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)

A report of 2 cases of solitary fibromatosis in a 10-day-old boy and a girl 3 years and 10 months old is presented. Both lesions were deep-seated and showed a nodular and infiltrating growth, predominantly buil-up by immature fibroblast-like cells and including hemangiopericytoma-like areas. One of the lesions also showed leiomyoma-like areas. An ultrastructural study however, revealed no intra-cytoplasmatic myofilaments. At follow-up examinations after 21 years and 1 year, respectively, there were no signs of recurrences or metastases. These 2 cases are considered to represent a solitary form of congenital generalized fibromatosis. The differential diagnosis from infantile hemangiopericytoma and fibrous lesions seen in infancy and early childhood, such as infantile fibrosarcoma, diffuse infantile fibromatosis, extra-abdominal desmoid, fibrous hamartoma of infancy and juvenile aponeurotic fibroma, is discussed.
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PMID:Congenital solitary fibromatosis of soft tissues, a variant of congenital generalized fibromatosis. 2 cases reports. 92 Jan 81

In a study of 256 nonepithelial neoplasms involving the nasal cavity, paranasal sinuses, and nasopharynx, 23 lesions were classified as fibrous tissue tumors, including four cases of "fibroma", six of fibromatosis, and thirteen of fibrosarcoma. The clinical findings associated with these lesions are described, their histologic features illustrated, results of therapy presented and clinicopathologic correlations made. The "fibromas" presented a small localized nodules. None recurred after local excision. Fibromatosis, a locally aggressive tumor, does not metastasize, but may cause considerable morbidity or even death due to local infiltration which may be difficult to control surgically. Fibrosarcoma may cause death either by local infiltration or by metastasis, but has a better prognosis than most other sarcomas of this region. We recommend that a large en block resection be performed initially for fibromatosis and fibrosarcoma growing in this area, after the diagnosis has been made by biopsy. In this series, including patients who had more than one operation, recurrent tumor was seen following 10 of 12 limited local excisions performed for fibromatosis and fibrosarcoma, but in only one of 13 patients after a large bloc resection. The problems involved in histologically differentiating fibrous tissue tumors from other lesions are discussed. A patient with the rare syndrome of multicentric fibromatosis with spontaneous regression of lesions is presented.
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PMID:Nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx. A clinicopathologic study. VI. Fibrous tissue tumors (fibroma, fibromatosis, fibrosarcoma). 94 12

A 68-year-old man sought dermatologic attention for a tumor of the arm. Biopsy specimen showed abnormal, essentially amelanotic, spindle-shaped cells in the cutis, greatly fibrotic stroma, and focal epidermal invasion. Desmoplastic malignant melanoma was diagnosed. The lesion was widely excised and axillary lymphadenectomy performed; one node showed metastasis. Nine months later, he died with widespread metastatic disease. To our knowledge, this is the first report of this entity since its delineation in 1971 and the only case in which diagnosis was established on initial biopsy and followed by definitive therapy. Desmoplastic melanoma has been confused with benign fibrosis, invasive fibromatosis, and fibrosarcoma, and is another example, with morpheaform basal cell carcinoma and sclerodermoid metastatic lesions from breast carcinoma, in which desmoplastic stroma may obscure the epithelial nature of cutaneous neoplasm.
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PMID:Desmoplastic malignant melanoma. 113 19

Fibromatosis is the proliferation of connective tissue with local infiltrative growth and a tendency to relapse in contrast with fibrosarcoma which never produces metastases. Two patients, a mother and son diagnosed with fibromatosis of initial cervical location in whom surgical extirpation and complementary radiotherapy were performed. The mother underwent polychemotherapy (adriamicine plus dacarbazine) because of extensive relapse. Definitive resolution was achieved in both cases.
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PMID:[Cervicobrachial fibromatosis presenting in the mother and the child simultaneously]. 146 Aug 99

Aggressive juvenile fibromatosis is a destructive, locally invasive process, with a propensity for local recurrence after inadequate excision. Although it does not metastasize, uncontrolled growth can eventually lead to death. The role for radiation and chemotherapy is controversial and these modalities are best reserved for advanced unresectable or recurrent lesions. The treatment of choice is en bloc surgical resection with wide margins.
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PMID:Aggressive juvenile fibromatosis involving the paranasal sinuses. 190 71

We report a new case of aggressive juvenile fibromatosis (A.J.F.) in a 20-month-old girl. The lesion affected the inferior border of the left mandible and the adjacent soft tissues. The child presented with a painless mass, which had grown over a period of 2 months. Radiographs and computed tomographic scan showed a multilacunar bone defect with subcutaneous and gingival involvement. A biopsy was performed, followed by a partial hemimandibulectomy and a costal graft. The surgical specimen measured 4 X 3 X 3 cm. The patient did well 1 year after surgery. 16 cases of A.J.F. have been reported; with ours, 14 are mandibular. A.J.F. is a locally aggressive lesion, which doesn't metastasize. It occurs chiefly in childhood and adolescence from 1 1/2 to 18 years (median 6.5 year-old). Duration of symptoms prior to presentation varies from a few weeks to months. Clinically, it is a firm nodule. Radiographs are non-specific, but 9 cases have poorly defined destruction of the mandibular inferior border. Following a block resection of the tumor (13 cases), there is no recurrence. In this review, we discuss the clinico-pathologic diagnosis of this impressive tumor which is compared with other mandibular fibrous tumors in children.
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PMID:[A case of aggressive juvenile fibromatosis of the mandible. Review of the literature]. 266 Aug 11

