,Complication ,business - Abstract
Pelvic recurrence after resection of rectal adenocarcinoma is a feared complication and is associated with a worse prognosis and low resectability rates. The differential diagnosis is difficult, as biopsy is seldom performed preoperatively. We report two cases of desmoid pelvic tumor after resection of rectal adenocarcinoma. Therapeutic options and literature review are described. Resumo: O aparecimento de tumor pélvico após ressecção de adenocarcinoma de reto é complicação temida e está associado à pior prognóstico e baixos índices de ressecabilidade. O diagnóstico diferencial é difícil, pois o diagnóstico histológico usualmente não é realizado no pré-operatório. São relatados dois casos em que houve o aparecimento de tumor pélvico após a ressecção de adenocarcinoma de reto, com diagnóstico histológico de tumor desmóide. As condutas adotadas e revisão da literatura são descritas. Keywords: Desmoid tumor, Rectal adenocarcinoma, Pelvic recurrence, Palavras-chave: Tumor desmóide, Adenocarcinoma de reto, Recidiva pélvica
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- 2013
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30. Reporte de caso: tumor desmoides gigante de pared abdominal con rápido crecimiento durante el embarazo
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Jorge Tadeo Palacios-Zertuche, Servando Cardona-Huerta, Everardo Valdés-Flores, María Luisa Juárez-García, and Gerardo Muñoz-Maldonado
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Gynecology ,Medicine(all) ,medicine.medical_specialty ,Embarazo ,business.industry ,030230 surgery ,Desmoid tumour ,Rápido crecimiento ,03 medical and health sciences ,0302 clinical medicine ,Pregnancy ,Tumor desmoide ,030220 oncology & carcinogenesis ,Medicine ,Surgery ,Rapid growth ,business - Abstract
ResumenAntecedentesLos tumores desmoides son neoplasias raras monoclonales de tejido blando, que surgen a partir de células madre mesenquimales. Son uno de los tumores más raros en todo el mundo, con una incidencia anual estimada de 2-4 nuevos casos por millón de personas. Los cambios hormonales e inmunológicos que ocurren durante el embarazo pueden desempeñar un papel en la severidad y curso de la enfermedad.Caso clínicoMujer de 28 años de edad, en su quinta semana de gestación, a quien, al realizar ultrasonido de control prenatal, se le encontró tumoración de pared abdominal adherida a anexos izquierdos y útero. Se dejó a la paciente en vigilancia clínica y ecográfica. Acudió con actividad uterina anormal, se ingresó a Obstetricia con 38.2 semanas de gestación, se realizó cesárea y se tomó biopsia de la tumoración. Anatomía patológica reporta fibromatosis desmoide. Se realizó tomografía axial computada contrastada, que reportó tumoración con bordes bien definidos, en contacto con el útero, anexo izquierdo, vejiga y pared abdominal; sin datos de infiltración a estructuras adyacentes de 26×20.5×18cm. Se operó de forma electiva, se realizó laparotomía exploradora, con resección tumoral, histerectomía y salpingo-ooforectomía izquierda, técnica de separación de componentes, colocación de malla de polipropileno y drenajes. El reporte definitivo de enfermedad fue fibromatosis desmoide. Seis meses después de su intervención quirúrgica no ha presentado recurrencia.ConclusiónLos tumores desmoides son localmente agresivos y la resección quirúrgica con márgenes libres representa la base en el tratamiento de esta dolencia; la radioterapia, quimioterapia y la hormonoterapia se utilizan como complemento en el tratamiento de estas pacientes.AbstractBackgroundDesmoid tumours are one of the rarest tumours worldwide, with an estimated yearly incidence of 2-4 new cases per million people. They are soft tissue monoclonal neoplasms that originate from mesenchymal stem cells. It seems that the hormonal and immunological changes occurring during pregnancy may play a role in the severity and course of the disease.Clinical caseThe case is presented on 28-year-old female in her fifth week of gestation, in whom an abdominal wall tumour was found attached to left adnexa and uterus while performing a prenatal ultrasound. The patient was followed up under clinical and ultrasonographic surveillance. When she presented with abnormal uterine activity at 38.2 weeks of gestation, she was admitted and obstetrics decided to perform a caesarean section. Tumour biopsy was taken during the procedure. Histopathology reported a desmoid fibromatosis. A contrast enhanced abdominal computed tomography scan was performed, showing a tumour of 26×20.5×18cm, with well-defined borders in contact with the uterus, left adnexa, bladder and abdominal wall, with no evidence of infiltration to adjacent structures. A laparotomy, with tumour resection, hysterectomy and left salpingo-oophorectomy, components separation techniques, polypropylene mesh insertion, and drainage was performed. The final histopathology report was desmoid fibromatosis. There is no evidence of recurrence after 6 months follow-up.ConclusionsDesmoid tumours are locally aggressive and surgical resection with clear margins is the basis for the treatment of this disease, using radiotherapy, chemotherapy and hormone therapy as an adjunct in the treatment.
