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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Observations were conducted on 26 patients with protruding dermatofibrosarcoma of the skin. Histological and histochemical characteristics, as well as the mitotic regimen of these tumours, were investigated. Protruding dermatofibrosarcoma is a fibroblastic tumour characterized by a slow growth, usually a big size, persistent multiple reoccurrence, late metastases into the lymphatic nodes and lungs. No regularities concerning periods and frequency of developing of reoccurrences could be established. Clinico-anatomical collations failed to show correlation between the mitotic activity of dermatofibrosarcomas and the degree of their malignancy and rate of growth of the tumour.
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PMID:[Dermatofibrosarcoma protuberans]. 101 85

A retrospective study of 57 patients with atypical fibroxanthoma of the skin is presented. The light microscopy is described and the differential diagnosis is discussed. Most of the atypical fibroxanthomas (32 out of 57 cases) were originally diagnosed as soft tissue sarcomas, e.g. fibrosarcoma, dermatofibrosarcoma, neurofibrosarcoma, myosarcoma and unspecified sarcoma. The tumour occurred chiefly in middle-aged and elderly patients; three patients were 15 years old or younger. The median age was 73 years in patients in whom the tumour occurred in the head and neck, and 34 years in patients in whom the tumour developed on the extremities and trunk. The sex ratio (male to female) was almost equal. Follow-up information about 43 patients was available. The follow-up period ranged from 1 year to 25 years with a median of 9 years. Eight patients died from intercurrent disease; all the other 35 patients are alive and well. The clinical course was benign in all but one patient in whom a recurrence developed and metastases to the regional lymph nodes appeared 7 years after the initial excision. The tumour in this case did not differ histologically from the other atypical fibroxanthomas with respect to cellularity, cellular and nuclear atypia or mitotic activity. It is suggested that the recurrence per se might be of prognostic importance.
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PMID:Atypical fibroxanthoma of the skin. A clinico-pathological study of 57 cases. 125 44

A case is presented of dermatofibrosarcoma protuberans of the gluteal region with metastasis to the lung appearing 7 years after wide excision of the primary lesion. The world literature is reviewed. The clinical and pathologic features of dermatofibrosarcoma are reviewed and treatment is discussed, with the aim of emphasizing the need for long-term follow-up examination of lymph nodes and for metastases following wide excision of these lesions.
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PMID:Dermatofibrosarcoma protuberans metastatic to the lung. A case report. 160 61

Progressive and recurrent dermatofibrosarcoma, described by Darier and Ferrand in 1924, is a fibrous skin tumour with essentially local malignancy. The authors report a case with pulmonary metastasis, a rare event as only 13 cases of visceral metastases have been reported in the literature. The clinical course of this case was favourable (follow-up of 5 years), in contrast with the usually pejorative nature of metastatic disease (death after several months to one year following the discovery of the first metastasis).
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PMID:[Pulmonary metastasis of a dermatofibrosarcoma]. 176 32