Aggressive infantile fibromatosis, one of the juvenile fibromatoses, is generally considered a benign lesion, but it does tend to recur locally because of its invasive nature. Distant metastases are considered rare. This case report documents pulmonary metastases arising 2 years after resection of the primary lesion from the thigh of an infant. This is the seventh reported case of distant metastasis from aggressive infantile fibromatosis. Computed tomography of the original lesion as well as the metastases are presented.
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PMID:Aggressive infantile fibromatosis. Pulmonary metastases documented by plain film and computed tomography. 276 75

We report 65 cases of a hitherto undescribed neoplasm that occurs chiefly in children and young adults, and has morphologic features reminiscent of both a fibrous histiocytoma and fibromatosis. The median age of the 65 patients was 14.5 years; two-thirds (67.7%) of the patients were younger than 20 years. The lesion was more common in female patients (46 cases) than in male patients (19 cases). It usually presented as a slow-growing, poorly demarcated dermal or subcutaneous mass that rarely exceeded 3 cm in greatest diameter. Its most common location was the upper extremity (63.1%), especially the regions of shoulder and forearm. Under the microscope, the lesions were characterized by a multinodular or plexiform proliferation of histiocyte- and fibroblast-like cells associated with multinuclear giant cells. Differential diagnosis chiefly includes cutaneous fibrous histiocytoma, plexiform neurofibroma, fibromatosis, and benign and malignant giant cell tumor. Twenty of the 32 cases (62.5%) with follow-up information were alive and well after local excision, but the tumor recurred in 12 cases (37.5%). In two patients with recurrence, the disease metastasized to regional lymph nodes 9 and 36 months after the initial excision, respectively. Metastasis to the lung or other organs was not observed. We were unable to demonstrate a close correlation between biologic behavior and any specific clinical or morphologic parameter.
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PMID:Plexiform fibrohistiocytic tumor presenting in children and young adults. An analysis of 65 cases. 284 69

We describe seven cases of invasive fibrous tumors of the trachea and major bronchi with distinctive histologies and patterns of growth. The tumors are composed of proliferating fibroblasts and have moderate nuclear pleomorphism and low mitotic activity. The tumors bear some resemblance to inflammatory pseudotumor of the lung, fibrous histiocytoma, and fibromatosis, but differ from each of these entities. The tumors are neoplastic and invade down to or between plates of cartilage. Because of their proximal location, these tumors are usually amenable to sleeve resection. Recurrence is possible. Metastasis has not occurred. Distinction from more malignant mesenchymal tumors of the trachea and bronchus will prevent unnecessarily radical surgery.
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PMID:Invasive fibrous tumor of the tracheobronchial tree: clinical and pathologic study of seven cases. 291 2

Two molecular recombinants (EU-8 and K-3) constructed from ring-necked pheasant virus and UR2AV, the helper virus associated with avian sarcoma virus UR2, caused a high incidence of a hitherto unreported pathological condition in chick skeletal muscle. A disease spectrum was observed in which muscle was infiltrated by proliferating fibroblasts and caused white streaks, white diffuse areas, or well-defined elongated tumors. Fibroblast proliferation was progressive, and the gross presentation depended on the rapidity and extent of proliferation. There was evidence of anaplasia but not frankly malignant disease or metastases. The disease was produced at a high frequency when 10-day-old embryos were infected. Breast muscle (pectoralis major and minor) was affected with variable severity in all birds, thigh muscles were affected occasionally, while other thoracic, external abdominal, and wing muscles were affected very rarely. Progression of proliferative muscle lesions was demonstrated by sequential necropsies and microscopic examination of muscle samples. The disease appeared before 2 weeks of age, and thin white streaks with minimal cellular proliferation were the earliest lesions observed. Elongated, spindle-shaped tumors were the most advanced form of proliferation observed. The advanced lesions showed cellular anaplasia and signs of rapid growth, and appeared most commonly at 6 weeks of age or later. Histologically, mild proliferation correlated with normal appearing fibroblasts producing collagen. Severe proliferation correlated with anaplastic, rapidly dividing cells producing little collagen and a high mitotic index. A decrease in the virus dose resulted in less severe fibromatosis, but at least one chicken infected with a low virus dose showed severe disease. When 10- or 35-day-old hatched chicks were injected in the breast muscle with EU-8 or K-3, a low incidence of lymphoid leukosis was observed, but no fibromatosis resulted during an observation period of 27 weeks. Infectious virus was extracted from the fibromatosis in the breast muscle, and extracted virus caused the same disease when injected in 10-day-old embryos. No transforming activity was observed on normal chick embryo fibroblast monolayers. Cell cultures were established from areas in the breast muscle affected by fibromatosis, and from discrete tumors. These cells did not show any signs of transformation in culture. Supernatants obtained from these cell cultures caused fibromatosis when injected into 10-day-old embryos, but did not transform normal chick embryo fibroblasts.
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PMID:Proliferative fibromatosis in avian skeletal muscle caused by cloned recombinant avian leukosis viruses. 303 May 43


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