Fibromatosis del recto anterior del abdomen. Reporte de caso / Fibromatosis of the rectus abdominis. Case report

La fibromatosis es un tumor fibroblástico benigno, poco frecuente. El músculo recto anterior del abdomen es uno de los músculos de la pared abdominal más habitualmente afectado. Es un tumor con una alta frecuencia en la invasión local y recurrencia. La marcación para ß-catenina en el inmunofenotipo certifica el diagnóstico. Los márgenes negativos microscópicos (R0) deben ser el objetivo de la resección quirúrgica. La edad joven, el gran tamaño tumoral y los márgenes positivos son parámetros asociados a la recurrencia local. Presentamos el caso clínico de una paciente joven a la cual en el contexto de una abdominoplastia se le diagnostica una tumoración en el sector medio del recto anterior del abdomen izquierdo. Se hizo una biopsia incisional que certificó que correspondía a una fibromatosis abdominal (FA). Confirmado el diagnóstico se realizó resección de la tumoración y reparación de la pared abdominal con malla de polipropileno.

Fibromatosis is a rare, benign fibroblastic tumor. The rectus abdominis muscle is one of the most commonly affected abdominal wall muscles. It is a tumor with a high frequency of local invasion and recurrence. Marking for ß-catenin in the immunophenotype certifies the diagnosis. Microscopic negative margins (R0) should be the target of surgical resection. Young age, large tumor size, and positive margins are parameters associated with local recurrence. We present the clinical case of a young patient who, in the context of an abdominoplasty, was diagnosed with a tumor in the middle sector of the left rectus abdominis. An incisional biopsy was performed, which certified that it corresponded to abdominal fibromatosis (AF). Once the diagnosis was confirmed, the tumor was resected and the abdominal wall repaired with polypropylene mesh

Fibromatosis mesentérica solitaria. Reporte de caso y revisión de la literatura / Sporadic mesenteric fibromatosis. A case report. Literature review / Fibromatose mesentérica solitária. Relato de caso. Revisão da literatura

Resumen: 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.

Summary: Mesenteric fibromatosis is a deep sub-type of desmoid tumors consisting of a benign tumor of fibroblastic origin which is locally aggressive given its tendency to infiltrate adjacent tissues. They are unusual and sporadic, and may be associated to Gardner's Syndrome. Complete resection is the treatment of choice, avoiding local recurrence. The study reports the clinical case of a patient with intra-abdominal sporadic mesenteric fibromatosis, well circumscribed that appeared to be a pelvic mass in MR imaging.

Resumo: A fibromatose mesentérica é um subtipo profundo de tumor desmóide (DT); é um tumor benigno de origem fibroblástica que é localmente agressivo devido à sua tendência a infiltrar tecidos adjacentes. São raros, esporádicos e podem estar associados à síndrome de Gardner. O tratamento de escolha é a ressecção completa, evitando recidiva local. Relatamos o caso clínico de uma paciente com fibromatose mesentérica intra-abdominal única e bem circunscrita que simulava uma massa de origem pélvica na imagem.

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)

Fibromatosis intraabdominal mesentérica de identificación postmortem. Reporte de caso / Post mortem identification of Intra abdominal Mesenteric Fibromatosis. Case report

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...(AU)

Laparoscopic Excision of Anterior Abdominal Wall Tumors: A Case of Desmoid-Type Fibromatosis Arising in the Rectus Muscle

PURPOSE: Laparoscopic approaches to the anterior abdominal wall are difficult because of the inherent limitations of laparoscopic surgery. METHODS: A 25-year-old young female visited to our hospital with an incidentally-found palpable abdominal mass. Computed tomography revealed a 7-cm round enhancing mass arising from the left posterior rectus sheath. The patient underwent laparoscopic excision of the inner mass arising from the anterior abdominal wall. RESULTS: The operation lasted for 45 minutes and there was no measurable bleeding during the procedure. Pathological assessment revealed that the tumor was a DTF 7.0 cm in size. The patient was discharged on the second postoperative day in good condition, and is currently being followed on a routine basis for surveillance without adjuvant therapy. CONCLUSION: Laparoscopic approach for the anterior abdominal wall tumor could be feasible in select patients. This minimally-invasive approach helps to ensure good cosmetic outcomes and quality of life.

