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)

The treatment strategy for mesenchymal tumors of the gastrointestinal tract is based upon typing of the tumor. Especially differential diagnosis of gastrointestinal stromal tumors (GISTs) to leiomyomas is crucial for determining radicality of surgery. L1 cell adhesion molecule (CD171) plays an essential role in tumor progression. The aim of this study was to determine expression of L1 in GISTs, smooth muscle tumors, desmoid-type fibromatosis and peripheral nerve sheath tumors (PNSTs). We retrospectively analyzed a total of 129 surgically resected primary tumors or metastases of 72 GISTs, 29 smooth muscle tumors, seven PNSTs and 21 desmoid-type fibromatosis by immunohistochemistry for c-kit, CD34, smooth muscle actin, desmin, vimentin, S-100 and L1 expression. L1 expression was detected in 53 (74%) of 72 GISTs but in none of 29 smooth muscle tumors or 21 desmoid-type fibromatosis (P<0.01 by Fisher's test). In all, four (57%) of seven peripheral nerve sheath tumors were L1-positive. Survival analysis of 55 surgically completely resected GISTs presenting without metastasis at initial diagnosis revealed no tumor-specific death among L1-negative patients (P=0.13 by log-rank test; median follow-up time 41 months) and one recurrence was observed (P=0.12). Interestingly high levels of L1 were seen in tumor vascular endothelial cells of smooth muscle tumors and PNSTs, but not in GISTs. Our data show that L1 is highly expressed in GISTs but not in smooth muscle tumors and desmoid-type fibromatosis being important differential diagnoses. The trend towards a reduced survival of L1-positive patients in this study has to be further evaluated in future trials with higher patient numbers.
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PMID:L1 (CD171) is highly expressed in gastrointestinal stromal tumors. 1640 Mar 20

Nodular fasciitis (NF) is a rapidly growing cellular mass composed of fibroblasts/myofibroblasts, usually localized in subcutaneous tissues, that typically undergoes fibrosis and almost never recurs. Desmoid tumours (DTs) are rare forms of fibroblastic/myofibroblastic growth that arise in deep soft tissues, display a propensity for local infiltration and recurrence, but fail to metastasize. Given that both entities are primarily fibroblastic/myofibroblastic lesions with overlapping histological features, their gene expression profiles were compared to identify differentially expressed genes that may provide not only potential diagnostic markers, but also clues as to the pathogenesis of each disorder. Differentially expressed transcripts (89 clones displaying increased expression in DTs and 246 clones displaying increased expression in NF) included genes encoding several receptor and non-receptor tyrosine kinases (EPHB3, PTPRF, GNAZ, SYK, LYN, EPHA4, BIRC3), transcription factors (TWIST1, PITX2, EYA2, OAS1, MITF, TCF20), and members of the Wnt signalling pathway (AXIN2, WISP1, SFRP). Remarkably, almost one-quarter of the differentially expressed genes encode proteins associated with inflammation and tissue remodelling, including members of the interferon (IFN), tumour necrosis factor (TNF), and transforming growth factor beta (TGF-beta) signalling pathways as well as metalloproteinases (MMP1, 9, 13, 23), urokinase plasminogen activator (PLAU), and cathepsins. The observations provide the first comparative molecular characterization of desmoid tumours and nodular fasciitis and suggest that selected tyrosine kinases, transcription factors, and members of the Wnt, TGF-beta, IFN, and TNF signalling pathways may be implicated in influencing and distinguishing their fate.
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PMID:A gene expression signature that distinguishes desmoid tumours from nodular fasciitis. 1644 Feb 90

Soft tissue tumours and tumour-like lesions of the chest wall are uncommon. The purpose of this pictorial essay is to describe the imaging findings of chest wall soft tissue tumours and tumour-like lesions. We searched the radiological and pathological archive at our institution retrospectively and reviewed the literature on soft tissue tumours of the chest wall. Common chest wall soft tissue tumours and mass-like lesions include peripheral nerve tumours, lipomas, liposarcomas, haemangiomas, elastofibromas, metastases, lymphoma and abscesses. Other lesions encountered include desmoid tumours and malignant fibrous histiocytoma. Many have distinctive radiological findings or occur in specific locations, allowing a specific radiological diagnosis to be suggested.
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PMID:Soft tissue tumours and mass-like lesions of the chest wall: a pictorial review of CT and MR findings. 1672 17

