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)

Small intestinal neoplasms are uncommonly encountered in clinical practice. They may occur sporadically, in association with genetic diseases (e.g., familial adenomatous polyposis coli or Peutz-Jeghers syndrome), or in association with chronic intestinal inflammatory disorders (e.g., Crohn's disease or celiac sprue). Benign small intestinal tumors (e.g., leiomyoma, lipoma, hamartoma, or desmoid tumor) usually are asymptomatic but may present with intussusception. Primary malignancies of the small intestine-including adenocarcinoma, leiomyosarcoma, carcinoid, and lymphoma-may present with intestinal obstruction, jaundice, bleeding, or pain. Extraintestinal neoplasms may involve the intestine via contiguous spread or peritoneal metastasis. Hematogenous metastases to the intestine from an extraintestinal primary are unusual and are most typical of melanoma. Because the small intestine is relatively inaccessible to routine endoscopy, diagnosis of small intestinal neoplasms is often delayed for months after onset of symptoms. When the diagnosis is suspected, enteroclysis is the most useful imaging study. Small bowel endoscopy (enteroscopy) is increasingly widely available and may permit earlier, nonoperative diagnosis.
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PMID:Small intestinal neoplasms. 1158 39

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

Magnetic resonance (MR) imaging is often used in the detection and staging of large pelvic masses. Many large masses in the female pelvis arise from the reproductive organs (eg, uterus, cervix, ovaries, fallopian tubes). In addition, these masses may arise from the gastrointestinal system, urinary system, adjacent soft tissues, peritoneum, or retroperitoneum or from metastases. The majority of large masses in the female pelvis represent such commonly encountered entities as uterine fibroid tumor, dermoid tumor, ovarian cyst, and ovarian cancer. However, uncommon pelvic masses such as mesothelioma, adenocarcinoma, carcinosarcoma, leiomyosarcoma, and desmoid tumor may also be seen. Thus, the differential diagnosis for female pelvic masses is extensive. However, the site of origin, MR imaging characteristics, and clinical history may all help narrow the differential diagnosis. Although with large tumors it may not always be possible to determine the site of origin or distinguish between various tumors at radiology, familiarity with the clinicopathologic and MR imaging features of common and uncommon pelvic masses is important for diagnosis and treatment.
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PMID:MR imaging of common and uncommon large pelvic masses. 1264 Jan 56

Deeply seated aggressive fibromatosis also termed as desmoid tumors are rare tumors that invade surrounding structures. Although they never metastasize mortality rate may be as high as 10% due to their aggressive local behavior. Intraabdominal desmoid tumors are usually associated with familial poliposis coli and have a high recurrence rate regardless of the therapy instituted. Sporadic cases are very rare and generous surgical excision may be of benefit. We hereby report 2 siblings with sporadic pancreatic desmoid tumors who also harbor additional fibrotic masses in the pelvis. Although in previously reported cases there is usually a triggering event such as trauma, in the present cases there was no inciting event. Furthermore, the cases are without an associated FAP history, which provides the first clinical clue of a possible genetic determinant in this rare disorder.
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PMID:Sporadic hereditary pancreatic desmoid tumor: a new entity? 1286 94

Desmoids are rare mesenchimal tumors that may originate also inside the abdomen or in the abdominal wall. These tumors are biologically characterized by a tendency to local growth, and only rarely are they able to develop distant metastases. Surgical excision usually is the best treatment with a chance of a cure. In the few reports on intraabdominal or abdominal wall desmoids, open surgery always was performed. The first case of successful laparoscopic resection of a symptomatic anterior wall desmoid tumor with intraabdominal growth is reported. During the procedure, it was difficult to mobilize and grasp the mass using the common laparoscopic instruments, but with the help of the "marionette trick," modified suture traction technique, the tumor could be removed easily using only three trocars. With four traction sutures minimizing the wall trauma, the trick made it possible to mobilize the mass in at least, seven directions, according to the principles of physical forces and vectors. This simple trick can be helpful for other common laparoscopic procedures, avoiding the insertion of sometimes ineffective instruments through more traumatic trocars.
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PMID:Laparoscopic resection of an abdominal wall desmoid using a modified suture traction technique: the "marionette trick". 1497 54

The desmoid tumor (DT) is a soft tissue neoplasm most frequently localized in the anterior abdominal wall typically in females of childbearing age. Because its particular incidence in women who had recently been pregnant, it was correlated with delivery's trauma stimulating proliferation of muscolo-aponeurotic tissues. Complete surgical resection is the recommended treatment approach to prevent recurrence. Many authors emphasize the role of radiotherapy in regression of DT and in controlling local recurrence in patient who had incomplete resection. Many others emphasize the role of chemotherapy or antiestrogenic compounds, even though tumour does not express estrogen receptors. DT, otherwise, is neoplasm with high rates of recurrence after surgery but it never develops distant metastases, so that function and structure-sparing surgery may be a reasonable choice in young women when possible without leaving macroscopic residual disease. Furthermore literature data suggest that the presence of incomplete histological surgical resection does not correlate with local recurrence and that pregnancy does not represent a risk factor. In women of childbearing age, even after non radical histological DT primary resection, adjunctive radiotherapy, chemotherapy or antiestrogen therapy could be avoided and clinical observation alone may be considered.
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PMID:[Desmoid tumor of rectus muscle of abdomen in a woman of childbearing age: what can we do?]. 1501 10