Pediatric fibromatosis involving mandible: case reportand a five-year post-operative follow-up / Fibromatose pediátrica em mandíbula: relato de caso e avaliação pós-operatória de cinco anos

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.

Abdominal Fibromatosis in a Young Child: A Case Study and Review of the Literature

Fibromatoses comprise many different entities of well-differentiated fibroblastic proliferation with variable collagen production and form a firm nodular mass. Abdominal fibromatosis is distinguishable from other forms of fibromatosis because of its location and its tendency to occur in women of childbearing age during or following pregnancy. Abdominal fibromatosis in children is an extremely rare condition. A 15-month-old boy presented with an abdominal wall mass that had recently increased in size. Mass excision was perfomed. The tumor was 4.3x4.1 cm and partly circumscribed. Histologically, the tumor was composed of parallel long fascicles of spindle-cells with a uniform appearance. The edges of the resected mass were infiltrative, and the surgical margins were positive. Mitotic figures were <1/10 high power fields. No cellular atypia or necrosis was present. The tumor cells were positive for vimentin and nuclear beta-catenin staining.

Plexiform angiomyxoid myofibroblastic tumor of stomach / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunophenotype and differential diagnosis of plexiform angiomyxoid myofibroblastic tumor (PAMT) of the stomach.</p><p><b>METHODS</b>The clinical and pathologic findings of 3 cases of PAMT in the gastric antrum were retrospectively analyzed. Immunohistochemical study was carried out and the literature was reviewed.</p><p><b>RESULTS</b>The age of patients ranged from 31 to 47 years. The male-to-female ratio was 1:2. The clinical presentation included epigastric pain and distension. Endoscopically, the tumor mass protruded into the gastric cavity at the antrum and ranged from 4.5 cm to 8.0 cm in greatest dimension. One of the tumors studied was associated with surface ulceration. Histologically, the tumors were located in the gastric wall. They were composed of bland spindle cells and small vessels arranged in a plexiform or nodular pattern within a myxoid stroma. Immunohistochemical study showed that the spindle cells were consistently positive for smooth muscle actin and muscle-specific actin. There was focal staining for h-caldesmon, desmin in case 3 and focal positive for epithelial membrane antigen, CAM5.2 in case 1. Further, CD10 and progesterone receptor were positive in case 3.</p><p><b>CONCLUSIONS</b>PAMT represents a rare novel mesenchymal tumor of the stomach, with a propensity of gastric antral involvement. The distinctive pathologic features help to differentiate this entity from other benign and malignant tumors.</p>

Desmoid tumour of lower abdomen

A desmoid tumour is slow growing fibromatosis with aggressive infiltration of adjacent tissue and extremely unusual systemic metastases. We report a case of a female patient aged 25 years who had a mass in lower abdomen for 2 years. There was no previous history of any surgical intervention. Preoperative evaluation included ultrasound and computed tomography. Patient underwent primary resection with wide margins. Histology revealed a desmoid tumour

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

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

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

Mesenteric Fibromatosis Mimicking Recurrence after Distal Gastrectomy for Gastric Cancer

Mesenteric fibromatosis is a monoclonal, fibroblastic proliferation arising from musculoaponeurotic structure, and it is distinctive lesions defined as a group of non-metastasizing fibroblastic tumors which has local invasion and has a high recurrence rate after the surgical excision. The main treatment modality is the surgical excision. Radiation therapy, chemotherapy, and hormone therapy are also known as useful treatments. We report our experience of a recent case of Mesenteric fibromatosis. A 62-year old female patient had undergone gastrectomy due to gastric cancer. 18 months after gastrectomy, we detected an abdominal mass. The preoperative radiologic findings were suggestive of recurrence. Exploratory laparotomy was performed and post-operative pathologic diagnosis was confirmed as fibromatosis. We report a patient with mesenteric fibromatosis that mimic recurrence after gastrectomy for gastric cancer.