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- 2016
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31. Desmoid tumors: diagnostic and therapeutic considerations.
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Brener-Chaoul M, Cervantes-Gutiérrez Ó, Padilla-Longoria R, and Martín-Téllez KS
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- Anti-Inflammatory Agents therapeutic use, Antineoplastic Agents therapeutic use, Biopsy methods, Clinical Decision-Making, Female, Fibromatosis, Aggressive pathology, Humans, Male, Quality of Life, Radiotherapy, Uncertainty, beta Catenin metabolism, Fibromatosis, Aggressive diagnosis, Fibromatosis, Aggressive therapy
- 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., (Copyright: © 2019 Permanyer.)
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- 2020
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32. Intraabdominal desmoid tumor. Clinical clinical case and literature review.
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Palacios-Fuenmayor LJ, Naranjo-Isaza AM, Fuentes O, Palacio MF, Martelo A, Gómez LR, and García H
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- Abdominal Neoplasms diagnostic imaging, Abdominal Neoplasms surgery, Abdominal Wall surgery, Adolescent, Biopsy, Colectomy, Female, Fibromatosis, Aggressive diagnostic imaging, Fibromatosis, Aggressive surgery, Humans, Neoplasm Invasiveness, Neoplasm Recurrence, Local diagnostic imaging, Neoplasm Recurrence, Local pathology, Neoplasm Recurrence, Local surgery, Plastic Surgery Procedures, Surgical Mesh, Tomography, X-Ray Computed, Abdominal Neoplasms pathology, Fibromatosis, Aggressive pathology
- Abstract
Desmoid tumors are clinical entities rarely diagnosed at an initial presentation because of its low incidence, they are characterized by a locally aggressive presentation and high rates of local relapse. Its presentation can be intra- or extra-abdominal. We present a clinical case of a female, 15 year old patient, with three months of abdominal pain, a giant intra-abdominal mass was diagnosed with histologic diagnosis of desmoid tumor. Several surgical procedures were performed, having a las a R1 resection (focally microscopic margins). In this case association with pregnancy, abdominal trauma, previous surgeries and genetic syndromes were discarded., (Copyright: © 2020 Permanyer.)
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- 2020
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33. TUMOR DESMÓIDE – UMA REVISÃO DE LITERATURA
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Nagano, Stephanie YukaMatwijszyn, Passos, Renata Moreira Marques, Santana, Micael Cruz, and Guedes, Virgilio Ribeiro
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fibromatose músculo-aponeurótica ,tumor desmóide ,fibromatose agressiva - Abstract
Introdução: O tumor desmóide é uma neoplasia rara na qual ocorre proliferação de células do tecido conjuntivo. Materiais e Métodos: Foi realizada a revisão de literatura partindo de dados encontrados em artigos do Pubmed e Scielo. A pesquisa na internet foi feita com os seguintes descritores na língua portuguesa e inglesa: tumor desmóide, fibromatose agressiva e fibromatose musculo-aponeurótica Revisão: O tumor desmóide ocorre principalmente em região abdominal em mulheres em idade reprodutiva. É um tumor raro, com incidência de apenas 2-4 casos por milhão e corresponde a 3% de todos os tumores de partes moles. Há muitos meios terapêuticos que vem sendo estudados para tratamento do tumor desmóide, mas o padrão ouro ainda continua sendo a cirurgia. Conclusão: Apesar de raro e de não metastizar, o tumor desmóidepossui invasão local agressiva e deve ser tratado e estudado com maior afinco para evitar complicações secundárias.
