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)

Aggressive fibromatosis is a poorly defined, locally aggressive, yet histologically benign fibroblastic proliferative lesion that may occur in the head and neck. The lesion is highly cellular and locally infiltrative and has a propensity to invade and erode bone, compromising vital structures within the head and neck. However, it is not a true malignancy because it does not have malignant cytologic characteristics nor does it metastasize. We present two cases of aggressive fibromatosis occurring in young adult men. The first case involved a rapidly enlarging mass of the anterior maxilla that involved the upper lip, nasal alae, nasal septum, inferior turbinates, and hard palate. The patient underwent incisional biopsy to confirm the diagnosis. Because of difficulty in determining the actual margins of this extensive lesion and the significant morbidity that would have resulted from surgical resection, we elected to treat this patient with chemotherapy and radiation therapy. The second case was an extensive lesion involving the right temporal bone, pterygomaxillary space, and infratemporal, temporal, and middle cranial fossae. Incisional biopsy confirmed the diagnosis. Because of the lack of functional and cosmetic deficits and the unavoidable morbidity of a surgical resection, this patient was treated with radiation therapy. Although wide field resection is the most satisfactory form of treatment, in situations in which this modality would result in unacceptable morbidity or if surgical margins are positive, then radiation therapy and chemotherapy should be considered. Support for these therapeutic modalities is found in larger series of cases outside the head and neck.
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PMID:Nonsurgical treatment of aggressive fibromatosis in the head and neck. 190 Nov 63

Malignant soft-tissue tumors of the abdominal wall consist of desmoid tumors and nondesmoid soft-tissue sarcomas. These neoplasms tend to invade adjacent musculoaponeurotic and bony structures. Transperitoneal organ invasion can also occur with devastating sequelae, especially after an incomplete excision. Extirpation with the full-thickness of the abdominal wall is required except for small, superficial lesions. A wide-margin resection, including any adherent viscus, segments of adjacent ribs, and iliac or pubic crest, offers the best assurance of local control. Adjunctive radiotherapy can be given when the margin of resection is unavoidably limited, but an effective radiation dose can be difficult to administer, due to the sensitivity of underlying intestines. Metastases develop frequently with high-grade sarcomas; adjuvant chemotherapy may be tried in these patients, but its value remains unproven.
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PMID:Malignant soft-tissue tumors of the anterior abdominal wall. 253 Sep 9

Aggressive fibromatoses (desmoid tumours) tend to grow in an infiltrative and destructive manner without metastases. Computed tomography of aggressive fibromatoses yields no uniform attenuation pattern. The tumours are typically isodense when no contrast medium is used and enhance clearly to hyperdense during infusion of contrast medium. In magnetic resonance tomography (MRT) a high signal (long T2) on T2-weighted pulse sequences as well as an accumulation of i.v. Gd-DTPA seems to be the characteristic appearance of aggressive fibromatoses, although different signal intensities can be seen. The MRT histopathologic correlation shows increasing signal intensities on T2-weighted sequences dependent on an increment in cellular content of the tumours. Only histopathological methods can provide a definite diagnosis.
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PMID:[Imaging diagnosis of aggressive fibromatosis and the MRT-pathological correlation]. 254 14

Aggressive fibromatosis (desmoid, desmoid tumour) resembles, in its infiltrating and destructive growth, a fibrosarcoma, but does not metastasize. Because of its high recurrence rate, the tumour remains a surgical problem. Various imaging methods were evaluated retrospectively in 23 patients with histologically confirmed aggressive fibromatosis. Conventional radiological procedures are poor at demonstrating the extent and type of tumour. Modern tomographic methods are more able to determine the size of the lesion and a combination of angiography and CT can frequently provide a definite diagnosis.
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PMID:[Radiological diagnosis of aggressive fibromatosis]. 302 53

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

The authors report seven cases of desmoid tumors of the extremities. A tumour less than a few centimetres in size is best removed by wide local excision. Large growths should also be excised, but efforts should be made to preserve the vessels and nerves, since malignant transformation and metastases do not occur. Irradiation therapy should be considered for tumours which are surgically inaccessible. The problems of differential diagnosis between a desmoid tumour and a fibrosarcoma are discussed.
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PMID:Desmoid tumours of the extremities. 318 30

A case of omento-mesenterial fibromatosis in a 6-year-old boy revealing through acute intestinal obstruction is reported. Mesenterial fibromatosis, also known as aggressive fibromatosis or desmoid tumor is generally associated with Gardner's syndrome but otherwise an extremely rare disease. Fibromatoses do not metastasize but are characterized by a high incidence of local recurrence. Primary treatment is operative. After multiple recurrence irradiation, cytostatics and antiestrogens have been used additionally to control the disease.
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PMID:[Mesenteric fibromatosis in childhood]. 321 Nov 72

Fibrous dysplasia (20%), metastases (16%) and chondrosarcoma (11%) are the most common bone tumors of the chest wall. Except lipoma, primary lesions of the soft tissue of the chest occur rarely as well. The Askin- and Abrikosoff tumor, elastofibroma dorsi and desmoid can be seen as typical exceptions. With respect to the histogenetic classification of undifferentiated sarcomas and thoracic metastases (f.e. prostatic carcinomas or mesotheliomas) an intimate cooperation of surgeons and pathologists as well as modern immunohistochemical investigations are required.
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PMID:[Primary and secondary chest wall tumors from the pathologist's viewpoint]. 332 39

A patient with a huge pelvic fibromatosis, a form of intra-abdominal desmoid, encroaching on the urinary bladder is described. Because of its rarity, there is frequently a lack of diagnostic awareness of this tumor, particularly in the gynecological or urological patient. Although this tumor is a locally invasive benign tumor which dose not metastasize, management by radical tumor excision without the sacrifice of the major pelvic nerves and vessels is mandatory, since this tumor has generally a high postsurgical recurrence rate.
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PMID:Huge pelvic fibromatosis encroaching on the urinary bladder. A case report. 338 37

Aggressive fibromatoses which may develop either in soft tissue or in the bone present considerable problems for the pathologist trying to establish a diagnosis as well as for the radiologist and surgeon. In radiographs, a destruction of the soft and osseous tissue is seen which suggests a malignant tumor. Histologically a monomorphic connective tissue prevails in the biopsy showing no essential signs of malignancy. Under pathoanatomical aspects often a benign proliferation of the connective tissue is assumed. Surgically the tumor may either be removed in a too radical and mutilating way, or the excision may remain incomplete. Two cases of desmoplastic bone fibroma (aggressive fibromatosis in the ulna and in the sacrum) are described in which the complete tumor removal led to healing, whereas the incomplete excision of the tumor resulted in recurrences. Aggressive fibromatosis represents a semimalignant tumor which has a locally destructive and invasive growth tendency but does not metastasize. The various fibromatoses are defined with regard to their biological growth tendency and the therapeutic consequences are discussed.
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PMID:[Aggressive fibromatoses in orthopedics]. 375 Dec 51


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