Prolapso genital en la Maternidad “Concepción Palacios” / Genital prolapse in Maternidad

Evaluar la prevalencia del prolapso genital, factores relacionados, tratamiento y complicaciones en pacientes hospitalizadas durante el año 2003. Estudio descriptivo, retrospectivo, epidemiológico. Se revisaron los libros de admisiones desde el 01/01/2003 hasta 31/12/2003, seleccionando las pacientes con alteraciones del piso pélvico. Se evaluaron los factores asociados: edad, embarazos, partos, incontinencia urinaria de esfuerzo (IUE), fibromatosis uterina, intervenciones ginecológicas previas. Se clasificaron los tipos y grados de prolapsos, tratamientos y complicaciones. De 21 144 ingresos para 2003, se seleccionaron 2 616 con edad ≥ 35 años, de las cuales 423 fueron casos ginecológicos con patología quirúrgica y sólo 47 (11 por ciento) presentaron prolapso. El promedio de edad fue 56,65 ± 11,5 años, el 93 por ciento refirió síntomas de peso, tumor vaginal o incontinencia urinaria. El promedio de embarazos 5,7 ± 3,2 y de partos 4,9 ±2,6. Los factores asociados más frecuentes fueron la fibromatosis uterina en 11 por ciento y la histerectomía abdominal previa en 8,5 por ciento. El tipo de prolapso más frecuente fue el histerocele (51 por ciento), cistocele II y III º + rectocele (36 por ciento) y de cúpula vaginal (11 por ciento). La intervención quirúrgica más realizada fue histerectomía vaginal, en menor número la histerectomía abdominal, colposuspención de Burch, sacropexia y colpoperineoplastia. Hubo 14 por ciento de complicaciones intraoperatorias. La frecuencia de hospitalizaciones para corrección de prolapso genital es muy baja, el histerocele avanzado sintomático es el más frecuente, son pacientes de edad avanzada, multíparas, los factores relacionados la fibromatosis uterina y la histerectomía abdominal previa. La intervención más realizada la histerectomía vaginal y el porcentaje de complicaciones intraoperatorias fue importante

To assess the prevalence of genital prolapse, related factors, treatment and complications in patients hospitalized during 2003. Descriptive, retrospective, epidemiologic. We reviewed the books of admissions from 01/01/2003 until 31/12/2003, by selecting patients with pelvic floor disorders. We evaluated the factors associated with age, pregnancy, childbirth, urinary incontinence, uterine fibromatosis, prior gynecological interventions. Ranked the types and degrees of prolapse, treatments and complications. Of 21 144 income for 2003 was 2 616 identified with age ≥ 35 years, of which 423 were cases with gynecologic surgical pathology, and only 47 (11 percent) had prolapse. The average age was 56.65 ± 11.5 years, 93 percent said weight symptoms, urinary incontinence or vaginal tumor. The average pregnancy 5.7 ± 3.2 and 4.9 ± 2.6 births. The associated factors were the most frequent uterine fibromatosis in 11 percent after abdominal hysterectomy and 8.5 percent. The most common type of prolapse was histerocele (51 percent), II and III cystocele rectocele II º (36 percent) and vaginal vault (11 percent). The surgery was performed more vaginal hysterectomy, fewer abdominal hysterectomy, Burch colposuspencion, sacropexia and colpoperineoplastia. There were 14 percent of intraoperative complications. The frequency of hospitalization for correction of genital prolapse is very low, the advanced histerocele is the most common symptoms are the elderly, multiparous, related factors and the uterine fibromatosis after abdominal hysterectomy. The most accomplished vaginal hysterectomy and the rate of intraoperative complications was significant

