Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021843 (bowel obstruction)
9,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Desmoid tumors are rare neoplasms. They occur mostly in females in their reproductive age and may present with pelvic pain and intestinal obstruction. These connective tissue neoplasms are because of uncontrolled proliferation of differentiated myofibroblasts. The cells may synthesize vast amounts of collagen fibers in response to various stimuli. We describe a case of a pelvic desmoid tumor simulating a uterine leiomyoma recurrence. We review the literature on the epidemiology and the treatment options for desmoid tumors and suggest a strong index of suspicion when a new pelvic mass arises in an adult with previous pelvic surgery. We advise a planned multidisciplinary treatment approach at the first hint of the diagnosis of desmoid tumor.
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PMID:Not every recurrent pelvic mass in a female is a leiomyoma. 2298 14

Desmoid tumors (DTs) are benign tumors which are not seen very often, and most of the radiologists and clinicians do not know the characteristics of them very well. Correct and early diagnosis of DTs is important for decreasing mortality and morbidity. Computed tomography enterography (CTE) is a new modality for small bowel imaging which combines the improved spatial and temporal resolution of multidetector computed tomography (CT) with large volumes of ingested enteric contrast material to permit evaluation of the small bowel wall and lumen and also the entire abdomen. We report a familial adenomatous polyposis (FAP) patient with localized mesentery and abdominal wall DTs. We showed the exact location of the DTs and their relation with the small bowel by CTE. In conclusion, CTE is a useful technique for DT localization, the degree of extension and invasion to local structures, presence of partial and complete small bowel obstruction, and the relationship of the tumors with vasculature and whether ischemia has occurred as a result or not.
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PMID:Desmoid Tumor Associated With Familial Adenomatous Polyposis: Evaluation With 64-Detector CT Enterography. 2332 58

Background/Aims Desmoid tumors (DTs) are rare, locally invasive neoplasms that may affect 10-25% of familial adenomatous polyposis (FAP) patients. Our aim was to evaluate incidence and clinical presentation among our patients, the potential impact on FAP outcome and to discuss treatment. Materials and methods Charts from 133 FAP (1977-2013) were reviewed. Patients with DTs were separated to retrieve demographic, clinical and management data. Follow-up was focused on disease evolution causing complications or death. Results 19 (14.3%) DTs were diagnosed, either after previous trauma (16) or during FAP surgery (3). This group comprised 8 men (42.1%) and 11 women (57.9%) with an average age of 32.9 years. Intervals from surgical trauma to DTs ranged from 7 to 60 months. ECMs were detected in 12 (63.1%) patients. DTs were located in the abdominal wall (8), abdominal cavity (8), abdominal wall and cavity (2) and left arm (1). Five patients (26.3%) referred family history of DTs. Patients presented severe complications such as small bowel obstruction (4) and hydronephrosis (2), being directly responsible for death in three patients. Conclusions (1) DTs developed in 14.3% of FAP, mostly after surgical trauma; (2) 30% caused severe morbidity; (3) identification of clinical risk factors may help surgeons to develop screening and therapeutic decisions.
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PMID:Desmoid tumors: clinical features and outcome of an unpredictable and challenging manifestation of familial adenomatous polyposis. 2548 Jul 28

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

Intestinal obstruction is a frequently encountered entity in surgical practice. The signs & symptoms, many a times, are suggestive of the level of obstruction, making the diagnosis of obstruction evident. There are various causes of intestinal obstruction which diversify to an enormous extent, stamping on the famous paradigm for the mysterious nature of the abdomen being referred to as the Pandora's Box. In accordance with the above saying, we report a rare case of a desmoid tumour, presenting as intestinal obstruction, which entices us to strongly believe the same.
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PMID:Desmoid tumour: a rare etiology of intestinal obstruction. 2688 39

Intraabdominal desmoid tumours are rare and can cause intestinal obstruction. Based on the review of the literature, surgical resection with negative margins and adjuvant chemotherapy is the optimal strategy for treatment of this pathology.
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PMID:Intestinal obstruction caused by desmoid tumours: a review of the literature. 2976 Sep 35

Desmoid tumors develop from connective tissue, fasciae, and aponeuroses, and may occur in the context of familial adenomatous polyposis or may arise sporadically; also, they may be extra-abdominal, intra-abdominal, or located in the abdominal wall. These benign tumors have a great aggressiveness with a high rate of local recurrence. Familial adenomatous polyposis is an inherited condition with autosomal dominant transmission, and is characterized by the development of multiple colonic and rectal adenomatous polyps, as well as desmoid tumors. We present the case of a 54-year-old woman with germline APC gene mutation, who underwent a total colectomy, subsequently developing two large infiltrative solid intra-abdominal lesions consistent with desmoid tumors. Medical treatment with Cox-2 inhibitors was initiated without result. She was submitted to resection for intestinal obstruction, but developed local recurrence. The lesions were also unresponsive to tamoxifen, and chemotherapy was initiated with dacarbazine plus doxorubicin, switching to vinorelbine plus methotrexate, achieving a good response in all lesions after 12 months. The approach to these intra-abdominal lesions should be progressive, beginning with observation, then a medical approach with non-steroidal anti-inflammatory drugs or with an anti-hormonal agent. Afterwards, if progression is still evident, chemotherapy should be started. Surgery should be reserved for resistance to medical treatment, in palliative situations, or for extra-abdominal or abdominal wall desmoids tumors.
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PMID:Large desmoid tumors in familial adenomatous polyposis: a successful outcome. 3077 22


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