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)

Inflammatory myofibroblastic tumor (IMT) or inflammatory pseudotumor is a spindle cell proliferation of disputed nosology, with a distinctive fibroinflammatory and even pseudosarcomatous appearance. Although the lung is the best known and most common site, inflammatory myofibroblastic tumor occurs in diverse extrapulmonary locations. We report our experience with 84 cases occurring in the soft tissues and viscera of 48 female patients and 36 male patients between the ages of 3 months and 46 years (mean, 9.7 years; median, 9 years). A mass, fever, weight loss, pain, and site-specific symptoms were the presenting complaints. Laboratory abnormalities included anemia, thrombocytosis, polyclonal hypergammaglobulinemia, and elevated erythrocyte sedimentation rate. Sites of involvement included abdomen, retroperitoneum, or pelvis (61 cases); head and neck, including upper respiratory tract (12 cases); trunk (8 cases); and extremities (3 cases). The lesions ranged in size from 1 to 17 cm (mean, 6.4; median, 6.0). Excision was performed in 69 cases. Eight had biopsy only. Five patients received chemotherapy or radiation in addition to undergoing biopsy or resection as initial treatment. Sixteen patients had multinodular masses involving one region. Clinical follow-up in 53 cases revealed that 44 patients were alive with no evidence of disease, four were alive with IMT, and five were dead. Thirteen patients had one or more recurrences at intervals of 1-24 months (mean, 6 months; median, 10 months). No distant metastases were documented. The five patients who died had complications either due to the location of the lesion (heart, peritoneum, retroperitoneum, or mesentery) or related to treatment (lymphoproliferative disorder following hepatic transplantation; sepsis following wound infection). The abdominal masses were the largest. All tumors were firm and white with infiltrative borders and focal myxoid change. Three basic histologic patterns were recognized: (a) myxoid, vascular, and inflammatory areas resembling nodular fasciitis; (b) compact spindle cells with intermingled inflammatory cells (lymphocytes, plasma cells, and eosinophils) resembling fibrous histiocytoma; and (c) dense plate-like collagen resembling a desmoid or scar. Immunohistochemistry demonstrated positivity for vimentin, muscle-specific actin, smooth muscle actin, and cytokeratin consistent with myofibroblasts. Based on this series, inflammatory myofibroblastic tumor is a benign, nonmetastasizing proliferation of myofibroblasts with a potential for recurrence and persistent local growth, similar in some respects to the fibromatoses.
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PMID:Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. 866 41

Inflammatory pseudotumor, as a histologic diagnosis and carrying with it the prospect of a benign clinical course, is rather firmly entrenched as a pulmonary lesion. Extrapulmonic forms, however, are farraginous and, as reported, have included lesions having few, or even none, of the histologic features of those in the lungs. For those more closely aligned with the fibroblastic or myofibroblastic phase of the lung lesions, their biologic behavior can belie their histology in that they can be locally aggressive and metastasize as sarcomas. For those tumors in the upper airway, and especially in the abdomen, pathologists should be very circumspect in the use of inflammatory pseudotumor as a diagnosis, and clinicians should not be lulled into denying the extrapulmonic forms their due respect.
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PMID:"Inflammatory pseudotumor": what is it? How does it behave? 771 28

Both thallium-201 and iodine-123 alpha-methyltyrosine (123I-IMT) have been shown to be useful in the diagnostic evaluation of brain tumours. The aim of this study was to investigate the respective contributions of 201Tl and 123I-IMT single-photon emission tomography (SPET) in the non-invasive evaluation of intracerebral tumours. We analysed 65 patients with the following brain tumours: 8 non-neoplastic lesions, 4 meningiomas, 12 low-grade gliomas, 28 high-grade gliomas, 11 metastases and 2 high-grade lymphomas. 201Tl SPET and 123I-IMT SPET were performed [start of 201Tl SPET: 15 min p.i. (early) and 180 min p.i. (delayed); start of 123I-IMT SPET: 15 min p.i.]. The intensity of uptake was quantified as the ratio between tracer accumulation in the tumour and in the contralateral hemisphere. None of the non-neoplastic lesions or low-grade gliomas expressed marked 201Tl uptake. All malignant tumours except one small metastasis and all meningiomas except one small, cystic and degenerated lesion showed significant 201Tl accumulation [Tl(15')>2.0]; 123I-IMT uptake was either absent or intermediate in non-malignant lesions except in two low-grade gliomas; the highest levels were observed in high-grade gliomas followed by metastases and lymphomas (mean IMT: 2.7 vs. 2.1 vs. 1.8), with metastases showing a high variability in 123I-IMT uptake (range: 0.8-3.6). Using 201Tl to distinguish non-neoplastic lesions from malignant tumours and meningiomas, 63 of 65 patients were characterised correctly. In the latter group, high-grade gliomas were correctly identified in 27 of 28 cases by their amino acid uptake. It is concluded that the combination of 201Tl and 123I-IMT surpasses the accuracy of each single test in the differentiation of space-occupying lesions of the brain. Based on these preliminary results, a sequential strategy is proposed involving an initial 201Tl SPET study and an additional 123I-IMT SPET study in the event of positive 201Tl uptake.
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PMID:Sequential scintigraphic strategy for the differentiation of brain tumours. 1085 11

