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)

Dermatofibrosarcoma protuberans is a rare low-grade fibrohistiocytic tumor with onset normally at ages between 20 and 50 years. It presents as a violaceous plaque or macule with an appearance suggestive of vascular lesion, on which nodular lesions appear later. Histological diagnosis is based on the presence of a spindle-cell tumor arranged in small bundles in a characteristic cartwheel pattern. The local recurrence rate is high but metastases are rare. The treatment is surgical resection with wide margins. The tumor rarely affects children under 16 years of age and it is even less common at birth--only 27 congenital cases have been described in the literature. We describe the case of a 10-year-old boy with dermatofibrosarcoma protuberans present since birth and currently without signs of recurrence.
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PMID:[Congenital dermatofibrosarcoma protuberans: a case report]. 1796 51

We present a rare case of a dermatofibrosarcoma protuberans arising on the dorsum of the hand in a 51-year-old woman, who had experienced four recurrences of the tumour following local excisions. We performed a radical surgical excision of the lesion and covered the defect with a distal ulnar artery skin island flap. Adjuvant radiation therapy followed the surgical treatment. Forty months postoperatively, the patient has a functional hand without signs of recurrence and no evidence of disease. Wide surgical excision with margins of 2.5-3 cm is the optimal treatment for dermatofibrosarcoma protuberans. For selected patients, like those presenting large tumours involving the hand, adequate removal is not easily obtainable, or may result in major functional deficits. An alternative strategy may be the combination of less extensive surgery and radiotherapy, in order to prevent mutilation, decrease the local recurrence rates, and minimize the risk of metastases.
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PMID:Multiple recurrent dermatofibrosarcoma protuberans of the hand. 1857 14

Dermatofibrosarcoma protuberans (DFSP) is a cutaneous low-grade malignancy with a high recurrence rate that rarely generates distant metastases. In most cases this tumor is associated with a chromosomal translocation involving the COL1A1 gene on chromosome 17 and the platelet-derived-growth-factor B gene on chromosome 22, generating a fusion gene that constitutively activates the PDGF receptor (PDG-FR). In the early stages of disease traditional surgery (wide excision) or Mohs micrographic surgery represent the standard of care. When surgical margins are positive, postoperative radiotherapy is a valuable option. Recently it has been shown that inhibiting PDGFR with imatinib can induce a high response rates in case of unresectable or metastatic disease. This targeted agent now represents the therapy of choice of advanced DFSP and it is the fi rst great therapeutic success in this disease after unsuccessful years using cytotoxic drugs. It is likely that a better knowledge of molecular biology of DFSP could, as it was the case for GISTs, may improve treatment results leading to the development of new targeted agents.
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PMID:[Molecular approach in the treatment of dermatofibrosarcoma protuberans]. 1899 38

Dermatofibrosarcoma protuberans (DFSP) is a locally malignant neoplasm derived from fibrous tissue. Similar picture of such lesions to malignant tumors as well as to benign changes creates a risk of too gentle treatment and consequently of recurrence and malignant transformation of the lesions with possible remote metastases. Therefore accurate diagnosis and aggressive surgical treatment can prevent the recurrence of the tumor. Authors of the study present case report of 49-year-old woman with DFSP of vulvar area.
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PMID:[Dermatofibrosarcoma protuberans of the vulva]. 1917 38

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant dermal neoplasm characterized by slow infiltrative growth, little metastatic potential but a high tendency to recur locally after surgical excision. DFSP is associated with a high cure rate. The optimal therapy is complete surgical resection. The recurrence potential of DFSP is directly related to the extent of resection. The need for wide excision margins has been amply documented. Wide local excision is a frequently used practice. Mohs micrographic surgery with continuous histological margin control is further propagated to reduce local recurrence rates. In more than 90% of DFSP, a specific chromosomal aberration is described, involving Chromosomes 17 and 22. It leads to a constitutive activation of the platelet-derived growth factor receptor (PDGFR) followed by continuous stimulation of the tumor cell growth. The use of targeted inhibitors of PDGFR is a good therapeutic option in the treatment strategy of unresectable locally advanced, recurrent or metastatic disease. With Imatinib, a selective PDGFR tyrosin kinase inhibitor, partial and complete remissions of DFSP could be achieved. This article reviews the current opinion and literature about DFSP and resulting therapy strategies.
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PMID:Current treatment options in dermatofibrosarcoma protuberans. 1920 37

Dermatofibrosarcoma protuberans (DFSP) is a morphologically distinct dermal/subcutaneous fibrohystiocytic neoplasm seen mainly on the trunk and the proximal extremities, which generally is regarded as a low-grade or "borderline" neoplasm. Dermatofibrosarcoma protuberans is characterized clinically by locally aggressive growth and a high rate of local recurrence, but distant metastases and tumor-related deaths are very rare. DFSP in the breast is extremely uncommon. Immunohistochemically, DFSP is characterized by positive staining for vimentin and CD34. We present a case of a 65 years old female patient with a giant DFSP developed in the sight of several excised fibroadenoma and neurofibroma treated by radical mastectomy and axillary block-dissection. Perioperative hystological and immunohistochemical images demonstrate the characteristics of the giant nonphylloid sarcoma.
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PMID:[An uncommon malignancy of the breast: dermatofibrosarcoma protuberans with fibrosarcomatous transformation (DFSP-FS)]. 1921 66

