Cases reported "Fibromatosis, Abdominal"

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1/55. An aggressive desmoid tumor in a patient with familial adenomatous polyposis: immunohistochemical findings.

    A case of an aggressive desmoid tumor in a patient with familial adenomatous polyposis is described. The lesion rapidlyenlarged with compression of adjacent structures including the ureter and small bowel, and the patient died because of small bowel perforation and hydronephrosis 3 years after detection of small desmoid tumors at the time of a prophylactic coloproctectomy for a colon carcinoma. Immunohistochemically, proliferating cell nuclear antigen (PCNA), p21WAF1/CIP1 and cathepsin d indices, but not the bcl-2 index, which were defined as the numbers of immunoreactive tumor cells per 1000 tumor cells, increased in line with tumor progression. The tumor did not show staining for collagen IV, but was characterized by intense staining for basic fibroblast growth factor (bFGF). Accordingly, tumor aggression was related to increases in both cell proliferation and protease activity, as well as an enhanced expression of bFGF. In addition, the desmoid tumor showed deregulation between PCNA and p21WAF1/CIP1 because the normal inverse relation between these two was not apparent. ( info)

2/55. Desmoid tumour. The risk of recurrent or new disease with subsequent pregnancy: a case report.

    Desmoid tumours are rare, benign tumours arising from fibrous tissue in muscle fascia or aponeurosis. They are most common in women of child-bearing age and most often appear during or after pregnancy in this age group. The recommended treatment is wide surgical excision, if possible, but unresectable tumours may be treated with radiotherapy, anticancer drugs, nonsteroidal anti-inflammatory agents or antiestrogenic compounds. The recurrence rate is high and seems to be related to the achievement of resection margins free of tumour. The literature is not specific about how to counsel women who have had a desmoid tumour and subsequently wish to have a child. patients should be advised that these tumours may be estrogen sensitive but subsequent pregnancy is not necessarily a risk factor for recurrence or development of new disease. ( info)

3/55. Mesenteric fibromatosis: case report and literature review.

    Mesenteric fibromatosis is a rare, benign fibrous lesion found in the bowel mesentery or the retroperitoneum. Its biological behavior is intermediate between benign fibrous tissue proliferation and fibrosarcoma. Fibromatosis characteristically is locally invasive and tends to recur but does not metastasize. Most reported cases have been in older individuals, and there is a frequent association with familial polyposis coli, previous trauma, and hormonal imbalance. The authors report a case of mesenteric fibromatosis in a 32-month-old girl with a 1-month history of abdominal pain who was discovered to have an abdominal mass. After appropriate investigations, the mass was excised. The pathology report confirmed the diagnosis of fibromatosis. Mesenteric fibromatosis in children, as in adults, presents a management challenge for the surgeon. ( info)

4/55. An abdominal desmoid tumor involving the xyphoid and costal chondrium associated with pregnancy: report of a case.

    We report herein the case of a 27-year-old woman found to have an abdominal desmoid tumor involving the xyphoid and costal chondrium associated with pregnancy. The patient was referred to our department on the third day after delivery for investigation of a fist-sized, firm tumor in the infrasternal angle, first noticed during the eighth month of gestation. Computed tomography and magnetic resonance imaging revealed a well-defined mass, 9 x 7.5 cm in size, invading the xyphoid. The tumor could be radically excised with the xyphoid, a lower part of sternum, and the chondrium of the bilateral sixth and seventh ribs, including 1.5 cm of free margin. The resected specimen measured 10 x 11.5 x 11.5 cm and weighed 395 g. Histological examination revealed the lesion to be a desmoid tumor consisting of spindle cells with small, elongated nuclei infiltrating dense collagenous fibers. The patient had an uneventful postoperative course and was discharged on the 11th postoperative day. There has been no evidence of recurrence in the 6 months since her operation. This case is of interest with regard to the possible contribution of sex hormones, especially estrogen, to desmoid tumor development. ( info)

5/55. Mediastinal fibromatosis presenting with superior vena cava syndrome.

