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)

The imaging findings of CT and MRI in patients with abdominal and pelvic solitary fibrous tumor and its pathological features / 中国基层医药

Objective To investigate the imaging findings of CT and MRI in patients with abdominal and pelvic solitary fibrous tumor and its pathological features.Methods 30 patients with abdominal and pelvic solitary fibrous tumor were chosen.Patients with CT and MRI,and pathologic features control study were analyzed.Results In 30 patients,mesenchymal tumors,malignant fibrous histiocytoma and ovarian fibroma patients were 12 cases,12 cases, 6 cases.CT showed that mass state clearly,smooth contours,the substance of the composition of two different densi-ties,increase significantly enhanced scan,saw a number of tumor blood vessels within the shadow arterial aneurysm. MRI showed that internal tumors were low signal stripes appear.Tumor necrosis internal punctate liquefaction zone, after enhanced scan,the patient did not appear to strengthen.6 patients had resection specimens.4 cases had a more complete capsule,two cases with incomplete capsule.Conclusion SFF has certain characteristics in CT and MRI imaging screening,combined with its pathological features analysis will help improve the diagnosis.

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.

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.

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.

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.