In high-grade musculoskeletal sarcomas, adjuvant chemotherapy is often performed to prevent distant metastases. As the efficacy of chemotherapy varies according to the histological type of sarcoma, its indication is determined according to the histological type and the stage. Prognoses are poor in patients with osteosarcoma, Ewing's sarcoma, or rhabdomyosarcoma, when surgery alone is performed. However, because these sarcomas are chemosensitive, their prognoses are improved with adjuvant chemotherapy, so it is absolutely necessary. Drugs commonly used for osteosarcoma include adriamycin, cisplatin, methotrexate, vincristine, and ifosfamide. For Ewing's sarcoma and rhabdomyosarcoma, vincristine, actinomycin-D, cyclophosphamide, etoposide, and ifosfamide are commonly used. On the other hand, the efficacy of chemotherapy is unclear in most of the non-round cell sarcomas, e. g., malignant fibrous histiocytoma, pleomorphic liposarcoma, and leiomyosarcoma, so adjuvant chemotherapy is relatively indicated and often performed preoperatively. The efficacy is evaluated by reduction of the tumor volume as a surrogate marker. Postoperative chemotherapy is performed when the preoperative chemotherapy is effective. Nowadays, several kinds of antitumor agents are usually used for non-round cell sarcomas, and many authors have reported various kinds of regimens and their clinical results. Among them, the key drugs are adriamycin and ifosfamide. Recently, taxanes and gemcitabine are sometimes used. For chemoresistant sarcomas, e. g., chondrosarcoma, chordoma, alveolar soft part sarcoma, chemotherapy is rarely indicated, even if the tumor is histologically high grade and large. Low-grade musculoskeletal sarcomas, e. g., low-grade chondrosarcoma, central low-grade osteosarcoma, parosteal osteosarcoma, well-differentiated liposarcoma, and dermatofibrosarcoma protuberans, are well cured only by surgical excision, and adjuvant chemotherapy is therefore not indicated. Superficially-located, small-size non-round cell sarcomas, even though histologically high grade, are well healed only by surgical excision, and adjuvant chemotherapy is rarely indicated.
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PMID:[Indication of chemotherapy according to histological type of musculoskeletal sarcomas]. 1922 36

Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignancy of the skin and subcutaneous tissues that only rarely forms distant metastases. More than 90% of cases are associated with a chromosomal translocation involving the COL1A1 gene on chromosome 17 and the PDGFB gene on chromosome 22. Management of this disease is primarily surgical with excellent rates of local control obtained using either wide local excision or Mohs micrographic surgery. Data have recently shown that inhibiting platelet-derived growth factor receptors (PDGFR) with imatinib mesylate can induce high rates of clinical response in patients with unresectable or metastatic DFSP. Although wide surgical excision remains standard care, patients with locally advanced disease not suitable for surgical excision can be treated with imatinib mesylate, which sometimes allows residual DFSP to be surgically excised.
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PMID:[New molecular approaches in dermatofibrosarcoma protuberans]. 1969 63

Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor with tendency to recur locally and only rarely metastasizes to vital organs. Surgery with wide margins remains the standard treatment. DFSP is characterized by specific chromosomal abnormalities involving the platelet derived growth factor B locus (PDGFB). In the majority of cases a supernumerary ring chromosome containing amplified t(17; 22) translocation or a linear unbalanced t(17; 22) containing the COL1A1 -PDGFB fusion gene is present. This molecular event causes aberrant expression of a functional PDGFB leading to activation of PDGFR. Imatinib mesylate is a tyrosine kinase inhibitor with activity against activated PDGFR, and has significant activity against DFSP. Clinical evidence suggests that it has a role in locally advanced and metastatic disease and clinical trials are ongoing examining its role in this rare but potentially fatal sarcoma.
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PMID:Imatinib in the treatment of dermatofibrosarcoma protuberans. 1970 5

Dermatofibrosarcoma protuberans (DFSP) is a low-grade sarcoma seldom seen in the vulva with only 29 cases reported. We present the clinicopathologic and immunohistochemical features of 13 such cases seen in our institution over a period of 29 years (1978 to 2007). Patient age ranged from 23 to 76 years (mean, 46 y). Twelve patients had a vulvar mass. One patient presented with a pigmented skin lesion. Tumor size ranged from 1.2 to 15 cm (median, 4 cm). Microscopically, all the cases showed typical features of DFSP. In 1 case, myxoid changes were also noted; 3 cases showed fibrosarcomatous transformation. Of interest, in 7 of our 13 cases, a variety of diagnoses, such as cellular dermatofibroma, cellular leiomyoma, neurofibroma, low-grade leiomyosarcoma, fibrosarcoma, low-grade malignant schwannoma, desmoplastic melanoma, cellular neurofibroma, and low-grade malignant peripheral nerve sheet tumor were initially considered. All 11 cases tested for CD34 were positive, whereas 7/9 cases, 8/9 cases, and 9/9 cases were positive for PDGFR-alpha, PDGFR-beta, and c-abl, respectively. All patients were initially treated with excisional biopsy, wide local excision, or radical vulvectomy. Local recurrences occurred in 7 cases. One patient also developed distant metastases. All recurrences were treated surgically; 1 patient also received chemotherapy and radiotherapy and another received imatinib (Gleevec). Follow-up data ranging from 2 to 444 months was available for all patients. Nine patients had no evidence of disease, 2 patients were alive with disease, 1 patient had died of disease, and 1 patient had died of other causes. DFSP affects women of a wide age range and has a propensity to recur locally. The frequent expression of PDGFR-alpha, PDGFR-beta, and c-abl in these cases agrees with the findings of other investigators and supports the use of imatinib (Gleevec) in cases that are recurrent or not amenable to surgery.
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PMID:Dermatofibrosarcoma protuberans of the vulva: a clinicopathologic and immunohistochemical study of 13 cases. 2013 58


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