Cases reported "Fibromatosis, Abdominal"

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1/13. Diffuse intra-abdominal fibromatosis-report of a new entity with review of literature.

    Fibromatosis, arare non-neoplastic spindle cell proliferation of unknown aetiology, can occur anywhere in the body. Though extra-abdominal sites are commonly involved, intra-abdominal fibromatosis has also been described. Described herein is an unusual case of diffuse intra-abdominal fibromatosis in a 9-year-old boy, who could not be salvaged despite extensive medical management.
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2/13. Familial polyposis coli: clinical manifestations, evaluation, management and treatment.

    Familial adenomatous polyposis (FAP) is an autosomal dominant, hereditary colon cancer syndrome that is characterized by the presence of innumerable adenomatous polyps in the colon and rectum. Gardner's syndrome is a variant of FAP, which in addition to the colonic polyps, also presents extracolonic manifestations, including desmoid tumors, osteomas, epidermoid cysts, various soft tissue tumors, and a predisposition to thyroid and periampullary cancers. Mutations of the APC gene are thought to be responsible for the development of FAP, and the location of the mutation on the gene is thought to influence the nature of the extracolonic manifestations that a given patient might develop. Though patients are often asymptomatic, bleeding, diarrhea, abdominal pain and mucous discharge frequently occur. Diagnostic tools include genetic testing, endoscopy, and monitoring for extra-intestinal manifestations. Currently, surgery is the only effective means of preventing progression to colorectal carcinoma. Restorative proctocolectomy with ileal pouch anal anastomosis (RPC/IPAA) with mucosectomy is the preferred surgical procedure, since it attempts to eliminate all colorectal mucosa without the need for an ostomy. Periampullary carcinoma and intra-abdominal desmoid tumors are a significant cause of morbidity and mortality in these patients after colectomy. Frequent endoscopy is needed to prevent the former, while there is no definitive treatment available yet for the latter. The following article presents a case and reviews the evaluation, management and treatment of Gardner's syndrome.
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keywords = intra-abdominal
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3/13. Unresectable desmoid tumours causing obstructive uropathy in familial adenomatous polyposis.

    Desmoid tumour-related ureteral obstruction in familial adenomatous polyposis presents difficult management problems. Its role in altering prognosis for the worse in young patients has been neglected. Two patients are reported and 14 cases in the literature reviewed. Both our patients developed intra-abdominal desmoid tumours following proctocolectomies with pouch reconstruction (performed 0.7 and 2.5 years earlier). Despite therapy with sulindac and tamoxifen, both suffered progressive ureteral obstruction that required initial percutaneous drainage. Ureteral stenting was effective and safe in both patients (10 and 18 months' follow-up, respectively), but the long-term outcome awaits assessment. The optimal regimen of adjuvant medical therapy is uncertain. Successful renal autotransplantation and combination chemotherapy have been tried after failure of conventional therapy.
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4/13. Localized intra-abdominal fibromatosis of the small bowel mimicking a gastrointestinal stromal tumor: a case report.

    Intra-abdominal fibromatosis (IAF) is a benign mesenchymal lesion that can occur throughout the gastrointestinal tract. Although rare, it is the most common primary tumor of the mesentery and can develop at any age. We describe a rare case of primary IAF involving the mesentery and small bowel which clinically, macroscopically and histologically mimicked malignant gastrointestinal stromal tumor (GIST). This report highlights the fact that benign IAF can be misdiagnosed as a malignant GIST localized in the mesentery or arising from the intestinal wall. Their diagnostic discrimination is essential because of their very different biological behaviors and the fact that the introduction of effective therapies involving tyrosine kinase inhibitor STI571 (imatinib mesylate) has greatly changed the clinical approach to intra-abdominal stromal spindle cell tumors.
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keywords = intra-abdominal
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5/13. Aggressive intra-abdominal fibromatosis in children and response to chemotherapy.

    Intra-abdominal fibromatosis (IAF) is a rare benign neoplasm arising from the abdominal fibrous tissue, mostly in the mesentery. IAF is characterized by a tendency to infiltrate the surrounding vessels and vital structures and recurrence after usually incomplete surgical removal. Accordingly, IAF is associated with considerable morbidity and mortality. The authors report on a boy who presented with a large IAF at the age of 5 years. Within 6 months after initial presentation, he underwent 4 subsequent abdominal explorations for diagnosis, tumor reduction, and intestinal obstructions. IAF was confirmed by the presence of vimentin and absence of other biological cell markers. Due to accelerated tumor growth and deteriorated general condition, as a last resort, a chemotherapy trial with vincristin and methotrexate was carried out. This regimen proved to be effective in reducing the tumor burden and improving the patient's general condition. Outcome of IAF depends on early diagnosis and complete tumor resection, and, if indicated, timely employment of neo/adjuvant chemotherapy. radiotherapy must be considered in life-threatening conditions as the last resort in a growing child [2-4].
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keywords = intra-abdominal
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6/13. Mesenteric fibromatosis successfully resected with duodeno-jejunectomy and nephrectomy.

