Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0403608 (ureter)
 9,655 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:An aggressive desmoid tumor in a patient with familial adenomatous polyposis: immunohistochemical findings. 1002 64

The authors report a case of isolated mesenteric fibromatosis un associated with familial adenomatous polyposis or Gardner's syndrome or prior abdominal surgery. These neoplasms are usually asymptomatic until when the compression of the small or large bowel or the ureter causes symptoms; although they are benign lesions without metastases, local recurrences are very frequent. Surgical removal is the primary treatment; until now no satisfactory results have been obtained with other therapeutic modalities.
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PMID:[Isolated mesenteric fibromatosis. A clinical case]. 1038 Mar 62

Desmoid tumours (DT) are slow-growing tumours that consist of proliferations of well-differentiated fibroblasts. Although the typical characteristics of malignant tumours, such as distant metastases, are absent, the tumours are locally aggressive and grow into neighbouring structures. The prevalence of desmoid tumours in patients with FAP is 7-12%. The lifetime risk of developing desmoid tumours is about 20%. In FAP, most tumours are intra-abdominal or located in the abdominal wall. Next to colorectal cancer, desmoid tumours are the most frequent cause of death in FAP. Possible risk factors for the development of desmoid tumours are previous surgical procedures, pregnancy, female sex, a family history of desmoid tumours, and specific mutations in the APC-gene. Both CT scanning and MRI can be used to detect the tumours. An excision biopsy is needed to establish the diagnosis. Medicinal treatment with NSAIDs is the treatment of first choice, followed by hormonal treatment (e.g., tamoxifen) in combination with NSAIDs. Both forms of treatment lead to a response in about 30-50% of the patients. Surgery is the preferred treatment for extra-abdominal tumours or tumours located in the abdominal wall. Surgical treatment of intra-abdominal tumours is only indicated in patients with obstruction of the bowel or ureter. Chemotherapy is indicated in patients with progressive desmoid tumours when non-cytotoxic treatment has failed. Radiotherapy may play a role in the treatment of irresectable extra-abdominal or abdominal wall tumours, or as adjuvant treatment of tumours with positive margins.
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PMID:[Desmoid tumors in patients with familial adenomatous polyposis]. 1216 72

There is evidence that aspirin--and apparently other NSAIDs--may be protective agents against cancer in the gastrointestinal tract. These effects are particularly well documented in the colon and rectum. Even considered in isolation, the observational data regarding colorectal neoplasia are quite strong, and the reality of a protective effect is buttressed by clinical trial data showing that aspirin prevents sporadic adenomas. Furthermore, the NSAIDs sulindac celecoxib have actually led to the regression of existing colorectal polyps in patients with FAP. Clearly, NSAIDs have the potential to suppress carcinogenesis in the large bowel. Observational data suggesting inverse associations of NSAIDs with cancers of the stomach and esophagus have emerged from several case-control studies and a few cohort analyses. In some studies the findings display features often associated with causal relationships, for example decreasing risks with increasing doses or duration of use. Nonetheless, the data currently do not support a secure conclusion that NSAIDs protect against these malignancies. The relevant data are not nearly as extensive as those for the colorectum, and case-control investigation of these upper gastrointestinal sites may be particularly delicate. It is conceivable that early symptoms of cancer (or of pre-invasive lesions) may have discouraged NSAID use in the cancer patients, creating the appearance of a protective association of the drugs with the risk of these malignancies. More extensive observational data particularly from cohort studies would be desirable to confirm the existing findings and clarify the doses and durations of use required for an effect. Clinical trial investigation might also be practical for pre-neoplastic endpoints, or--in carefully selected populations--perhaps with cancer as the focus. There are only relatively limited data available regarding the effect of NSAIDs on cancer of the pancreas. However, the studies that have investigated this malignancy have reported indications that NSAIDs may have a protective effect. The effects of NSAIDs on cancers outside the gastrointestinal tract are not clear. Some investigations suggest that NSAID use, particularly aspirin, is inversely associated with risk of cancers of the breast or ovary, but several well-done studies have not seen these associations, and the observations could have been due to bias or confounding. Findings regarding prostate cancer are similarly conflicting. The urinary tract is one organ system in which several studies have reported an increased cancer risk in association with NSAID use. Nonetheless, the effects remain unclear. There is only limited available information regarding carcinoma of the bladder, and no firm conclusions can be drawn at this point. More extensive data have been generated regarding the effect of NSAIDs--largely salicylates--on renal cell carcinoma or cancer or the renal pelvis and ureter. Although some studies have reported increased risks, there are also findings suggesting no association. It is particularly difficult for observational studies to ascertain with confidence the true effects of aspirin because of the suspected relationship of these cancers with use of phenacetin and perhaps acetaminophen. Further data--particularly from careful and large cohort studies--would be important to clarify these issues. As a body of research, the findings discussed here from epidemiological studies and clinical trials have begun to clarify the effect of NSAIDs on carcinogenesis in various organs in humans. There is clear potential for protective effects at several anatomic sites. Even for the colorectum, however, it is probably premature to now begin to use these drugs widely for cancer prevention. To reach that point, a weighing of the risks and benefits of the drugs needs to be made, together with a judgement regarding the benefits of alternative means of prevention. For colorectal cancer, for example, aspirin may provide only limited benefit over regular colonoscopy [95, 96]. Nonetheless, with the increased understanding of the clinical effects of NSAIDs on cancer, the development of effective chemoprevention with these drugs appears to be a real possibility.
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PMID:Epidemiology of non-steroidal anti-inflammatory drugs and cancer. 1279 46