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- 2015
34. Tratamento dos Tumores Desmóides Intra-Abdominais associados à Polipose Adenomatosa Familiar
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Martins, Sheila B., Leite, Júlio S., Oliveira, Ana S., Sá, Anabela, and Castro-Sousa, Francisco
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Familial adenomatous polyposis ,Polipose adenomatosa familiar ,Staging ,Tumor desmoide ,Estadiamento ,Desmoid tumour - Abstract
A evolução clínica dos tumores desmóides intra-abdominais é imprevisível. O objectivo deste estudo consistiu na análise dos resultados do tratamento dos doentes com tumores desmoides intra-abdominais associados a PAF, bem como o valor clínico do estadiamento de Church. Métodos: Estudo retrospectivo de todos os doentes operados de PAF, que nos últimos 25 anos desenvolveram tumores desmoides intra-abdominais. O estadiamento de Church baseia-se nos seguintes graus: I - Assintomático, 20cm, crescimento rápido. Todos foram submetidos a tratamento de tipo setp-up: AINEs, tamoxifeno, quimioterapia (baseada na doxorrubicina) e cirurgia para tratamento das complicações. Resultados: Foram estudados 10 doentes, sendo 8 mulheres, com idade média de 28 anos (17-67a); análise com seguimento médio de 7 anos. O diagnóstico de tumor desmóide foi estabelecido, em média, 3 anos após a cirurgia. Os quatro doentes no estádio I apresentaram regressão total após o tratamento médico. Os dois doentes do estádio III foram submetidos a cirurgia por obstrução intestinal e fístula da bolsa ileoanal, estando atualmente assintomáticos. Foi instituída quimioterapia a três doentes no estádio IV, que apresentaram regressão parcial do tumor e encontram-se assintomáticos, apesar de um necessitar de hemodiálise. O quarto doente do estádio IV faleceu por sépsis resultante do crescimento fulminante do tumor, apesar do tratamento com tamoxifeno e sulindac. Conclusões: Confirmou-se o valor clínico do estadiamento de Church assim como a eficácia da terapêutica step-up e dos esquemas de quimioterapia baseados na doxorrubicina para os tumores em crescimento progressivo. Evolution of intra-abdominal desmoids tumours is unpredictable. The aim of this study was to evaluate the management of intra-abdominal desmoids tumours in a surgical unit and the clinical value of Church’s desmoid tumour staging (2005). Methods: Retrospective analysis of patients with PAF that undergone surgery and developed intra-abdominal desmoid tumours in the last 25 years. Church staging: I - asymptomatic, 20cm or rapidly growing. All had conservative stepwise approach: NSAIDs, tamoxifen, chemotherapy (doxorubicin-based) and surgery for imminent complications. Results: There were 10 patients, 8 female, with a mean age of 28 years (17-67y); the median follow-up was 7 years. Desmoid tumours were diagnosed in a mean time of 3 years after surgery. After medical treatment four stage I patients had complete regression. Two stage III needed surgery due to bowel obstruction and another due to associated pouch fistula; both patients are asymptomatic. Chemotherapy was given to 3 stage IV patients which are asymptomatic with partial regression, although one needs hemodyalisis. Another stage IV patient died from sepsis and fulminant overgrowth of the tumour under tamoxifen and sulindac treatment. Conclusions: The clinical value of the desmoid staging was confirmed as well as the successful outcome of the conservative step-up approach and the doxorubicin-based chemotherapy for the progressively growing tumours.
