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

Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon fibrohistiocytic tumor that is locally aggressive. DFSP is associated with frequent local recurrences but rarely metastasizes. It has been suggested that fibrosarcomatous change within a dermatofibrosarcoma protuberans (DFSP-FS) is associated with increased risk of local recurrence and distant metastases. Metastases remain rare, however, and are typically preceded by multiple local recurrences. We report a rare case of a DFSP-FS metastasis to the head of the pancreas without any preceding local recurrence.
 ...
PMID:Metastatic potential of dermatofibrosarcoma protuberans with fibrosarcomatous change. 1261 65

Dermatofibrosarcoma protuberans has a high rate of recurrence, presumably as a consequence of persistent residual tumour. This is probably the main reason why repeated surgical treatment is frequently required. In the article we present our experience of surgical treatment of such malignancy on the neck and shoulder in the elder male after ten years follow-up period. The case is interesting because we started with aggressive treatment relatively late in the unfavourable, fully-developed stage of the disease, with multiple satellite nodules spreading and signs of partial myxoid degeneration of the largest tumour mass itself. Despite initial wide excision, five local recurrences--fortunately without regional or distant metastases--were observed during the first eight years. The recurrences appeared mostly in the region of greatest tumour degeneration and infiltration and where the excision margins were less than 2 cm apart from the visible tumour mass. A sufficiently wide excision, long-term follow-up and good co-operation between surgeon and experienced pathologist are crucial for the strategy of treatment and the prognosis of this unusual malignancy.
 ...
PMID:Dermatofibrosarcoma protuberans: our experience after ten years of patient's surgical follow-up. 1292 Dec 58

A 37-year-old man was diagnosed with metastatic dermatofibrosarcoma. Dermatofibrosarcoma protuberans is a rare skin tumour with a strong tendency for infiltrative growth, resulting in a high rate of local recurrences; metastatic disease develops in approximately 5% of patients, especially in the lungs. On the basis of some recent publications about the use of imatinib in patients with dermatofibrosarcoma, the patient was prescribed this drug. The treatment resulted in a partial response. Imatinib is the first registered selective tyrosine-kinase inhibitor for treatment of chronic myeloid leukaemia. The inhibition of tyrosine kinase interrupts the proliferative signal transduction.
 ...
PMID:[Good response to treatment with the selective tyrosine-kinase inhibitor imatinib in a patient with metastatic dermatofibrosarcoma protuberans]. 1460 47

Dermatofibrosarcoma protuberans is a rare soft tissue cancer of the skin with the potential for intermediate malignity, characterized by local invasion and recurrence. Presenting with local atrophic and sometimes erythematous plaques, these lesions are best treated with surgery in which large excisions are necessary to reduce the risk of recurrence and to prevent rarely seen metastases. We report on a 26-year-old female patient with dermatofibrosarcoma protuberans on her breast, an very unusual site. As a novel surgical approach to treat the patient's tumor, a keyhole pattern was used, allowing wide safety margins during resection, while preserving the cosmetic appearance of the breast.
 ...
PMID:Dermatofibrosarcoma protuberans of the breast. 1462 60

In July 1994, a 27 year-old man presented with a twice recurrent, pigmented tumor on his left wrist. Wide excision and histological examination showed a pigmented dermatofibrosarcoma protuberans (PDFSP) or Bednar tumor with foci of fibrosarcomatous (FS) change. Immunohistochemistry helped to distinguish it from cellular blue nevus and malignant melanoma. In January 1997, he presented with 3 swellings on his thighs which showed features of fibrosarcoma. In March 1998, he developed multiple tumors on his thighs, chest wall, supraclavicular region, parapharyngeal region and two lung nodules. To the best of our knowledge, there are only 3 previously published reports of metastases in patients with Bednar tumor. We present the fourth such case.
 ...
PMID:Fibrosarcomatous Bednar tumor with distant metastases--a case report. 1547 Nov 19

In the current review, the authors set out to discuss the natural history and treatment of dermatofibrosarcoma protuberans (DFSP), a rare indolent cutaneous tumor. Approximately 10-15% of all DFSPs contain areas of fibrosarcoma (DFSP-FS), and such cases tend to exhibit more aggressive behavior. The optimal treatment for DFSP is resection with wide margins; the likelihood of local control associated with this procedure exceeds 90%. The probability of regional or distant metastases is </= 5%. Patients with positive or close surgical margins have an elevated risk of local recurrence after resection alone; however, postoperative radiotherapy results in local control rates of >/= 85% in such patients. Postoperative radiotherapy also is indicated in the rare event that a patient has unresectable macroscopic disease. Experience with the use of radiotherapy alone to treat macroscopic disease is limited.
 ...
PMID:Dermatofibrosarcoma protuberans. 1550 5

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive cutaneous tumor that usually does not metastasize. Clinically, it may present as a reddish macule to protuberant nodule affecting the trunk and proximal extremities of young and middle-aged adults. Histologically, it is characterized by a monomorphous storiform proliferation of spindle cells, involving the dermis and hypodermis, with a honeycomb pattern of infiltration of the subcutaneous fat. In case of difficulty in diagnosis, immunohistochemical markers (especially CD34) are highly sensitive for DFSP. It is important to remember that the rarity of DFSP, its resemblance to hypertrophied scars and keloids, and its appearance at trauma sites sometimes make the diagnosis of DFSP difficult and delayed. In the literature, DSFP was not described in a child until 1957. Here we report an instance of DFSP at an unusual site in a 9-year-old child, which was the location of a previous central venous line insertion in the left supraclavicular area. A complete excision of the tumor with a wide surgical margin of 3cm of visibly uninvolved tissue was performed, followed by a deltopectoral flap and skin split graft. Postoperatively, the patient's general condition was stable and he was discharged after 5 days to be followed up 3 months later in our clinic.
 ...
PMID:Dermatofibrosarcoma protuberans at the site of a central venous line. Case report. 1567 91

Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor which may recur locally and rarely causes metastases to vital organs. DFSPs have specific chromosomal abnormalities involving the platelet-derived growth factor beta-chain locus (PDGFB) which may render these tumors responsive to targeted therapy with the tyrosine kinase inhibitor imatinib mesylate. A patient with locally recurrent and metastatic DFSP resistant to first-line chemotherapy was treated with imatinib mesylate 400 mg/day. The tumor was examined by a novel fluorescence in situ hybridization (FISH) method for specific rearrangements of the PDGFB locus. The patient was followed for response and toxicity by physical examination and imaging studies. FISH revealed PDGFB rearrangement indicative of multiplication of the PDGFB fusion locus within a ring chromosome. Physical examination showed response within the first month of treatment, and subsequent computed tomography and fluorodeoxyglycose positron emission tomography documented complete response to imatinib therapy. Our patient is now in sustained complete remission for 20 months with minimal toxicity. We conclude that sustained complete remission of metastatic DFSP with specific FISH abnormalities involving the PDGFB locus can be obtained with imatinib mesylate with minimal toxicity for the patient.
 ...
PMID:Sustained complete remission of metastatic dermatofibrosarcoma protuberans with imatinib mesylate. 1574 84

A subset of low-grade fibrosarcomas is composed of CD34-positive spindle cells. These include dermatofibrosarcoma, its morphologic variants, and its associated fibrosarcoma, solitary fibrous tumor, hemangiopericytoma and their malignant counterparts, and some cases of myxoinflammatory fibroblastic sarcoma. Dermatofibrosarcoma and related lesions are characterized by a t(17;22)(q22;q13) rearrangement resulting in fusion of the genes COL1A (17q21-22) and PDGFB1 (22q13). Solitary fibrous tumor displays varying cellularity and fibrosis and a peripheral hemangiopericytomatous pattern; most tumors formerly called hemangiopericytoma are now subsumed into the category of solitary fibrous tumor, although a few strictly defined examples are recognized; however, these are probably not composed of pericytes. Myofibroblastic malignancies are best identified by electron microscopy, with which varying degrees of differentiation, including the presence of fibronexus junctions, can be identified. Low-grade sarcomas showing myofibroblastic differentiation include myofibrosarcomas and inflammatory myofibroblastic tumors. Myofibrosarcomas are spindle cell neoplasms that occur in children or adults in the head and neck, trunk, and extremities as infiltrative neoplasms and that display a fascicular or fasciitis-like pattern with focal nuclear atypia and variable expression of myoid antigens. These sarcomas are prone to recurrence and a small number metastasize. Inflammatory myofibroblastic tumor (synonymous with inflammatory fibrosarcoma) is a neoplasm arising predominantly in childhood, and frequently in intraabdominal locations. It has spindle cells in fascicular, fasciitis-like and sclerosing patterns, with heavy chronic inflammation including abundant plasma cells. Many IMT have clonal chromosomal abnormalities involving 2p22-24, and fusion of the ALK gene with tropomyosin 3 (TPM3-ALK) or tropomyosin 4 (TPM4-ALK) is found in a subset.
 ...
PMID:Low-grade sarcomas with CD34-positive fibroblasts and low-grade myofibroblastic sarcomas. 1576 78

The hallmark characteristics of cancer include an unrestrained proliferation involving activation of growth signals, loss of negative regulation and dysfunctional apoptotic pathways. Targeting abnormal cell signalling pathways should provide a more selective approach to cancer treatment than conventional cytotoxic chemotherapy. Tyrosine kinases play an essential role in the signalling pathways involved in the control of cellular proliferation and growth. Imatinib is a small-molecule tyrosine kinase inhibitor of the ABL fusion gene, platelet derived growth factor receptors (PDGFR) and KIT. This agent has demonstrated considerable activity in chronic myeloid leukaemia (CML) by inhibiting the BCR-ABL fusion protein and gastrointestinal stromal tumours (GISTs), which are predominantly driven by activating mutations in KIT. A number of other rare conditions are also responsive, for example, dermatofibrosarcoma protuberans, which is driven by a chromosomal translocation involving PDGF-B and Col1A1, resulting in overexpression of PDGF-B, and hypereosinophillic syndrome, which can be caused by activating PDGFR mutations. The pivotal registration study for newly diagnosed CML was a large randomised trial comparing 400 mg/day of imatinib to a combination of IFN-alpha and cytarabine, which demonstrated a significantly higher complete haematological and cytogenetic response rate in the imatinib arm. In the case of GIST a randomised study in patients with inoperable or metastatic disease explored doses of 400 - 600mg and reported a response rate of > 50% in each arm plus disease stabilisation and an improvement in performance status. Large randomised trials have subsequently been performed, comparing 400 with 800mg/day. The first to report indicates that the larger dose is associated with improved progression-free survival, although it is not yet known whether or not this will translate into a difference in overall survival. The most common KIT mutation involves exon 11 and is associated with a statistically significant better response and prognosis compared with other mutations or no detectable mutations. Mutational analysis is likely to become increasingly important in the selection of patients for neoadjuvant and adjuvant treatment and in helping to understand the nature of acquired resistance.
 ...
PMID:The development and application of imatinib. 1579 12


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>