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 locally aggressive histiocytic tumor that, on rare occasions, may metastasize to regional lymph nodes or even to distant sites. We report a dermatofibrosarcoma protuberans with lymph node involvement in a 27-year-old woman. This tumor recurred repeatedly after local excisions, and ten years after the first resection, widespread pulmonary metastases developed in the patient and she died four years later. More than 400 patients with dermatofibrosarcoma protuberans have been reported in the literature; including the present case, five of these patients had lymph node metastases, 17 patients had hematogenous spread, and three had both lymphatic and blood-borne metastases.
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PMID:Dermatofibrosarcoma protuberans with lymph node and pulmonary metastases. 64 79

A case of dermatofibrosarcoma protuberans of the lower extremity with metastasis to an inguinal lymph node appearing 5 years after wide excision and skin grafting of the primary lesion is presented. The world literature is reviewed. This case is the 24th instance of metastasis and the 7th case of lymphatic metastasis. The other 17 cases were hematogenous metastases. The clinical and pathologic features of dermatofibrosarcoma protuberans are reviewed, and treatment is discussed, with the aim of emphasizing the need for long-term followup examination of the regional lymph nodes following wide and deep local excision.
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PMID:Dermatofibrosarcoma protuberans metastatic to a regional lymph node. Report of a case and review. 119 74

Dermatofibrosarcoma protuberans (DFSP) is a rare tumor of the skin with a strong tendency to recur locally. Nineteen cases of DFSP are presented. In eight of them a total of 20 local recurrences occurred, in five after irradical and in three after 'narrow' excisions. After wide excision (greater than 2 cm) for primary or wide re-excision for recurrent tumor, all patients remained free of tumor with a mean follow-up of 13.2 years (range 2-28 years). An extensive literature review revealed 913 cases of DFSP. The overall recurrence rate is about 50%; after adequate wide excision, 13%. Recurrent tumor is safely treated by wide re-excision and the recurrence rate is then 12%. Regional and distant recurrences are infrequent. Eleven cases (1%) were reported to have regional lymph node metastases and 37 (4%, 17 of whom were histologically confirmed) distant metastases, principally in the lung. The prognosis after appearance of regional or distant recurrence is bad. The role of radiotherapy in the management of this tumor is unclear. Primary or recurrent DFSP is best treated by surgical excision with a minimal margin of 2- preferably 3-cm of surrounding skin including the underlying fascia. Elective lymph node dissection is not advised.
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PMID:Dermatofibrosarcoma protuberans: treatment and prognosis. 160 35

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

The clinicopathological features and results of surgical treatment of 20 patients with dermatofibrosarcoma protuberans (DFSP) were reviewed. All patients have been observed until the present time or death. The primary treatment usually consisted of marginal, wide or radical excision. Re-excision because of recurrence was performed in six of the 20 patients who were treated primarily only by marginal excision. Following re-excision two patients have remained disease-free until the present, the other four of these six patients had a second recurrence. Three of these four patients with a second relapse were successfully treated by excision. Although six patients out of 20 had 13 recurrences at the site of primary therapy, no patient developed lymph node or distant metastases. To date, none of the remaining 14 patients has had recurrence, all having been treated by excision of from 1 to 5 cm, the mean follow-up time was 8.75 years. Based on our data, the high recurrence rate after marginal surgical treatment implies an initial radical resection.
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PMID:Surgical treatment of dermatofibrosarcoma protuberans. A retrospective study of 20 cases with review of literature. 193 91

Report of a dermatofibrosarcoma protuberans (DFSP) of the face with a follow-up of more than 40 years in a 63-year-old women. A total of 64 operations and a radiotherapy were performed to treat the recurrences, which increased in number in the last 9 years. Defects were mostly closed primarily, with free grafts or local flaps. Two metastases, probably of yatrogenic origin, were found at the skin of the clavicula and the breast one year after a Bakamjian flap at the former site of the drains. Histology revealed mature fibroblasts growing in bundles, cart-wheel or storiform with destructive infiltration of the dermis. The DFSP is mainly a locally malignant tumor, growing slowly and painless as a flesh-coloured multinodular mass localised in the dermis. Local recurrence occurs in more than 50% of the cases, whereas metastases are very rare.
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PMID:[Dermatofibrosarcoma protuberans of the face. A case report]. 202 46