Desmoid tumors are nonencapsulated, locally invasive tumors of fibrous origin. Although lacking the ability to metastasize, they are notorious for recurrence. A wide variety of locations have been documented in the literature with respect to the origin of these tumors. Intra-abdominal desmoids are commonly found in the mesentery. We present the report of a case in which a sporadic desmoid tumor originated from the wall of the ileum. The patient was successfully treated with surgery alone, and has remained recurrence free on follow-up. Modalities of treatment documented in the literature include surgery, nonsteroidal, anti-inflammatory drugs such as sulindac, and chemotherapeutic drugs such as vinblastine and methotrexate, radiotherapy, and, recently, tyrosine kinase inhibitors such as Imatinib (gleevec). Of all the options described, surgery with tumor-free margins has been defined as the best treatment modality.
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PMID:An unusual presentation of desmoid tumor in the ileum. 1698 93

Desmoid tumors are rare solid tumors that arise from musculoaponeurotic tissues. They are classified as benign as they do not metastasize. Desmoid tumors can, however, exhibit rapid local growth and clinically they can mimic sarcomas. Their histological appearance can also resemble some malignant neoplasms such as low grade sarcomas, rendering the differential diagnosis difficult. The present report describes a 60-year-old woman with a history of left mastectomy, performed for a lymph node negative adenocarcinoma. At follow-up 4 years later, a solid nodule was palpated below the right breast. The tumor increased in size over several weeks and caused local radiating chest pain. Clinically a breast cancer metastasis was suspected. Open biopsy revealed a desmoid tumor. The tumor was resected together with a part of the anterior hemithorax, and the defect in the chest wall covered with a Goretex-patch. Six months postoperatively, the patient is doing well with no signs of locally recurrent disease.
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PMID:[Desmoid tumor of chest wall--an important differential diagnosis to malignancies]. 1709 29

Aggressive fibromatoses (AF) are locally aggressive neoplasms that do not metastasize but are frequently associated with one or more recurrences and subsequent associated morbidity. AF in the urological system is quite rare and has mainly been described in single case reports or as isolated cases in a large series of extra-abdominal desmoid tumors. We report 2 cases of AF in the bladder and scrotum, and provide a review of similar published cases.
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PMID:Aggressive fibromatosis in the urological system. Report of two adult patients and review of the literature. 1719 43

Desmoid tumors or fibromatoses comprise a number of benign fibrous proliferative lesions that have local infiltrative growth and tendency to recur after incomplete excision. They never metastasize. The authors present a 31-year-old woman who, due to epigastric pain and palpable mass detected on presentation, underwent the excision of firm tumorous mass, 210 x 140 x 115 mm in diameter, from the lesser sac. Compressing the splenic vein, the tumor caused left-sided portal hypertension which subsided after the mass was removed. The recovery was uneventful. The histological examination verified typical desmoid tumor. Twelve years after surgery, the patient remained symptom-free with no signs of recurrence.
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PMID:[Desmoid tumor within lesser sac]. 1725 13

Aggressive fibromatoses, also known as desmoid tumours, are rare fibrous tissue proliferations with a tendency for slow, local infiltrative growth. There is an association with Gardner's syndrome and familial adenomatous polyposis. Histologically they are fairly bland with no abnormal mitoses or necrosis. They do not metastasize, but can cause significant morbidity through their locally destructive effects. Magnetic resonance imaging is the method of choice for diagnosis, pre-treatment planning and post-treatment follow-up. Surgical excision with a wide margin is the treatment of choice. However, there is a tendency for local recurrence and repeated excision may result in a poor functional or cosmetic outcome. Radiotherapy is used to reduce local recurrence rates after excision and is also used to treat inoperable tumours. Long-lasting remissions can be obtained. Treatment is now planned using modern three-dimensional conformal techniques, similar to those used in soft tissue sarcoma management. There is no definite dose-response relationship, but doses of 50-60 Gy in 1.8-2 Gy fractions are recommended. Systemic therapy has been used for lesions not controlled by surgery or radiotherapy, or less commonly, as a primary treatment. Tamoxifen and non-steroidal anti-inflammatory agents are used most often as they are relatively non-toxic, but there is limited experience with cytotoxic chemotherapy and biological agents. There are no randomised trials to help guide the management of this locally aggressive 'benign' tumour and treatment decisions are best made by the local soft tissue sarcoma multidisciplinary team.
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PMID:Fibromatosis: benign by name but not necessarily by nature. 1741 39