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- 2015
35. Estudo retrospetivo de lesões tumorais do punho e mão
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Silvério,Sílvia
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Osteochondroma ,Tumor ,tumor de células gigantes tenossinoviais ,Enchondroma ,punho ,Wrist ,Hand ,osteocondroma ,quisto ósseo aneurismático ,Aneurysmal bone cyst ,encondroma ,mão ,Giant cell tumor of the tendon sheath ,tumor desmóide ,tumor glómico ,Desmoid tumor - Abstract
Objectivos: Realizar um estudo retrospectivo dos tumores do punho e mão da Unidade de Cirurgia da Mão e comparar os resultados com os existentes na literatura. Material e Métodos: Estudo retrospectivo realizado a partir dos diagnósticos de uma base de dados da Unidade no período de 2000 a 2009. Foram excluídos quistos sinoviais, bossas cárpicas e tofos gotosos por decisão dos autores. Os processos consultados foram analisados com base nos parâmetros: sexo; idade; diagnóstico; benignidade/ malignidade; primário secundário; origem (óssea, cutânea , vascular, nervosa); localização; clínica; tipo de cirurgia; evolução. A maioria dos diagnósticos foi confirmada por exame anatomo-patológico. Resultados: Obtivemos um número total de 65 tumores em 65 doentes estudados. Idade média de 49,6 anos; 42 casos do sexo feminino; 55,4% de tumores de tecidos moles (n:36); 44,6% de tumores ósseos (n:29); 1 caso (1,5%) de tumor ósseo maligno. Conclusões: Os resultados encontrados são semelhantes aos descritos na literatura. Os trabalhos existentes sobre tumores da mão são escassos, especialmente em relação aos mais raros, e na maioria dos casos não apresentam um seguimento a longo prazo que permita estabelecer uma orientação de tratamento. Devida à raridade dos tumores malignos, um especialista, na sua prática clínica, acaba por ter experiência limitada. Se a este facto se juntar um quadro clínico inicial pouco específico, será o suficiente para explicar a elevada percentagem de erros no seu diagnóstico numa fase inicial. Objective: to conduct a retrospective study of wrist and hand tumors treated in our Unit of Hand Surgery, and compare the results with those found in existing literature. Material and Methods: a retrospective study was carried out based on the diagnoses found in the database of our Unit, from 2000 to 2009. Synovial cysts, carpal lumps and gouty tophi were excluded from the study. The clinical files were analyzed using the following criteria: gender; age; diagnosis; benignity/malignancy; primary/secondary; origin (bony, cutaneous, vascular, neural); location; clinical presentation; surgical procedure; evolution. Most diagnoses were confirmed by anatomopathological exam. Results: we obtained a total number of 65 tumors, representing 65 patients. Mean age was 49.6 years, 42 females, 55.4% were soft tissue tumors (n = 36), 44.6% were bone tumors (n = 29), 1 case (1.5%) of malignant secundary bone tumor. Conclusion: the results are similar to those found in literature. The existing studies concerning hand tumors are scarce, especially regarding the rarest ones. Most studies do not include long-term monitoring, which would be necessary to establish a guideline for treatment. Given the rarity of malignant tumors, specialists end up having limited experience in their clinical practice. Furthermore, the initial clinical presentation is close to unspecific. This fact results in a high percentage of misdiagnoses in the initial phase.
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- 2014
36. Fibromatose pediátrica em mandíbula: relato de caso e avaliação pós-operatória de cinco anos
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Laura Priscila Barboza de Carvalho, Marcos Antônio Farias de Paiva, Vilson Lacerda Brasileiro Junior, and Cláudia Roberta Leite Vieira de Figueiredo
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medicine.medical_specialty ,fibroma desmoplásico ,business.industry ,desmoid tumor ,Clinical Biochemistry ,Fibromatosis ,extra-abdominal fibromatosis ,Mandible ,Benign lesion ,fibromatose extra-abdominal ,medicine.disease ,Surgical specimen ,desmoplastic fibroma ,tumor desmoide ,Pathology and Forensic Medicine ,Surgery ,Medical Laboratory Technology ,Desmoplastic fibroma ,Benign Fibrous Neoplasm ,medicine ,lcsh:Pathology ,Surgical excision ,Post operative ,business ,lcsh:RB1-214 - Abstract
Extra-abdominal fibromatosis is a benign fibrous neoplasm of locally aggressive behavior. Surgical excision with a wide margin is the treatment of choice. The aim of the present work is to report the case of fibromatosis in an 11-year-old melanodermic patient, who showed swelling in the area of the right mandible. Conservative surgery was performed based on the initial histopathological diagnosis of benign lesion suggestive of neural origin. The new anatomopathological examination of the surgical specimen was compatible with fibromatosis. The patient is still under periodic observation as part of the five-year surgical follow-up, showing no signs of recurrence. Fibromatose extra-abdominal é uma neoplasia fibrosa benigna, caracterizada por comportamento agressivo local. Geralmente, o tratamento de escolha consiste na excisão cirúrgica com ampla margem de segurança. O objetivo deste trabalho é relatar um caso de fibromatose em uma paciente de 11 anos, melanoderma, que apresenta aumento de volume na mandíbula do lado direito. A partir do diagnóstico histopatológico inicial sugestivo de lesão benigna de origem neural, foi realizado tratamento cirúrgico conservador. Um novo exame anatomopatológico da peça cirúrgica foi compatível com fibromatose. A paciente encontra-se sob controle periódico de cinco anos da cirurgia, não havendo recorrência clínica da lesão
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- 2013
37. Management of desmoid-type fibromatosis involving peripheral nerves
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Siqueira, Mario G., Tavares, Paulo L., Martins, Roberto S., Heise, Carlos O., Foroni, Luciano H.L., Bordalo, Marcelo, and Falzoni, Roberto
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fibromatose tipo desmoide ,fibroma ,nervos periféricos ,desmoid tumor ,peripheral nerve ,desmoid-type fibromatosis ,fibromatosis ,tumor desmoide - Abstract
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.