A 30-year-old man with familial adenomatous polyposis (FAP) underwent prophylactic proctocolectomy by laparoscopy-assisted surgery. After 10 months, we found an intra-abdominal tumor, which grew rapidly to 25 cm in diameter. We performed an emergency operation, which revealed that it was a desmoid tumor derived mainly from colorectal mesenterium. The tumor was removed with three short segments of intestine and the left ureter. A computed tomography (CT) scan done 3 months later showed a 10 cm mesenteric desmoid tumor at the beginning of jejunum, approaching the root of the superior mesenteric artery (SMA). Fortunately, we were able to remove the tumor without injuring the SMA. To our distress, however, another recurrent mesenteric desmoid tumor was discovered in the pelvis one month later, which grew rapidly from 5 cm to 16 cm within 4 months. During this period, we gave the patient several regimens, including antiestrogen (tamoxifen), a nonsteroidal anti-inflammtory drug and imatinib mesylate (Gleevec), which had little or no effect. Finally, when the desmoid occupied the pelvic space, we gave the patient dacarbazine (DTIC) and doxorubicin (DOX). After seven courses, the mesenteric tumor showed an almost complete response (CR). The chemotherapy caused grade 3 to 4 leukocytopenia, but without any hazardous events. No evidence of further recurrence of mesenteric desmoid has been seen for 4 years. This combination chemotherapy is a promising strategy, even against an extremely aggressive, life-threatening mesenteric desmoid associated with FAP.
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PMID:Dacarbazine-Doxorubicin therapy ameliorated an extremely aggressive mesenteric desmoid tumor associated with familial adenomatous polyposis: report of a case. 1830 51

Hereditary colorectal cancer (CRC) is highly heterogeneous, both genotypically and phenotypically. The most frequently occurring hereditary colorectal cancer syndrome is Lynch syndrome, accounting for approximately 3% of the total colorectal cancer burden. Polyposis syndromes, such as familial adenomatous polyposis, account for a lesser percentage. Familial colorectal cancer, defined by family history, occurs in an estimated 20% of all colorectal cancer cases. With a worldwide annual colorectal cancer incidence of over one million, and annual mortality of over 600,000, hereditary and familial forms of colorectal cancer are a major public health problem. Lynch syndrome is attributable to DNA mismatch repair germline mutations, with the MSH2, MLH1, MSH6, and PMS2 genes being implicated. The characteristics of Lynch syndrome-associated colorectal tumors, including early age of onset and predilection to the proximal colon, mandate surveillance by colonoscopy beginning by age 20 to 25 and repeated every other year through age 40 and annually thereafter. Besides colorectal cancer, Lynch syndrome also predisposes to a litany of extracolonic cancers, foremost of which is endometrial cancer, followed by cancer of the ovary, stomach, renal pelvis and ureter, small bowel, hepatobiliary tract, pancreas, glioblastoma multiforme in the Turcot's variant, and sebaceous skin tumors in the Muir-Torre variant and, more recently identified, cancers of the breast and prostate. The most common polyposis syndrome is familial adenomatous polyposis, caused by mutations in the APC gene. Affected individuals have multiple colonic adenomas and, without treatment invariably develop colorectal cancer. Colonic surveillance with polypectomy may be pursued until the appearance of multiple colonic adenomas, at which time prophylactic colectomy should be considered. Extra-intestinal manifestations include desmoid tumor, hepatoblastoma, thyroid carcinoma, and medulloblastoma. Other polyposis syndromes include the hamartomatous polyp syndromes, including juvenile polyposis syndrome, Peutz-Jeghers syndrome, Cowden syndrome, and Bannayan-Ruvalcaba-Riley syndrome.
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PMID:Practical genetics of colorectal cancer. 2584 92

Desmoid tumors (DTs) are benign myofibroblastic neoplasms originating from the fascia or muscle aponeurosis, which occur in one-third of patients with familial adenomatous polyposis (FAP). Most FAP-associated DTs occur in the intra-abdominal or abdominal wall region, thus, their infiltrative or expansive growth causes life-threatening organ damage, such as intestinal obstruction, urethral obstruction, and mesenteric infiltration with the involvement of mesenteric vessels. Treatments including surgical resection, cytotoxic chemotherapy, nonsteroidal anti-inflammatory drugs and anti-estrogen therapy have all been tried with variable success. Here, we report on three patients with FAP who developed multiple intra-abdominal and abdominal wall DTs after total proctocolectomy and ileal pouch-anal anastomosis. Two cases underwent surgical resection of uncontrolled abdominal wall DTs after successful control of intra-abdominal DTs by systemic chemotherapy. The remaining case underwent repeated surgical resections of multiple intra-abdominal and abdominal wall DTs, and consequently had recurrent intra-abdominal DTs, with involvement of the small bowel and ureter. Surgical intervention as tumor volume reduction (cytoreduction) may be useful for cases with medical treatment-refractory or symptomatic FAP-associated abdominal DTs.
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PMID:Cytoreductive strategy for multiple intra-abdominal and abdominal wall desmoid tumors in familial adenomatous polyposis: report of three cases. 2618 Oct 76

A 20-year-old woman with Gardner syndrome and intra-abdominal desmoid tumors presented with increasing abdominal pain. CT demonstrated a new area of central hypodensity in a presumed desmoid tumor, compressing the left ureter. Findings were suspicious for abscess or fistula to the ureter. Subsequent 99mTc-MAG3 renogram demonstrated persistent extraureteral radiotracer activity in the region of the tumor, confirming a desmoid tumor-ureteral fistula. Desmoid tumors are benign but locally aggressive fibrous neoplasms that can be sporadic or associated with familial adenomatous polyposis syndromes, specifically Gardner syndrome. Fistula formation to the ureter has been reported infrequently.
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PMID:Renographic Demonstration of Desmoid Tumor-Ureteral Fistula. 2628 72