We report the case of a 56-year old male patient with pulmonary, abdominal and pelvic metastases from a dermatofibrosarcoma (DFS) which had developed 23 years previously in the right scapulo-humeral region and had recurred twice after the first excision, in 1983. Surgery was only performed once. The most remarkable feature of the disease was the progressive transformation, with each recurrence, of a typical DFS into a poorly differentiated and highly malignant tumour, about as active as a fibrosarcoma. A review of the literature yielded 31 cases of histologically proven metastatic DFS. Although the actual incidence of metastases is difficult to determine with accuracy, their frequency may be estimated at 3 p. 100 (most probably a maximum figure). The disease spreads mostly through the blood, but also sometimes through the blood and lymph. However, since lymphatic involvement is rare the usefulness of lymph node excision is extremely doubtful. Pulmonary lesions are the most frequent ones, but various other organs may be affected, although this seldom applies to the liver. Following a first excision, metastases occur within 6 years on average (range: 1 to 33 years). This mean delay is superior to the mean follow-up period for most of the important series without metastases. The histopathological picture is usually one of typical DFS but in some cases, including ours, it is much less typical and may even be frankly malignant. A few reports mention the presence of histiocytic infiltrates associated with the fibroblasts. The prognosis in patients with metastases is particularly poor: in most of the cases reported the patient died within the year following the discovery of metastatic lesions. The very high progressivity of the secondary lesions contrasts with the slow course of the primary tumour. None of the treatments tried (surgery, radiotherapy, chemotherapy) has resulted in a significant prolongation of life. No correlation can be established between the occurrence of metastases and the following parameters taken together: patient's age and sex, tim elapsed between the appearance of the tumour and the date of exicision, and histological features of the initial tumour. The development and number of recurrences seems to be the most significant predictive factor as regards the risk of metastasis. The noxious role of incomplete and/or repeated surgery creating vascular breaks, disrupting the defense barriers and encouraging malignancy in some cases has been mentioned by most authors; it emphasizes the value of the widest possible initial surgery.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Metastatic Darier-Ferrand dermatofibrosarcoma. Review of the literature apropos of a case]. 332 93

Dermatofibrosarcoma protuberans is a slow-growing but locally aggressive malignant neoplasm of the skin. The preferential locations are on the trunk and proximal extremities but it can occur in the head and neck region in about 15% of the cases. This is a report of such a case and a review of the literature of head and neck cases attempting to analyse the profile of patients and surgical prognosis factors. The recurrence rate is important (20% to 55%) and dependent on the free tissue margins. Those margins are at least 3 cm in all directions; this is difficult to assess in the head and neck region because of both functional and cosmetic aspects. Distant metastases are rare but related to a long recurrence history. This behaviour gives the impression that prognosis is poorer for dermatofibrosarcoma in the head and neck than within other locations.
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PMID:Dermatofibrosarcoma protuberans of the upper lip: an overview and a case report. 923 7

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.
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PMID:[Good response to treatment with the selective tyrosine-kinase inhibitor imatinib in a patient with metastatic dermatofibrosarcoma protuberans]. 1460 47

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.
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PMID:Low-grade sarcomas with CD34-positive fibroblasts and low-grade myofibroblastic sarcomas. 1576 78

We report the case of a young man diagnosed with dermatofibrosarcoma protuberans lung metastases seven years after primary tumor resection. Notably, no previous local recurrences had been observed. A multimodal approach was used for the management of this patient: surgery, radiotherapy and targeted therapy with Imatinib. The patient is alive with stable disease after thirty months of the metastases diagnoses. Dermatofibrosarcoma protu-berans metastasizes very rarely, and when it does, it is usually either after local recurrence or whenever fibrosarcomatous transformation is found in the histopathological analysis, which confers an increased risk of local recurrence and metastases. This is the second report of a metastatic dermatofibrosarcoma protuberans occurring in a patient with no previous local recurrence or histological fibrosar-comatous features, emphasizing the rarity of the disease presentation and the importance of targeted therapy in improving patient quality of life and survival.
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PMID:Dermatofibrosarcoma Protu-Berans with Lung Metastasis Requiring Pneumonectomy. 2678 73

Leiomyomatosis peritonealis disseminata (LPD) is typically a benign and rare disorder found in female patients, prior to menopause. It can be found in the subperitoneal or peritoneal spaces and is represented by multiple different sized myomatous nodules (smooth muscle tumors). Additionally, it has also been found in women after menopause as well as in men. Despite the fact that high levels of estrogen and progesterone play a significant role in this disorder, the mechanism behind LPD development and a definitive therapeutic concept has yet to be conceived. This disorder is mostly found incidentally during imaging or surgery as it is often an asymptomatic condition. The present case reports an incident of LPD, clinically similar to peritoneal metastases, in a patient with a past history of dermatofibrosarcoma of Darier and Ferrand.
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PMID:Leiomyomatosis peritonealis disseminata: case report and review of the literature. 2793 Nov 59


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