Fibrous tumors of the soft tissue are usually benign, but some fibrous neoplasms such as dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), and malignant fibrohistiocytoma (MFH) can be very destructive locally with a high recurrence rate after local excision. On occasion, they can metastasize. Previous reports have confirmed the high success rate of Mohs micrographic surgery for the treatment of DFSP, but data have been lacking on the potential benefit of this surgical approach for MFH and AFX tumors. Over the past 6 years, we have treated 17 patients with MFH (20 tumors) and 5 patients with AFX with Mohs micrographic surgery. A retrospective analysis of the surgical results is presented. To date (average 3-year follow-up), all patients contacted are tumor free with only one recurrence; no patient has developed metastatic disease. Our results to date are very encouraging; they lend support to Mohs micrographic surgery as a desired surgical approach for these difficult-to-cure neoplasms.
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PMID:Treatment of malignant fibrous histiocytoma and atypical fibrous xanthomas with micrographic surgery. 255 64

The case of a 21-year-old man with dermatofibrosarcoma protuberans of the scalp that spread to deep cervical lymph nodes is presented. Available literature is reviewed, and the infrequency of metastasis is discussed. Since cases of metastases almost always involve recurrent lesions, the importance of wide and deep resection in the initial operation cannot be overemphasized.
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PMID:Dermatofibrosarcoma protuberans with regional lymph node metastasis: a case report. 258 12

The wide range of oncogenic proliferative potentials of the fibroblast is demonstrated with a series of eight patients. Diagnoses included infantile digital fibromatosis, "aggressive fibromatosis," aggressive fibromatosis progressing to poorly differentiated sarcoma, infantile myofibromatosis, recurrent desmoid tumor, fibrosarcoma arising in a keloid, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma of left atrium. Still other types of fibroblastic tumefactions might have been included. Oncogenic factors that may have been operative in the causation of the lesions presented include: genetic factors, sex-linked factors, hormonal factors, numerous growth factors, and certain viruses, especially retroviruses. Certain fibromatoses in children are commonly self-limited and need only be monitored carefully as the process regresses. Aggressive fibromatosis, on the other hand, can prove fatal if the lesion is not completely resected with a wide margin and, occasionally, the process may become frankly malignant, with metastases. The standard triad of excisional surgery, radiotherapy, and chemotherapy has been used to treat frankly malignant fibrous tumors with variable results.
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PMID:The ubiquitous fibroblast. Multiple oncogenic potentials with illustrative cases. 303 75

From 1964 to 1984, 87 patients with soft tissue sarcoma (excluding lymphreticulum system sarcoma) in the head and neck were treated in our hospital. All were proved by pathology. The incidence rate of fibrosarcoma was the highest (36.8%), hemangiosarcoma the lowest (5.8%). There was no liposarcoma or synovial sarcoma in this group. The most frequently involved site was the area near the nasal cavity and maxillary sinus for fibrosarcoma (66%), the base of tongue for hemangiosarcoma (60%), scalp for dermatofibrosarcoma protuberans (54%), parapharyngeal space and soft tissue of the neck for neurogenic sarcoma (45%). Metastasis rate to the lymph nodes was 16-20% for rhabdomyosarcoma, hemangiosarcoma and malignant fibrohistiocyte tumor. No distant metastasis was found in dermatofibrosarcoma protuberans and malignant fibrohistiocyte tumor. Distant metastasis rate was 10-20% for the other types. Local recurrence rate was 9.1% for dermatofibrosarcoma protuberans, 88.9% for malignant fibrohistiocyte tumor, 30-66.7% for the other sarcomas. The 5- and 10-year survival rates were 20% and 0%, 37.5% and 0%, 91.6% and 91.6% for rhabdomyosarcoma, malignant fibrohistiocyte tumor and dermatofibrosarcoma protuberans. There were differences in clinical behavior for soft tissue sarcomas in the head, neck and in the trunk. Highly malignant sarcomas, such as liposarcoma, synovial sarcoma and rhabdomyosarcoma were rare in the head and neck but the low malignant sarcomas in the head and neck were 3-10 times as common as those in the trunk. Distant metastasis rate of the fibrosarcoma was 20% in head and neck and 6.7%in trunk, the 5- and 10-year survival rates were 36% and 77.9%, 25% and 73.6%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Soft tissue sarcoma in the head and neck--analysis of 87 patients]. 324 87


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