Inflammatory pseudotumor is a rare lesion that generally is considered to be benign in biological behavior, although some may recur or metastasize. The authors report on a patient with inflammatory pseudotumor of the liver whose preoperative radiologic findings resembled those of focal nodular hyperplasia. The biological investigation showed a polyclonality of the cells and diploidy of the DNA content and suggested benign characteristics of the lesion. J Pediatr Surg 36:663-666.
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PMID:Inflammatory pseudotumor of the liver: case report and review of the literature. 1168 18

Inflammatory myofibroblastic tumor is a rare entity composed of spindle cells admixed with variable amounts of extracellular collagen, lymphocytes, and plasma cells. In the genitourinary tract, inflammatory myofibroblastic tumor most commonly occurs in the bladder. Isolated case studies of inflammatory myofibroblastic tumor of the kidney, renal pelvis, and ureter have been previously reported. Our series includes 12 cases of inflammatory myofibroblastic tumor occurring in the renal pelvis (six cases), renal parenchyma (four cases), and immediate perirenal soft tissue (two cases). Clinical presentation included flank pain (two patients), painless gross hematuria (one patient), and ureteropelvic junction stenosis with hydronephrosis (one patient). The remaining eight patients were asymptomatic. All patients underwent nephrectomy. The tumors were characterized by firm white tissue or had a myxoid "gelatinous" appearance. Three histologic patterns were identified in the tumors, including a myxoid vascular pattern, a compact spindle cell pattern, and a hypocellular fibrous pattern. Immunohistochemical and electron microscopic studies supported a myofibroblastic proliferation. All cases were negative for anaplastic lymphoma kinase. Follow-up was available in eight cases and ranged from 1 to 17 years with no evidence of recurrence. Based on this series, renal inflammatory myofibroblastic tumor is a proliferative lesion of myofibroblasts of uncertain pathogenesis with no identified potential for recurrence or metastases.
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PMID:Inflammatory myofibroblastic tumors of the kidney: a clinicopathologic and immunohistochemical study of 12 cases. 1271 50

Inflammatory myofibroblastic tumor, also referred to as inflammatory fibrosarcoma, is a rare tumor composed of myofibroblastic spindle cells of uncertain etiology and disputed nosology. We report a case of inflammatory myofibroblastic tumor of the omentum with involvement of the bone marrow in an 18-year-old man. Histologic and immunohistochemical studies of the abdominal mass and bone marrow were consistent with inflammatory myofibroblastic tumor. Additionally, fluorescence in situ hybridization using a probe specific for the ALK gene showed disruption of the gene. The literature is reviewed with emphasis on the ability of inflammatory myofibroblastic tumor to recur, metastasize, and cause mortality.
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PMID:Inflammatory myofibroblastic tumor with bone marrow involvement. A case report and review of the literature. 1282 44

Inflammatory pseudotumor of the liver, which is rarely seen, can mimic abscesses, metastases, peripheral cholangiocarcinomas and hepatocellular carcinomas. While it has no specific clinical and laboratory findings, detection of a "target-like appearance" in triphasic CT and MRI examination may be differentiated from other liver pathologies. In this report, triphasic CT and MRI findings of an inflammatory pseudotumor of the liver were presented.
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PMID:[Case report: Inflammatory pseudotumor of the liver with target-like appearance]. 1466 Dec 97

The case of an old male is presented who had postoperative spindle cell nodule of urinary bladder. This benign condition mimics a sarcoma. However, the treatment modalities and the outcome are entirely different. Inflammatory pseudotumor/PSCN and leiomyosarcoma of the bladder overlap in clinical and immunohistochemical findings but are histologically as well as clinically distinct, correlating with different natural histories. Inflammatory Pseudotumor/PSCN are benign, not prone to metastasize, and is outside the spectrum of low-grade inflammatory sarcoma of urinary bladder.
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PMID:Pseudotumor of urinary bladder. 1476 66

Malignant tumors composed of myofibroblasts are increasingly being recognized, but their existence remains controversial. Currently accepted examples within this category represent spindle cell or pleomorphic neoplasms of the soft tissues with a spectrum of histological grades. Low- and intermediate-grade myofibrosarcomas are fascicular spindle cell neoplasms resembling fibrosarcoma or leiomyosarcoma. They infiltrate deep soft tissue with disproportionate involvement of head and neck sites and can recur locally but infrequently metastasize. They variably express myoid immunohistochemical markers, and their differential diagnosis includes benign myofibroblastic proliferations such as fasciitis and fibromatosis as well as other types of spindle cell sarcoma. High-grade (pleomorphic) myofibrosarcomas are an ultrastructurally defined subset of malignant fibrous histiocytoma, which they resemble in morphology and behavior. Inflammatory myofibroblastic tumor and infantile fibrosarcoma are neoplasms that have myofibroblastic features and have been included in this category, but they have distinctive genetic findings. This article reviews the concept of myofibrosarcoma and describes its variants.
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PMID:Myofibroblastic malignancies. 1522 Aug 22

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


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