Tumor desmoide abdômino-pélvico / Abdominopelvic desmoid tumor

O tumor desmoide é uma neoplasia não capsulada, localmente agressiva, originária dos fibroblastos dos tecidos músculo-aponeuróticos. Embora ele não tenha comportamento maligno, tal como capacidade de gerar metástases ou de invasão, o tumor desmoide tem uma alta capacidade de crescimento local, causando deformidades nos órgãos adjacentes, dor e, eventualmente, disfunção orgânica, dependendo da área envolvida. Relatamos o caso de um tumor desmoide intra-abdominal de grandes proporções, invadindo órgãos pélvicos em uma paciente de 53 anos. A neoplasia foi totalmente extirpada e, atualmente, cinco anos após a cirurgia, a paciente encontra-se sem sinal clínico ou radiológico de recidiva da lesão.

Desmoid tumor is a noncapsulated neoplasia, locally aggressive, originated from the fibroblasts of the musculo-aponeurotic tissues. Even though with no malignant behavior, such as the ability of generating metastasis or of invasion, the desmoid tumor has a high tendency for local growth, causing deformities in the adjacent organs, pain and sometimes organ dysfunction, depending on the area involved. We report the case of a large intra-abdominal desmoid tumor, invading pelvic organs in a 53 year-old patient. The neoplasia has been totally extirpated and, at the moment, five years after the surgery, the patient presents no clinical or radiologic sign of lesion relapse.

Masas mesentericas: evaluación por tomografia computada / Mesenteric masses: computed tomography assessment

The mesentery is a frequent site for abdominal diseases, since it constitutes the intestinal scaffolding and pathway for many structures. Nowadays, computed tomography (CT) is the imaging modality of choice for evaluating mesenteric masses and their origin. An isolated mesenteric mass (primary) is an unexpected and rare tomographic finding in the context of patients with nonspecific clinical signs. The identification of a primary mesenteric mass requires an effort to exelude the possibility of being a secondary source. Thus, knowledge of the spectrum of mesenteric disease (neoplastic, infectious, inflammatory, vascular, traumatic, congenital) and its computed tomographic appearance is the key for proper diagnosis and treatment.

El mesenterio es un sitio frecuente de enfermedades abdominales, ya que constituye el soporte intestinal y una vía de paso para muchas estructuras. Hoy en día, la tomografía computada (TC) es la modalidad de imagen de elección para evaluar las masas mesentéricas y su origen. Una masa aislada (primaria) es un hallazgo tomográfico raro e inesperado de encontrar en el contexto de pacientes con signos clínicos inespecíficos. La identificación de una masa mesentérica primaria requiere un esfuerzo para descartar la posibilidad de un origen secundario. Así pues, el conocimiento del espectro de la enfermedad mesentérica (neoplásicas, infecciosas, inflamatorias, vasculares, traumáticas y congénitas procesos) y su apariencia tomográfica es clave para el diagnóstico y el tratamiento adecuado.

Intrathoracic Desmoid Tumor Mimicking Pleural Mass: A Case Report / 결핵및호흡기질환

Desmoid tumor (fibromatosis) is a histologically benign fibrous neoplasm showing locally infiltrating growth. This type of tumor commonly occurs in the abdomen, but intrathoracic desmoid tumor is uncommon. To date, 12 cases of intrathoracic desmoid tumor protruding into the pleural cavity, radiologically mimicking pleural masses, have been reported. Here, we report on a case of intrathoracic desmoid tumor protruding into the pleural cavity, and partially covered by parietal pleura. The main preoperative differential diagnoses included pleural solitary fibrous tumor, inflammatory pseudotumor or malignant mesothelioma. A near-total mass excision was performed. Pathologically, the tumor was composed of a paucicellular arrangement of spindle-shaped cells with fibromyxoid stroma. The resection margin was partially involved with spindle cells present. On histochemical staining, the spindle cells were strongly positive for vimentin and negative for CD34, consistent with a desmoid tumor. The patient was stable without further adjuvant treatment during 6-years of follow-up.