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

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)

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.

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.

[Abdominal desmoid tumor pathologic and therapeutic concepts]

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

Fibromatosis mesentérica: caso clínico-imagenológico y revisión bibliográfica / Retroperitoneal fibromatosis: Report of one case

We report a 42 years old female that presented with abdominal pain and no palpable mass. Imaging abdominal ultrasound, CAT scan and magnetic resonance showed a solid tumor located in the retroperitoneum. The patient was operated on excising the tumor. Pathological examination of the surgical piece disclosed a retroperitoneal fibromatosis or desmoid tumor. Postoperative evolution of the patient was uneventful and she was discharged 12 days after the surgical procedure.

Tumor desmóide intraperitoneal / Intraperitoneal fibromatosis

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 macarado, apenas laxamente aderido às vísceras circunjacentes. Na formulaçäo do diagnóstico diferencial os autores sugerem que sua eventual ocorrência deve ser lembrada, marcadamente na vigência de situaçöes clínicas similares.