    We report a rare case of intra-abdominal fibromatosis of the jejunal mesentery. A 71-year-old man, who was incidentally diagnosed with left hydronephrosis, was found to have a tumor near the duodeno-jejunal junction. The tumor appeared to invade the intestinal wall and was obstructing the left ureter. The tumor was indefinite in diagnosis preoperatively. The patient underwent an en-bloc excision of the tumor by a partial duodeno-jejunectomy combined with a left nephrectomy. Microscopic examination of the tumor showed that non-dysplastic fibroblasts proliferating in the jejunal mesentery had infiltrated into the adjacent small intestines and ureter, resulting in a diagnosis of intra-abdominal fibromatosis. This diagnosis was supported by findings of immunohistochemical analyses showing positive staining for vimentin and smooth muscle actin and negativity for keratin, CD34, C-kit and S-100. To our knowledge, this is the first documented case of intra-abdominal fibromatosis of the jejunal mesentery completely resected with the operative procedure described herein. In cases of a preoperatively undiagnosed retroperitoneal or mesenteric tumor that invades adjacent structures, it is important to consider intra-abdominal fibromatosis as a possible differential diagnosis. This may be helpful in planning the appropriate therapeutic strategies including extended multi-organ resection in selected patients.
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keywords = intra-abdominal
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7/13. Inoperable aggressive mesenteric fibromatosis with ureteric fistula. Case report and literature review.

    The purpose of our report is to illustrate an aggressive case of mesenteric fibromatosis in a 17-year-old girl with a ureteric fistula and to review imaging and pathological features, natural history and treatment options of this disease. Our patient underwent computed tomography that revealed a widespread intra-abdominal mass. The necrotic centre of this mass had a fistulous communication with the right ureter. Fibromatoses represent a spectrum of uncommon benign conditions characterised by proliferating fibrous tissue. The deep intra-abdominal form of mesenteric fibromatosis (MF), one of the rare subtypes of the 'fibromatoses' or 'desmoid tumours', grows rapidly and may become extensive. Surgery provides good results in limited disease and non-surgical modalities in cases of unresectable and residual disease.
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keywords = intra-abdominal
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8/13. Diffuse abdominal uptake of technetium-99m-HDP after colectomy in Gardner's syndrome.

    A 37-yr-old man presented with increasing abdominal girth and multiple palpable intra-abdominal masses 3 yr after colectomy for polyposis coli. Whole-body skeletal scintigraphy performed prior to laparotomy demonstrated diffuse abdominal uptake of 99mTc-HDP consistent with mesenteric fibromatosis confirmed at surgery. When diffuse abdominal uptake of skeletal imaging agents occurs in patients with prior colectomy for polyposis coli, mesenteric fibromatosis as a manifestation of Gardner's syndrome should be suspected. This case illustrates another cause of diffuse abdominal uptake of skeletal imaging agents.
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keywords = intra-abdominal
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9/13. abdominal wall desmoid mimicking intra-abdominal mass: MR features.

    A case of abdominal wall desmoid which enlarged toward the liver and mimicked an intra-abdominal tumor is presented. T*2-weighted MR images clearly demonstrated the tumor's continuity with the rectus abdominis muscle. The case presented suggests MRI may provide more valuable information concerning the origin of a right-upper-quadrant mass than CT does. However, the nature of the signals and attachment which this case showed were so unusual for desmoids that this case has not been diagnosed accurately.
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ranking = 0.83333333333333
keywords = intra-abdominal
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10/13. Use of doxorubicin and dacarbazine for the management of unresectable intra-abdominal desmoid tumors in Gardner's syndrome.

    PURPOSE: The aim of this study was to describe the natural history and management of surgically unresectable intra-abdominal desmoid tumors in two patients with Gardner's syndrome from two unrelated families, where each had failed on conventional therapy. methods: Two patients with Gardner's syndrome were placed on a chemotherapy regimen which included doxorubicin (90 mg/m2) and dacarbazine (900 mg/m2) in divided doses over four days of continuous infusion. Their progress on chemotherapy was assessed by abdominal computerized tomography and laparoscopy. RESULTS: The computerized abdominal tomography scans proved difficult to interpret because of adhesions and matted small bowel resulting from the patients original colectomies. These findings made it difficult to differentiate postoperative changes from residual desmoid tumor. Second-look laparotomy in such patients was contraindicated as this may predispose to further desmoid production. laparoscopy disclosed a complete response to this chemotherapy. Nevertheless, we had an iatrogenic small bowel perforation in one of these patients. Each patient showed a complete response to chemotherapy. CONCLUSION: Surgical resection remains the first-line treatment of intraabdominal desmoid tumors. However, doxorubicin/dacarbazine chemotherapy on a clinical trial basis may be indicated in patients whose intra-abdominal desmoid is unresectable, or who have failed to respond to treatment with hormones (tamoxifen, toremifene), steroids (prednisone), and nonsteroidal anti-inflammatory agents (Clinoril; Merck & Co., Inc., West Point, PA).
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