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- 2012
38. Management of desmoid-type fibromatosis involving peripheral nerves
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Carlos Otto Heise, Roberto Falzoni, Marcelo Bordalo, Luciano Foroni, Mario G. Siqueira, Roberto S. Martins, and Paulo Tavares
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fibromatose tipo desmoide ,Adult ,Male ,medicine.medical_specialty ,nervos periféricos ,medicine.medical_treatment ,desmoid tumor ,lcsh:RC321-571 ,Young Adult ,Peripheral Nervous System Neoplasms ,Humans ,Medicine ,Brachial Plexus ,Radical surgery ,lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry ,business.industry ,Cutaneous nerve ,Fibromatosis ,Soft tissue ,desmoid-type fibromatosis ,medicine.disease ,tumor desmoide ,Median nerve ,Median Nerve ,Peripheral ,Surgery ,Radiation therapy ,Fibromatosis, Aggressive ,fibroma ,Neurology ,peripheral nerve ,Female ,Neurology (clinical) ,Neoplasm Recurrence, Local ,business ,Brachial plexus ,fibromatosis - Abstract
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.
- Published
- 2012
39. Fibromatosis mamaria, diagnóstico diferencial del carcinoma invasor: Reporte de un caso
- Author
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DÍAZ C, M. LORENA, ARANCIBIA H, PATRICIA, MOYANO S, LEONOR, and BURGOS S, NELSON
- Subjects
desmoid tumor ,Breast fibromatosis ,mammary nodule ,Fibromatosis mamaria ,tumor desmoide - Abstract
Introducción: La fibromatosis o tumor desmoide, es una lesión infrecuente del tejido conectivo, que corresponde al 0,2% de los tumores mamarios. No tiene potencial de dar metástasis pero presenta alta tasa de recidiva local debido a sus márgenes infiltrativos. Su etiología es desconocida. La presentación clínica e imagenológica es casi indistinguible a la de un carcinoma mamario. El diagnóstico es histológico y el tratamiento de elección es la resección quirúrgica. Reporte de caso: Mujer de 31 años que consulta por retracción cutánea en mama derecha. No presenta antecedentes quirúrgicos ni de traumas previos. Se realiza ecografía mamaria que muestra, en relación al sitio de retracción, un nodulo irregular que se extiende hacia la piel. La mamografía muestra discreta retracción de la piel y aumento de densidad del tejido subcutáneo adyacente. Se realiza biopsia percutánea CORE bajo ultrasonido, que muestra proliferación estromal fusocelular, sugerente de un tumor filodes. Posteriormente, se realiza mastectomía parcial cuya histología muestra una proliferación mesenquimatosa de bordes infiltrativos, concordante con fibromatosis mamaria. Conclusión: La fibromatosis mamaria es una entidad poco frecuente y localmente agresiva. Su importancia radica en que las características clínicas, radiológicas y citológicas simulan con frecuencia tumores malignos de la mama. El tratamiento de elección es la cirugía, presentando gran tendencia a la recurrencia, incluso con márgenes libres. Se han descrito poco más de 100 casos en la literatura. Desmoid tumors or fibromatosis, a rare lesion of the connective tissue, corresponds to 0.2% of breast tumors. It does not metastasize but has high local recurrence rates due to margin infiltration. The clinical presentation and imaging is almost indistinguishable from breast carcinoma. Diagnosis is histological and the treatment of choice is surgical excision. We report a 31 years old woman consulting for skin retraction in right breast, without previous history of trauma or surgery. Breast ultrasound showed an irregular nodule that extended into the skin. The mammogram showed a discrete retraction of the skin and increased density of adjacent subcutaneous tissue. An ultrasound guided percutaneous biopsy showed stromal fusocelular proliferation, suggesting a phyllodes tumor. A partial mastectomy was performed and the pathological study of the surgical piece showed a mesenchymal proliferation with infiltrative borders, consistent with breast fibromatosis.
