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.

Fibromatosis mesentérica: reporte de un caso / Mesenteric fibromatosis: report of one case

El Tumor Desmolde o Fibromatosis Mesentérica es una enfermedad infrecuente en la que se reconocen 2 formas: una esporádica y otra asociada a poliposis familiar adenomatosa. De etiología desconocida, su diagnóstico preoperatorio es dificultoso y el tratamiento de elección es quirúrgico. Se pone en consideración un caso de esta entidad en un varón joven, que se presentó con dolor abdominal y fiebre. Se realizó cirugía resectiva agresiva, otorgándose el alta a los 18 días de la intervención.

Desmoid tumor or mesenteric fibromatosis is an uncommon disease with to variants: a sporadic form and one associated to familial adenomatous polyposis. Surgery is the treatment of choice. We report a 45 years old male presenting with abdominal pain and fever. An abdominal ultrasound informed the presence of a mass in the periumblical area. An Abdominal CAT sean showed a 13 cm diameter solid mass located in the left upper quadrant. The patient was operated and that mass is excised, requiring also a hemicolectomy, excision of the fourth portion of the duodenum and cholecystectomy. The pathology report of the excised tumor informs a mesenteric fibromatosis. The patient was discharged at the 18th postoperative day.

Fibromiomatosis uterina gigante: reporte de un caso / Giant uterine fibromyomatosis: case report

Comunicar el caso clínico de una paciente joven, con diagnóstico de fibromiomatosis uterina gigante, quien fue resuelta quirúrgicamente en forma satisfactoria. Se describe el caso, la técnica quirúrgica empleada y los hallazgos transoperatorios observados. Hospital Universitario “Dr. Luis Razetti”. Barcelona, Estado Anzoátegui. Se realizó histerectomía abdominal total, salpingectomía bilateral y ooforectomía derecha. El peso de la pieza quirúrgica fue de 13 kg. Durante el acto operatorio, se diagnosticó una lesión incidental de la vejiga, la cual fue reparada exitosamente. La evolución posoperatoria fue satisfactoria y la paciente manifestó un alto grado de satisfacción con el resultado quirúrgico. La fibromiomatosis uterina gigante es una entidad patológica benigna de muy escasa incidencia. Su resolución representa un reto para el equipo quirúrgico debido al gran volumen de la pieza quirúrgica y a las variaciones en la distribución de los órganos intraabdominales, originadas por el recimiento uterino.

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.