Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Characteristics of a little-studied tumor of soft tissues are described on the basis of the author's own material (4 observations) and data from the literature. The matter at issue is a benign or malignant schwannoma (less frequently, ganglioneuroblastoma) in which there are areas of rhabdomyosarcoma and sometimes tumor elements of mesenchymal origin (angio- lipo-, or osteogenic sarcoma). As a rule, this tumor is located along the peripheral nerve or arises in one of the nodes in Recklinghausen's disease and has a trend to hematogenic metastasising. It is suggested that the source of growth of neoplasias of this kind are the cells of "neural crest" migrating in the process of embryogenesis. These cells are responsible for the formation of lemmocytes, ganglial elements and melanocytes, as well as ectomesenchyma from which, in its turn, a part of cross-striated musculature is formed. Therefore, the tumors described in the paper should be designated as "neuroectomesenchymoma" (according to some authors, ectomesenchymoma).
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PMID:[Peripheral nerve ectomesenchymoma (neuroectomesenchymoma): a malignant tumor made up of neural crest elements]. 731 24

Recent cloning of the t(11;22) region has led to the detection of a number of sequences involved in the breakpoints by substituting a sequence which encodes a putative RNA binding domain for that of the DNA binding domain of the human homologue of murine FLI-1. Several tumours display consistent translocation at t(11;22) (q24;q12), a finding that suggests these fusion transcripts could be expressed and detected by reverse transcriptase polymerase chain reaction amplification. To date, only a small number of Ewing's sarcomas (Es) and peripheral neuroectodermal tumours (pPNET) of bone have been tested with this novel molecular biology approach. In this study, we confirmed the presence of the three putative chimaeric transcripts on 7 cases of Es and pPNET sarcomas of bone and soft tissue, providing 100% positivity for the tested tumours. For comparative purposes, a number of other neuroectodermal tumours were analysed with negative results: esthesioneuroblastoma, retinoblastoma, Schwannoma. A primitive soft tissue sarcoma (ectomesenchymoma) with a 22 chromosome rearrangement did not express any transcript, nor did a number of non-neuroectodermal small round cell sarcomas of soft tissue (rhabdomyosarcomas) and bone (microcellular osteosarcoma), conventional bone sarcomas, leiomyosarcomas, malignant fibrous histiocytomas and synovial sarcomas. These results reinforce the value of molecular biology techniques for the correct assessment of histology difficult evaluable neoplasms, such as the group of small round cell tumours within the Es family.
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PMID:EWS/FLI-1 rearrangement in small round cell sarcomas of bone and soft tissue detected by reverse transcriptase polymerase chain reaction amplification. 752 96

The lateral extracavitary approach was used for single-staged tumor resection in 12 patients with complex dumbbell or paraspinal tumors of the thoracic and lumbar spine. Six women and six men (age, 28-72 yr) were treated between August 1990 and January 1994. The tumors included schwannoma (6 patients), malignant meningioma (1 patient), hemangioma (1 patient), chondrosarcoma (1 patient), osteocartilaginous exostosis (1 patient), radiation-induced osteogenic sarcoma (1 patient), and metastatic renal carcinoma (1 patient). Gross total resection was achieved in 11 patients. Radical subtotal removal was performed in the remaining patient, who had a malignant osteogenic sarcoma. Concomitant spinal stabilization with internal fixation and anterior interbody strut grafting was performed on two patients. No significant perioperative complications occurred. Ten patients were alive and clinically stable at follow-up visits ranging from 14 to 55 months. Two patients died from systemic tumor dissemination during the follow-up period. The lateral extracavitary approach is useful when extensive or difficult spinal and paraspinal exposure is required. The surgical aspects of these neoplasms and the technique of lateral extracavitary approach are described in detail.
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PMID:Surgical management of dumbbell and paraspinal tumors of the thoracic and lumbar spine. 874 53

Between 1991 and 1994, 582 operations were performed in our service; 19 (3.26%) were on primitive tumors of the chest wall. We analyze the data for these patients, including age, sex, clinical findings, chest images, diagnoses, therapy and course. Ten tumors were benign and 9 were malignant. The most frequent clinical findings were pain and/or tumor. Diagnosis was achieved before surgery in only 2 cases. Except when there are clear macroscopic and X-ray signs that the tumor is benign, we performed broad exeresis of the chest wall, sometimes also resecting adjacent structures. The defect was repaired directly in 12 cases. The defects were covered by prostheses and/or muscle plasty in the remaining patients. The most frequent tumor was chondrosarcoma (3 cases), followed by 2 cases of osteoblastoma and osteochondroma. Only 1 each of the following tumors were found: plasmocytoma, chondroma, fibrous dysplasia, eosinophilic granuloma, osteosarcoma, Ewing's tumor, epithelioid sarcoma, fibrosarcoma, hemangioma, benign neurilemmoma, desmoid tumor and liposarcoma. Two patients with chondrosarcoma were operated on for recurrences and there was also recurrence in the patient with Ewing's tumor. We conclude that: 1) chest wall tumors are infrequent, 2) radical exeresis is the treatment of choice and prosthesis is often necessary, and 3) chondrosarcoma, with poor outcome in our patients, is the most frequent tumor.
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PMID:[Primary tumors of the thoracic wall (1991-1994)]. 1256 9