- Published
- 2009
40. Fibromatosis mamaria, diagnóstico diferencial del carcinoma invasor: Reporte de un caso
- Author
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DÍAZ C,M. LORENA, ARANCIBIA H,PATRICIA, MOYANO S,LEONOR, and BURGOS S,NELSON
- Subjects
Fibromatosis mamaria ,tumor desmoide - Abstract
Introducción: La fibromatosis o tumor desmoide, es una lesión infrecuente del tejido conectivo, que corresponde al 0,2% de los tumores mamarios. No tiene potencial de dar metástasis pero presenta alta tasa de recidiva local debido a sus márgenes infiltrativos. Su etiología es desconocida. La presentación clínica e imagenológica es casi indistinguible a la de un carcinoma mamario. El diagnóstico es histológico y el tratamiento de elección es la resección quirúrgica. Reporte de caso: Mujer de 31 años que consulta por retracción cutánea en mama derecha. No presenta antecedentes quirúrgicos ni de traumas previos. Se realiza ecografía mamaria que muestra, en relación al sitio de retracción, un nodulo irregular que se extiende hacia la piel. La mamografía muestra discreta retracción de la piel y aumento de densidad del tejido subcutáneo adyacente. Se realiza biopsia percutánea CORE bajo ultrasonido, que muestra proliferación estromal fusocelular, sugerente de un tumor filodes. Posteriormente, se realiza mastectomía parcial cuya histología muestra una proliferación mesenquimatosa de bordes infiltrativos, concordante con fibromatosis mamaria. Conclusión: La fibromatosis mamaria es una entidad poco frecuente y localmente agresiva. Su importancia radica en que las características clínicas, radiológicas y citológicas simulan con frecuencia tumores malignos de la mama. El tratamiento de elección es la cirugía, presentando gran tendencia a la recurrencia, incluso con márgenes libres. Se han descrito poco más de 100 casos en la literatura.
- Published
- 2009
41. Mesenteric desmoid tumor mimicking a testicular cancer recurrence
- Author
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Pérez García, Francisco Javier, Pinto Blazquez, Jesús, Rodríguez Martínez, Juan Javier, Gutiérrez García, Ricardo, Jorge Barreiro, José Ignacio, and Velasco Alonso, Julio
- Subjects
body regions ,endocrine system ,endocrine system diseases ,Testicular cancer ,Tumor desmoide ,Cancer testicular ,Desmoid tumor ,urologic and male genital diseases - Abstract
Objetivo: Presentar un caso de un tumor desmoide en un paciente tratado de un seminoma testicular que simulaba una recidiva del tumor testicular. Método: Presentamos el caso de un paciente de 41 años, tratado de un seminoma testicular 26 meses antes, mediante extirpación de una masa testicular retroperitoneal y quimioterapia, que presenta en el seguimiento, una masa abdominal mesentérica que se etiquetó clínicamente de recidiva de seminoma. Resultado: Histológicamente se informa de tumor desmoide mesentérico. Se hace diagnóstico diferencial con un tumor de estroma gastrointestinal mediante el estudio inmunohistoquímico. Conclusiones: El tumor desmoide es un tumor raro. Se han descrito pocos casos en pacientes afectos previamente de tumor testicular. Debe incluirse en el diagnóstico diferencial de las recidivas por tumor testicular. Objective: Report of one case of desmoid tumor in a patient who had been treated of a testicular seminoma 26 months before, with excision of a retroperitoneal mass and chemotherapy. On follow-up he presented with a mesenteric abdominal mass which was clinically labeleled as a recurrence of the seminoma. Results: Histologically it was reported as a mesenteric desmoid tumor. Differential diagnosis with gastrointestinal stromal tumor was performed with immunohistochemical studies. Conclusions: Desmoid tumor is rare. There are few cases reported in patients with history of previous testicular tumor. It should be included in the differential diagnosis of testicular tumor recurrences.
