Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

There are no specific MRI features which are diagnostic in bone and soft tissue sarcomas, but a combination of certain findings allows an accurate diagnosis in some cases including giant cell tumour, chondrosarcoma, liposarcoma, neurofibrosarcoma, aggressive fibromatosis and pigmented villonodular synovitis. MRI is the method of choice for staging bone and soft tissue sarcomas. It is of particular use for identifying satellite nodules and skip lesions within the same bone or anatomical compartment. CT scan is important for screening for pulmonary metastases, and bone scan remains useful for screening for distant skeletal disease. The biopsy should be planned and performed after any MRI examination. MRI is the most sensitive post-therapy evaluation for local recurrence of bone and soft tissue sarcoma.
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PMID:The role of magnetic resonance imaging. When to use it and what to look for. 905 83

Sporadic aggressive fibromatosis (also called desmoid tumor) is a monoclonal proliferation of spindle (fibrocyte-like) cells that is locally invasive but does not metastasize. A similarity to abdominal fibromatoses (desmoids) in familial adenomatous polyposis and a cytogenetic study showing partial deletion of 5q in a subset of aggressive fibromatoses suggests that the adenomatous polyposis coli (APC) gene plays a role in its pathogenesis. APC helps regulate the cellular level of beta-catenin, which is a downstream mediator in Wnt (Wingless) signaling. beta-Catenin has a nuclear function (binds transcription factors) and a cell membrane function (is a component of epithelial cell adherens junctions). Six cases of aggressive fibromatosis of the extremities from patients without familial adenomatous polyposis, or a family history of colon cancer, were studied. Immunohistochemistry, using carboxy and amino terminus antibodies to APC, and DNA sequencing showed that three of the six contained an APC-truncating mutation, whereas normal tissues did not contain a mutation. Western blot and Northern dot blot showed that all six tumors had a higher level of beta-catenin protein than surrounding normal tissues, despite containing similar levels of beta-catenin mRNA. Immunohistochemistry localized beta-catenin throughout the cell in tumor tissues, although it localized more to the periphery in cells from normal tissues. Reverse transcription polymerase chain reaction showed that the tumors expressed N-cadherin but not E-cadherin (a pattern of expression of proteins making up adherens junctions similar to fibrocytes), suggesting that the specific adherens junctions present in epithelial cells are not necessary for beta-catenin function. Increased beta-catenin may cause the growth advantage of cells in this tumor through a nuclear mechanism. The increased protein level, relative to the RNA level, suggests that beta-catenin is degraded at a lower rate compared with normal tissues. In some cases, this is caused by a somatic mutation resulting in a truncated APC protein.
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PMID:Increased beta-catenin protein and somatic APC mutations in sporadic aggressive fibromatoses (desmoid tumors). 925 Jan 46

Infantile fibromatosis, one of the fibrous tumours of infancy and childhood, is a fibroproliferative lesion characterized by aggressive local invasion without any tendency to metastasize, the absence of cytological evidence of malignancy and a high rate of local recurrence when incompletely excised. We report a case of infantile (desmoid-type) fibomatosis in a seven-year-old girl arising from the deep lobe of the parotid gland that was treated by complete surgical excision with preservation of the facial nerve. The clinical features, pathology and treatment are briefly discussed.
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PMID:Infantile (desmoid-type) fibromatosis of the parotid gland. 928 13

Soft tissue sarcomas are uncommon neoplasms that represent approximately 1% of all malignancies. It is clear that sarcomas require a therapeutic approach that establishes local control and thereby eliminates the potential of metastasis for patients with truly limited disease. Localized sarcomas are generally treated by surgery. Excision must be complete, with a wide margin of normal tissue, and along anatomic planes, or recurrence will almost certainly follow. Amputations are still occasionally required, although limb salvage procedures are being used increasingly, particularly in the context of multimodality therapy with irradiation or chemotherapy. Radiotherapy can be highly effective for improving local control and is used as adjuvant therapy, either preoperatively or postoperatively. In case of non-in-sano-resection a salvage surgery is indicated. Use of adjuvant postoperative radiotherapy allows for more conservative surgery without compromising local control, and therefore often may allow limb salvage where amputation might otherwise be necessary, e.g. in case of R1- or R2-resection without a new resection, a close margin or large tumors with histologic G2 or G3 grading and in case of local relapses. Local control rate of 90% are reported for the combination of pre- and postoperative radiotherapy. Prognosis is still limited by distant metastases. In case of unresectable tumors neutron radiotherapy results in 50% local control. New approaches e.g. hyperfractionated-accelerated radiotherapy, interoperative radiotherapy and chemoradiotherapy are promising perspectives, which are being evaluated in clinical studies. Desmoid tumors benefit of postoperative radiotherapy in case of R1-resection or relapse.
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PMID:[Radiotherapy of soft tissue sarcomas]. 975 91

Aggressive fibromatosis is a locally infiltrative fibroblastic tumour that arises from fascial planes of soft tissue but does not metastasize. It is known to invade muscle, subcutaneous tissue and neurovascular structures. However, bone involvement is very rare and there has been few reports of bone involvement. We present a case of a young man with aggressive fibromatosis of the right lower leg with fibula involvement.
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PMID:A case report on aggressive fibromatosis with bone involvement. 1041 72

