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) of the vulva is a rare, low- to intermediate-grade sarcoma of dermal origin. This case report represents the 13th reported patient with vulvar DFSP and the first reported patient with metastatic vulvar DFSP. Although metastasis is rare, the possibility remains that the frequent recurrence of DFSP will eventually metastasize. Therefore, patients must be followed up on a long-term basis to monitor the progress of this potentially lethal cancer.
 ...
PMID:Metastatic dermatofibrosarcoma protuberans of the vulva. 982 80

Dermatofibrosarcoma protuberans (DFSPs) are an uncommon malignancy that commonly recur, but rarely metastasize. The origin of DFSPs is controversial; however, they stain with the progenitor marker CD34. DFSPs usually show increased stromal mucin, mainly hyaluronic acid (HA). HA increases cellular proliferation, delays differentiation and increases cellular motion. We evaluated the pretreatment of DFSPs with intralesional injections of hyaluronidase (HD) prior to the surgical excision. Five of nine cases of DFSPs were pretreated with HD. In HD-pretreated cases the margins for excision of the residual tumor were reduced. HD pretreatment also decreased CD34 staining and increased polarizable collagen in the residual tumor.
 ...
PMID:Pretreatment with intralesional hyaluronidase prior to excision of dermatofibrosarcoma protuberans. 983 27

Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous tumor that occurs rarely on the extremities. This tumor has a particular propensity for local recurrence following seemingly adequate excision. Metastases are rare, and usually follow repeated local recurrences. On rare occasions, fibrosarcomatous change may arise in a DFSP, and appears to affect adversely the prognosis. The adequacy of the initial resection is the most important prognostic factor; however, suggested margins vary between 1.5 and 5 cm. The ideal margin of resection on the foot is unknown. We report a patient with recurrent, acral DFSP with fibrosarcomatous change and pulmonary parenchymal metastases in a 48-year-old black male.
 ...
PMID:Acral dermatofibrosarcoma protuberans with metastases. 1002 91

Superficial, especially cutaneous soft tissue tumors deserve attention because of their special biology, often with better prognosis than their deep counterparts. This fact also has been considered in the TNM Classification/UICC, as well as in the Typing of Soft Tissue Tumors/WHO.-Basically all types of soft tissue tumors can also be found in subcutaneous and cutaneous localisation. Dermatofibrosarcoma protuberans, however, is a typical superficial tumor involving cutis and frequently the superficial subcutis. Superficial leiomyosarcoma and atypical fibroxanthoma are examples of sarcomas, which in spite of histological criteria, which generally are associated with high grade malignancy, behave as low grade malignant tumors, i.e. chiefly with risk of recurrency albeit negligible risk of metastases. Angiosarcoma and epitheloid sarcoma, on the other hand, may also metastasize, even if restricted to the cutis. Here histological criteria as numerous mitoses, cellular atypia and tumor necrosis, may additionally influence the prognosis. In conclusion it is evident that exact tumor classification is the basic requirement for an optimal, prognosis-adapted therapy. Prerequisite, however, is a precise clinico-pathological correlation to understand the biology of the different tumor entities.
 ...
PMID:[Cutaneous and subcutaneous soft tissue tumors]. 1009 23

Dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous areas (DFSP-FS) is differentiated from ordinary DFSP by its unfavourable prognosis. We carried out sequential analysis of numerical chromosomal abnormalities in two cases of DFSP during their progression to metastatic disease with FS areas (DFSP-M-FS). They were compared with nine cases of ordinary DFSP and three cases of DFSP-FS, but without metastases. Numerical chromosomal changes were examined by fluorescence in situ hybridization (FISH) using alpha-satellite centromeric probes for chromosomes 1, 8, 11 and 17. Numerical imbalances of chromosome 1 were not clarified. A gain of chromosome 8 was demonstrated in the two cases of DFSP-M-FS. A gain of chromosome 11 was observed in one of the two cases of DFSP-M-FS and in one case of DFSP-FS. A gain of chromosome 17 was demonstrated in both metastatic tumours and in recurrent tumours in two cases of DFSP-M-FS, in addition to two cases of DFSP-FS and four cases of ordinary DFSP with recurrent tumours or large tumours. This study raised the hypothesis that a gain of chromosome 17 developed in recurrent or large-sized DFSP, which occurs in high-risk groups with the possibility of a progression to FS.
 ...
PMID:A progression to dermatofibrosarcoma protuberans with a fibrosarcomatous component: a special reference to the chromosomal aberrations. 1044 61