- Published
- 2007
42. Tumor desmoide mesentérico simulando una recidiva de cáncer testicular
- Author
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Pérez García,Francisco Javier and Pinto Blazquez,Jesús
- Subjects
Tumor desmoide ,Cancer testicular - Abstract
Objetivo: Presentar un caso de un tumor desmoide en un paciente tratado de un seminoma testicular que simulaba una recidiva del tumor testicular. Método: Presentamos el caso de un paciente de 41 años, tratado de un seminoma testicular 26 meses antes, mediante extirpación de una masa testicular retroperitoneal y quimioterapia, que presenta en el seguimiento, una masa abdominal mesentérica que se etiquetó clínicamente de recidiva de seminoma. Resultado: Histológicamente se informa de tumor desmoide mesentérico. Se hace diagnóstico diferencial con un tumor de estroma gastrointestinal mediante el estudio inmunohistoquímico. Conclusiones: El tumor desmoide es un tumor raro. Se han descrito pocos casos en pacientes afectos previamente de tumor testicular. Debe incluirse en el diagnóstico diferencial de las recidivas por tumor testicular.
- Published
- 2007
43. Fibromatosis agresiva en un varón adolescente.
- Author
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Corcuera Frutos, Isabela, Muñoa, Adelaida, Badiola, Laura, Bueno, Alejandro, Lobera, Sara, and Otaño, Larraitz
- Abstract
Desmoid tumours are locally aggressive tumours without metastasic potential which generally can be treated with local treatments such as surgery or radiotherapy. Systemic therapy also can be considered. Anyway there is an unpredictable pathology that will need a careful and personal clinic evaluation to choose the most suitable treatment. We report a case of a patient diagnosed of retroperitoneal desmoid tumour. [ABSTRACT FROM AUTHOR]
- Published
- 2003
- Full Text
- View/download PDF
44. [Case report: Rapidly growing abdominal wall giant desmoid tumour during pregnancy].
- Author
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Palacios-Zertuche JT, Cardona-Huerta S, Juárez-García ML, Valdés-Flores E, and Muñoz-Maldonado GE
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- Adult, Disease Progression, Female, Humans, Pregnancy, Time Factors, Abdominal Wall, Fibromatosis, Abdominal pathology, Pregnancy Complications, Neoplastic pathology
- Abstract
Background: Desmoid tumours are one of the rarest tumours worldwide, with an estimated yearly incidence of 2-4 new cases per million people. They are soft tissue monoclonal neoplasms that originate from mesenchymal stem cells. It seems that the hormonal and immunological changes occurring during pregnancy may play a role in the severity and course of the disease., Clinical Case: The case is presented on 28-year-old female in her fifth week of gestation, in whom an abdominal wall tumour was found attached to left adnexa and uterus while performing a prenatal ultrasound. The patient was followed up under clinical and ultrasonographic surveillance. When she presented with abnormal uterine activity at 38.2 weeks of gestation, she was admitted and obstetrics decided to perform a caesarean section. Tumour biopsy was taken during the procedure. Histopathology reported a desmoid fibromatosis. A contrast enhanced abdominal computed tomography scan was performed, showing a tumour of 26×20.5×18cm, with well-defined borders in contact with the uterus, left adnexa, bladder and abdominal wall, with no evidence of infiltration to adjacent structures. A laparotomy, with tumour resection, hysterectomy and left salpingo-oophorectomy, components separation techniques, polypropylene mesh insertion, and drainage was performed. The final histopathology report was desmoid fibromatosis. There is no evidence of recurrence after 6 months follow-up., Conclusions: Desmoid tumours are locally aggressive and surgical resection with clear margins is the basis for the treatment of this disease, using radiotherapy, chemotherapy and hormone therapy as an adjunct in the treatment., (Copyright © 2016 Academia Mexicana de Cirugía A.C. Publicado por Masson Doyma México S.A. All rights reserved.)