Sarcomas are mesenchymal cancers, which, in many cases, have distinctive molecular features. Limb-sparing surgery delivered at specialised sarcoma centres as part of a multidisciplinary approach has become the standard treatment for most patients and usually provides excellent local control. Preoperative treatment with chemotherapy is most common for patients with bone sarcomas. The ideal sequence of surgery and radiation for local management of soft-tissue sarcoma remains controversial on the basis of early versus late treatment complications, although preoperative radiation can provide the best results for improved long-term function. New methods for radiation delivery and tumour sensitisation might provide further improvements. However, metastatic disease is common, and conventional chemotherapy provides for only a narrow therapeutic window outside of a few responsive pathological subtypes. Targeting underlying molecular events in specific sarcomas can provide for dramatic benefits, as has been seen with imatinib treatment for gastrointestinal stromal tumours and dermatofibrosarcoma protuberans. Trials of agents targeting the cell cycle and angiogenesis in soft-tissue sarcomas, and of those targeting osteoclasts in bone sarcomas, are currently underway. Biological data and preclinical studies support trials using inhibitors of hedgehog signalling in chondrosarcoma, inhibitors of wnt/beta-catenin in osteosarcoma and aggressive fibromatosis, and inhibitors of histone deacetylases in synovial sarcoma and Ewing sarcoma. Pharmacogenetic approaches will be needed to identify individual determinants of response and outcome in order to maximise the benefits of targeting specific molecular events and keep side-effects to a minimum. Research in stem-cell biology and nanotechnology holds promise for additional novel treatment options in the future.
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PMID:Opportunities for improving the therapeutic ratio for patients with sarcoma. 1767 78

In defect reconstruction following radical oncologic resection of malignant chest wall tumors, adequate soft-tissue reconstruction must be achieved along with function, stability, integrity, and aesthetics of the chest wall. The purpose of this retrospective analysis was to evaluate the oncoplastic concept following radical resection of malignant chest wall infiltration with an interdisciplinary approach. Between 1999 and 2005, 36 consecutive patients (nine males, 27 females, mean age 55 years, range 20-78) were treated with resection for malignant tumors of the chest wall. Indications were locally recurrent breast carcinoma (patient n=22), thymoma (n=1), and desmoid tumor (n=1). Primary lesions of the chest wall were spinalioma (n=1), sarcoma (n=7), and non-small-cell lung cancer (n=2). There were distant metastases of colon and cervical cancer in one patient each. Soft-tissue reconstruction was carried out using primary closure (n=1), external oblique flap (n=1), pectoralis major myocutaneous flap (n=3), latissimus dorsi myocutaneous flap (n=18), vertical or transversal rectus abdominis myocutaneous flap (n=9), free tensor fascia lata- flap (n=6), trapezius flap (n=1), serratus flap (n=1), and one filet flap. In 15 reconstructive procedures microvascular techniques were used. An average of 3.4 ribs were resected. Stability of the chest wall was obtained with synthetic meshes. The latissimus dorsi flap is considered the flap of choice in chest wall reconstruction. However, alternatives such as pectoralis major flap, VRAM/TRAM flap, free TFL flap, and serratus flap must also be considered. Low mortality and morbidity rates allow tumor resection and chest wall reconstruction even in a palliative setting.
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PMID:[Plastic surgical reconstruction of extensive thoracic wall defects after oncologic resection]. 1778 94


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