    We encountered a fatal case of mediastinal fibromatosis in a 67-year-old female in whom there was aggressive infiltration into the large vessels, nerves and pericardium. She presented with the superior vena cava syndrome, Horner's syndrome, paralysis of bilateral vocal cords and diaphragm and heart failure. Mediastinoscopical examination revealed an extremely firm tumor adhering to the sternum, trachea and brachiocephalic artery. She died of severe heart failure due to the disturbed dilatation of the heart and ventilatory insufficiency. Although mediastinal fibromatosis is very uncommon and sometimes difficult to diagnose at an early stage, physicians should be aware of this disease for the differential diagnosis of mediastinal tumors. ( info)

6/55. Treatment of mesenteric desmoid tumours with the anti-oestrogenic agent toremifene: case histories and an overview of the literature.

    Desmoid tumours are histologically benign but due to their infiltration and compression of surrounding structures potentially life-threatening fibromatous lesions of unknown aetiology. The annual incidence rate is 2-4 per million people. The mesenteric variant constitutes about 10% of all desmoid tumours, although in familial adenomatous polyposis (FAP) patients this may be up to 70%. Due to the small number of patients with mesenteric desmoids the therapy is mainly empirical. This report describes the rationale as well as the value of the short- and long-term treatment (up to 6 years) with the anti-oestrogenic agent toremifene in combination with sulindac in two patients suffering from such a mesenteric desmoid tumour. These patients did not respond to sulindac alone and previous treatment with tamoxifen together with this non-steroidal anti-inflammatory drug had also failed. An overview of the literature on the management of these dismal tumours is presented. ( info)

7/55. Recurrent mesenteric desmoid tumors with multiple peritoneal dissemination: a case report and review of desmoid in japan.

    We report, herein, on the first case of a mesenteric desmoid tumor with multiple peritoneal dissemination. A 73 year-old Japanese woman, who had a history of uterine cancer that was treated with hysterectomy followed by a high dose of irradiation 25 years ago, had an unknown stenosis of the sigmoid colon, which was treated with partial resection of the stenosed colon 6 years ago, and then resulted in multiple small bowel obstructions due to the recurrence of mesenteric desmoids. The clinical behavior of this tumor is considered to be unpredictable. We emphasize that mesenteric desmoid tumors should be considered as one of the causes of stenosis of the colon and small bowel, and patients should receive careful follow-up after unknown stenosis. ( info)

8/55. Successful treatment of recurrent pelvic desmoid tumour with tamoxifen: case report.

    The case report of a young woman with recurrent pelvic desmoid tumour successfully treated with tamoxifen is described. The desmoid tumour recurred within 6 months after the initial exploratory laparotomy. tamoxifen therapy led to complete relief of ascites within 2 months and complete tumour regression by the end of the fourth month, and the patient has remained stable for 6 years. Without sacrificing pelvic organs or major vessels and preserving reproductive ability, tamoxifen should be considered as the first drug of choice in such a recurrent condition. ( info)

9/55. A case of Turner's syndrome complicated with desmoid tumor of the transverse colon.

    The association of Turner's syndrome and endometrial carcinoma has been previously established, but has never been described in conjunction with a desmoid tumor of the colon. A case is described of a colonic desmoid tumor developing in a 38-year-old female with Turner's syndrome. The association has not been previously reported. In this report, we describe a 38-year-old woman who has Turner's syndrome with a colonic desmoid and review the literature. ( info)

10/55. Current status of small bowel transplantation: a case study of transplant between identical twins.

    Small bowel transplantation is coming of age. Although still considered an experimental procedure, much has been accomplished to bring this surgery closer to routine. This article describes the current status of small bowel transplantation and discusses the first successful small bowel transplantation between identical twins. The issues of workup, surgery, postoperative follow-up, common complications, and the history and future of small bowel transplantation are addressed. ( info)
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