Context.-Bone morphogenetic proteins (BMPs) are thought to be responsible for bone formation; they cause bone to form in soft tissues and are clinically used in helping fracture union or tumor reconstructions. Skeletal metastases from epithelial tumors may be either bone-forming (blastic) or non-bone-forming (lytic).Objective.-We studied the expression of BMPs in a variety of primary and secondary lesions of bone (both bone-forming and non-bone-forming) to determine if there was a consistent relationship between bone formation and BMP expression.Design.-We compared a bone-forming lesion (fibrous dysplasia) with a non-bone-forming lesion (desmoid tumor), using reverse transcription-polymerase chain reaction, Northern blot analysis, and immunohistochemistry to detect BMPs. We also studied a number of non-bone-forming secondary lesions (carcinomas that formed lytic metastases to the skeleton) and found BMP production in most of these tumors.Results.-We found that BMPs were expressed in both bone-forming and non-bone-forming benign musculoskeletal lesions. In the first part of the study, BMPs were found in both fibrous dysplasia and desmoid tumors. Bone morphogenetic proteins were also expressed by several tumors. In the next part of the study (paraffin-embedded tissue), BMPs were expressed by a variety of tumors, irrespective of the radiological nature (blastic or lytic) of their metastases.Conclusions.-We conclude that BMP production alone cannot explain bone formation, and other factors either alone or in combination may be responsible for blastic metastases to the skeleton and for bone formation by primary bone lesions, such as fibrous dysplasia.
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PMID:Bone Morphogenetic Proteins Are Expressed by Both Bone-Forming and Non-Bone-Forming Lesions. 1550 26

Deep fibromatoses (desmoid tumors) are clonal myofibroblastic proliferations that are prone to aggressive local recurrences but that do not metastasize. They must be distinguished from a host of fibroblastic and myofibroblastic lesions as well as from smooth muscle neoplasms. Virtually all deep fibromatoses have somatic beta-catenin or adenomatous polyposis coli (APC) gene mutations leading to intranuclear accumulation of beta-catenin. Since low-grade sarcomas in general lack beta-catenin and since reactive proliferations would not be expected to have it, we predicted that nuclear beta-catenin expression would be detected in deep fibromatoses but absent in other entities in the differential diagnosis. We evaluated the role of beta-catenin to help differentiate distinguish deep fibromatoses from congeners. Formalin-fixed, paraffin-embedded sections from 21 lesions from 20 patients with deep fibromatoses were stained with monoclonal beta-catenin antibody (Transduction Laboratories) and compared with low-grade fibromyxoid sarcoma (n=12), leiomyosarcoma (n=10), various other fibrosarcoma variants (n=13, including 3 myofibrosarcomas, 3 sclerosing epithelioid fibrosarcomas, 5 low-grade fibrosarcomas, 1 classic fibrosarcoma arising in dermatofibrosarcoma protuberans, 1 inflammatory myxohyaline tumor/myxoinflammatory fibroblastic sarcoma), myofibroma/myofibromatosis (n=12), nodular fasciitis (n=11), and scars (n=9). Nuclear and cytoplasmic staining was assessed. All 21 examples of deep fibromatosis displayed nuclear beta-catenin (focal nuclear staining in one case to 90% staining). All other lesions tested (n=67) lacked nuclear labeling for beta-catenin, showing only cytoplasmic accumulation. beta-Catenin immunohistochemistry separates deep fibromatosis from entities in the differential diagnosis, a finding that can be exploited for diagnosis. Most fibromatoses have diffuse nuclear staining although occasional examples only focally label.
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PMID:Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions. 1583 90

Desmoid tumors (also called deep fibromatoses) are rare benign tumors associated with pregnancy and Gardner syndrome. These tumors are characterized by bland-appearing fibroblasts, indistinct margins, and an ability to cause pathology by local invasion and recurrence. They arise in the abdominal cavity, in the abdominal wall, or in the extremities/trunk, each with a slightly different biologic behavior. Though they are not cancer and do not metastasize, desmoids can cause significant morbidity and occasionally death through local/regional invasion of critical structures. Treatment primarily is surgical, although radiation or systemic therapy can be beneficial to the patient when surgery is not feasible. This article highlights the biology and clinical features of desmoid tumors.
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PMID:Desmoid tumors and deep fibromatoses. 1593 97

The detection of a solid abdominal mass after surgery for testicular cancer most frequently represents metastatic disease. We report an unusual case of mesenteric desmoid tumor presenting after retroperitoneal lymph node dissection for metastatic testicular cancer. To our knowledge, there are no similar reports in the radiology literature.
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PMID:Mesenteric desmoid mimicking recurrent testicular cancer. 1609 67


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