Dermatofibrosarcoma protuberans (DFSPs) are uncommon skin tumors that have a high incidence of local recurrence even with wide surgical margin, but DFSPs rarely metastasize. Previous reports have suggested that DFSPs may enlarge more rapidly during pregnancy. We report 3 additional cases of DFSPs that showed accelerated growth during pregnancy. Immunohistochemical stains for CD34, S-100 protein, factor XIIIa, and estrogen and progesterone receptors were performed on biopsy specimens. The tumors in all 3 patients, and 4 additional DFSPs from 2 male and 2 female subjects, showed expression of progesterone receptor. As with many other stromal neoplasms, DFSPs appear to express low levels of hormone receptors, which may be one factor that accounts for their accelerated growth during pregnancy.
 ...
PMID:Accelerated growth of dermatofibrosarcoma protuberans during pregnancy. 1053 46

Dermatofibrosarcoma Protuberans (DFSP) is an uncommon cutaneous soft tissue tumor. It is locally invasive and is known to recur. Metastases are rare and occur most commonly to the lungs. Metastasis to lymph nodes is extremely rare with only a few case reports in literature. The management strategy for such lymph node metastases remains controversial because of rarity of the condition. We report a case of DFSP of the lower limb with definite inguinal node secondaries managed by loco-regional surgery. We recommend that block dissection of lymph nodes should be performed in all cases with secondaries. J. Surg. Oncol. 1999;72;178-180.
 ...
PMID:Dermatofibrosarcoma protuberans metastasizing to lymph nodes: a case report and review of literature. 1056 66

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive tumor with a low potential for distant metastases. We report a 22-year-old female patient with a typical cutaneous DFSP who developed five local recurrences followed by left cavernous sinus metastasis at the eighth year and right cavernous sinus and lung metastases at the ninth year. In each local recurrence the tumor showed histological signs of progression as being more cellular, having higher mitotic index and being aggressively invasive through the underlying soft tissues. The histopathological evaluation of the metastatic tumor resected from the left cavernous sinus revealed dedifferentiation from low-grade DFSP to higher grade fibrosarcomatous morphology. Immunohistochemical studies of the primary tumor and also the recurrent and metastatic tumors showed diffuse CD34 positivity in all specimens and p53 positivity was detected in the metastatic tumor resected from left cavernous sinus.
 ...
PMID:Dermatofibrosarcoma protuberans metastasizing to cavernous sinuses and lungs: a case report. 1177 65

A female patient with progressive systemic sclerosis and pulmonary fibrosis had a dermatofibrosarcoma protuberans on the right thigh. After resection of the tumor, new lesions occurred in the scar, and wide excision was repeated. Two years later, a lung metastasis was discovered, and segmental resection was done. After 1 year of follow-up, no local recurrences or metastases were found.
 ...
PMID:Dermatofibrosarcoma protuberans with lung metastasis in a patient with progressive systemic sclerosis. 1190 8

Dermatofibrosarcoma protuberans (DFSP) is a rare superficial sarcoma usually affecting the trunk, with significant risk of local recurrence. It is characterized by the presence of ring chromosomes or chromosomal translocations fusing the promoter of the collagen gene COL1A1 to the platelet-derived growth factor beta-chain gene PDGFB, increasing the production of PDGF locally and promoting autocrine or paracrine tumor growth. Fewer than 5% of patients with DFSP develop metastatic sarcoma, with a poor subsequent prognosis. Imatinib (STI-571) was developed as an inhibitor of the PDGF receptor tyrosine kinase and has proven clinical activity against chronic myelogenous leukemia (expressing bcr-abl) and gastrointestinal stromal tumors (expressing c-kit). We describe 2 patients with metastatic and unresectable metastases from DFSP treated with imatinib. After confirmation of negative CD117 status of 2 sarcomas arising from DFSP, patients were given imatinib 400 mg po qd and assessed at regular intervals for their tolerance and response to therapy. One patient had a transient response, then progressed rapidly and died of disease. Another patient showed a partial response to therapy after 2 months, with resolution of superior vena cava syndrome and shrinking of metastatic lung lesions. His response is ongoing after 6 months of therapy. These clinical data confirm findings from models of DFSP and support the use of imatinib in the rare setting of metastatic DFSP. Imatinib may be useful for patients with locally advanced DFSP, when other options for local therapy are limited.
 ...
PMID:Differential sensitivity to imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans. 1220 98


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