Aggressive fibromatosis is a rare fibroproliferative disorder with a variable biologic potential that is locally morbid but does not metastasize. Eighteen patients with extraabdominal fibromatosis were treated with a multidisciplinary approach over a 27-year period. Our observations, coupled with a review of the literature, suggest that conservative surgery with the goal of a wide margin coupled with adjuvant therapies may result in adequate control of disease from infancy to adolescence. Amputation should be reserved for cases in which the disease or its treatment have resulted in a nonfunctional or chronically painful extremity. Radiation should be used as a last resort in the skeletally immature because of the risk of growth disturbance, contracture, and secondary malignancy. Chemotherapy may have a role in children with inoperable disease, in those who have gross residual tumor after an intralesional procedure, for disease progression or recurrence, and neoadjuvant therapy should be investigated as a means to achieve a wide margin in some cases.
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PMID:Aggressive fibromatosis from infancy to adolescence. 1057 49

Desmoid tumours are rare lesions with a local invasive potential and a risk of recurrence, considered to be benign due to the absence of metastases. They are classified as fibromatoses and may be associated with Gardner's syndrome. The authors report an unusual case, in a 33-year-old woman, of a desmoid tumour invading the right ureter with upper tract dilatation. Etiologic factors (traumatic, hormonal, auto-immune...) are discussed, together with the treatment of choice to lower the risk of recurrence.
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PMID:[Desmoid tumor of the mesentery secondary to colectomy. An exceptional cause of ureteric obstruction]. 1063 21

Desmoids are uncommon proliferations of fibroblasts that occur with disproportionate frequency in patients with familial adenomatous polyposis. They do not metastasize and are histologically benign. Despite this, the unpredictable and often aggressive nature of familial adenomatous polyposis-associated desmoids and their tendency to occur in intra-abdominal sites means that they present a difficult management problem, and they are a leading cause of death in patients with familial adenomatous polyposis who have undergone colectomy. We report a case of a patient with familial adenomatous polyposis who had extensive and aggressive desmoid disease and whose management was further complicated by a large intrahepatic desmoid. There are no previous reports of desmoids occurring in the liver.
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PMID:Familial adenomatous polyposis complicated by an intrahepatic desmoid tumor: report of a case. 1091 Feb 52

A Japanese woman with familial adenomatous polyposis in whom a duodenal ampullary adenoma underwent malignant change during a 10-year follow-up period is reported. After restorative proctocolectomy in 1989, and extensive small bowel resection for desmoid disease in 1991, regular surveillance duodenoscopies, including three to nine biopsies (mean, 4.8) were performed annually or biannually. Until 1995, the endoscopic findings of duodenal polyposis (including an ampullary polyp) did not progress and the histopathology did not worsen. In 1996, there was an increase in the number and size of the duodenal polyps, and the ampulla of Vater looked enlarged. Open surgery was discussed but not proceeded with because of the risk for short bowel syndrome. In January 1998, she was admitted with a diagnosis of acute pancreatitis. Duodenoscopy and radiological examination revealed that an advanced ampullary cancer had developed, and histopathology revealed a well-differentiated adenocarcinoma. Multiple hepatic metastases and ascites led to her death, in June, 1998. This in-vivo demonstration of the adenoma-carcinoma sequence highlights current limitations in the surveillance and treatment of duodenal lesions.
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PMID:Development of duodenal cancer in a patient with familial adenomatous polyposis. 1108 1

Patients with a soft tissue malignancy involving the sciatic nerve who present with neurologic loss generally are advised to have an amputation. Twenty patients who underwent limb-sparing procedures with complete resection of the sciatic nerve as treatment for neurofibrosarcomas (12 patients), liposarcomas (four patients), malignant fibrous histiocytomas (two patients), recurrent desmoid tumor (one patient), and epithelioid hemangioendothelioma (one patient) were reviewed retrospectively. The mean age of these nine women and 11 men at the time of surgery was 51 years (range, 28-84 years). The right sciatic nerve was affected in 12 patients. These tumors were large and high grade. A mean of 22 cm of the nerve had to be resected (range, 8-42 cm). Ten patients received preoperative radiotherapy and 16 patients had intraoperative or postoperative radiotherapy. At a mean followup of 35 months (range, 7-97 months), 14 of the 20 patients were alive. Two patients had local recurrences develop (10%), whereas 12 patients had distant metastases. The function of the 10 patients as assessed by the Toronto Extremity Salvage Score averaged 74%. Most patients indicated that walking in the house is not difficult, but walking is compromised as soon as an effort is needed. Four patients walk without a cane, four needed one cane, and two needed two canes. The patients experienced stiffness, a sense of numbness, and premature fatigue. The use of analgesics was infrequent. Generally, patients rated themselves to be mildly to moderately disabled. From this small number of patients, it is shown that a tumor involving the sciatic nerve can be treated by limb-sparing surgery, including complete nerve resection, as an alternative to hip disarticulation or hindquarter amputation because the limb salvage option provides an acceptable functional outcome.
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PMID:Sciatic nerve resection in the thigh: a functional evaluation. 1115 2


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