- Published
- 2017
- Full Text
- View/download PDF
45. Fibromatosis agresiva en un varón adolescente
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de Corcuera Frutos, Isabela Díaz, Muñoa, Adelaida Lacasta, Badiola, Laura Basterretxea, Bueno, Alejandro Martínez, Lobera, Sara Arévalo, and Otaño, Larraitz Egaña
- Published
- 2003
- Full Text
- View/download PDF
46. [Intraabdominal desmoid tumors in familial adenomatous polyposis].
- Author
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Galletto P, Leoz ML, Castells A, and Balaguer F
- Subjects
- Adult, Age of Onset, Anti-Inflammatory Agents, Non-Steroidal therapeutic use, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Disease Progression, Female, Genes, APC, Humans, Male, Mutation, Missense, Neoplasm Staging, Point Mutation, Prognosis, Risk Factors, Sex Distribution, Watchful Waiting, Wnt Signaling Pathway, beta Catenin genetics, beta Catenin physiology, Abdominal Neoplasms diagnosis, Abdominal Neoplasms epidemiology, Abdominal Neoplasms genetics, Abdominal Neoplasms pathology, Abdominal Neoplasms therapy, Adenomatous Polyposis Coli diagnosis, Adenomatous Polyposis Coli epidemiology, Adenomatous Polyposis Coli genetics, Adenomatous Polyposis Coli pathology, Adenomatous Polyposis Coli therapy, Fibromatosis, Aggressive diagnosis, Fibromatosis, Aggressive epidemiology, Fibromatosis, Aggressive genetics, Fibromatosis, Aggressive pathology, Fibromatosis, Aggressive therapy
- Abstract
Desmoid tumors are currently the main cause of morbidity and mortality in patients with familial adenomatous polyposis. More than 10% of these patients will develop these tumors during their lifetime and more than a third will suffer their consequences. The main risk factors for their development are female sex and abdominal surgery. The most frequent localization is intraabdominal. The therapeutic approach to these tumors has changed, and the surgical treatment of choice is currently the subject of debate. If a watch and wait approach is adopted, more than 50% of tumors will prove to be indolent. Therefore, the therapeutic strategy should be based on clinical presentation and should be decided by a multidisciplinary team working in a center with experience of these tumors. The present article proposes a prognostic classification to guide the therapeutic approach., (Copyright © 2012 Elsevier España, S.L. and AEEH y AEG. All rights reserved.)
- Published
- 2013
- Full Text
- View/download PDF
47. [Fibromatosis breast in the male. Case study].
- Author
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Muñoz-Atienza V, Manzanares-Campillo Mdel C, Sánchez-García S, Pardo-García R, and Martín-Fernández J
- Subjects
- Biomarkers, Tumor analysis, Breast Neoplasms, Male pathology, Breast Neoplasms, Male surgery, Fibroma chemistry, Fibroma diagnostic imaging, Fibroma pathology, Fibroma surgery, Humans, Male, Middle Aged, Prognosis, Remission Induction, Ultrasonography, Breast Neoplasms, Male diagnosis, Fibroma diagnosis, Mastectomy, Subcutaneous
- Abstract
Background: mammary fibromatosis is a rare pathology. It constitutes 0.2% of breast cancers, and case in men are exceptional. The definitive diagnosis is histological., Clinical Case: we report the case of a male of 52 years, diagnosed with breast fibromatosis after pathologic study of tumor in the right breast. Programmed surgery for excision with wide margins was done. We performed a mastectomy of the subcutaneous fibromatosis with a pathologic study with clear margins. The postoperative course was uncomplicated and did not require adjuvant therapy. At 6 months follow-up he remains free of disease. The treatment of choice is surgical excision with wide margins. Adjuvant treatment is controversial., Conclusions: the fibromatosis in the breast is very rare and an exceptional occurrence in men. Surgery is the definitive treatment; few results exist for adjuvant therapy.
- Published
- 2013
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