Trk receptors have been identified by immunohistochemical methods in primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (ES). However, the presence of different members of the Trk family of receptors in PNET/ES has not been specified. We have examined whether Trk A, B, and C receptors are specifically expressed in ES both with and without features of neural differentiation. Ten ES tumors (five primary tumors of bone and five extraosseous tumors transplanted into nude mice) were investigated for expression of Trk receptors by immunohistochemistry and reverse transcription-polymerase chain reaction. One primary ES and the five grafted ES tumors exhibited signs of neural differentiation; the remaining four primaries were undifferentiated ES. Other tumor types were analyzed as controls; they included three neuroblastomas (NB), two lymphomas, and single cases of pheochromocytoma (PHEO), schwannoma (SCHW), osteosarcoma, and carcinoma of breast, colon, and kidney. Trk receptors were detected in paraffin-embedded tumor tissue sections by means of a pan-Trk polyclonal antibody raised against the 14 carboxy-terminal residues of gp140trk, and trk A, B, and C transcripts were specifically detected by polymerase chain reaction-based amplification on cDNAs derived from tumor RNA with MuLV reverse transcriptase. Reactivity to the pan-Trk antibody was exhibited by six ES tumors, the three NBs, and the single PHEO and SCHW cases; immunoreactivity was restricted to differentiated tumors, in the case of ES. Tumor types positive for immunostaining were also distinguished by containing transcripts of TRK genes. However, the trk A, B, and C expression pattern of ES differed from that of NBs, PHEO, and SCHW. Transcripts of trk A, B, and C were detected in seven, four, and one case of ES, respectively, and in five, two, and five cases of NB, PHEO, and SCHW, respectively. Interestingly, all differentiated ES tumors contained trk A transcripts. Tumors of neuroectodermal phenotype and/or derivation were thus characterized by a distinct consensus expression pattern: trk A+/B-/C+ for differentiated ES and trk A+/B-/C+ for NB-PHEO-SCHW. These results indicate that the TRK gene family is frequently activated in ES; they also suggest that Trk A receptor is a feature of ES with neural differentiation, whereas Trk B and C receptors seem to be present in undifferentiated ES.
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PMID:Activation of TRK genes in Ewing's sarcoma. Trk A receptor expression linked to neural differentiation. 902 32

Chromosome region 9p21 contains a tumor suppressor locus (p16) that may be involved in the genesis of several kinds of malignant tumors. To characterize the role of this gene in the development of soft-tissue tumors (STTs), we investigated the frequency of loss of heterozygosity (LOH) at this locus. DNA was obtained from 77 tumors and the peripheral blood of 23 of the patients with the tumors. Using one microsatellite marker distal to p16(D9S171) and one intragenic sequence-tagged site (STS) marker (c5.1), we observed LOH in only one liposarcoma and one malignant schwannoma (2.6%). Homozygous deletions of the p16 markers were not found. The osteosarcoma cell line MG-63 was used as a control for loss of the p16 gene. Because of the low LOH frequency, we hypothesize that the p16 gene is not essential for STT oncogenesis.
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PMID:Loss of heterozygosity on chromosome 9q21 (p16 gene) uncommon in soft-tissue sarcomas. 904 81

Fibrous orbital tumors present clinically and radiographically in a broad spectrum ranging from a benign mass, to locally aggressive tumor, to invasive malignancy. Pathologic analysis and diagnosis are often challenging, usually based on a combination of light microscopy, immunohistochemistry, and electron microscopic findings. Some light microscopic and immunohistochemical findings, however, are relatively characteristic. A storiform or cartwheel pattern and vimentin staining are characteristic of fibrous histiocytoma. A herringbone pattern is usually found in fibrosarcoma. A "patternless pattern" and CD34 staining is found most commonly in solitary fibrous tumor. CT and MR imaging findings, as well as clinical presentation, in fibrous orbital lesions are often difficult to distinguish from those of other orbital masses, although there may be useful clues. Benign fibrous lesions are usually well-circumscribed and may chronically remodel bone, whereas more aggressive malignant fibrous tumors tend to have infiltrating margins and may destroy bone on CT or MR imaging. With malignant fibrous masses, enhancement patterns on CT or MR imaging may be more inhomogeneous, with avascular or necrotic nonenhancing regions. At MR imaging, benign lesions tend to be homogeneous on T1, T2, and postgadolinium T1-weighted images, whereas malignant soft tissue lesions may change their pattern from homogeneous on T1-weighted images to heterogeneous with low signal septations on T2-weighted images. Low T2 signal comprising part or all of a fibrous lesion correlates with dense collagen fibers, with a less cellular matrix. Areas of hyperintensity on T2-weighted images correspond with a more cellular matrix of fibroblasts and other cells. Calcification within a tumor, however, may give a similar appearance. Thus, if a lesion has predominantly low signal on T2-weighted images, or less specifically has low signal septations, then a fibrous orbital lesion with high collagen content may be ranked higher in the differential diagnosis (see Figs. 2E and 3B). When T2 signal is intermediate-to-high, then one has a difficult time narrowing the differential diagnosis. Radiographically, distinguishing these lesions from other fibrous orbital lesions, as well as from other varieties of orbital masses, is difficult. Differential diagnosis of fibrous orbital masses includes all the fibrous lesions described in this article, in addition to schwannoma (Fig. 7), neurofibroma (Figs. 4 and 8), hemangiopericytoma (Figs. 9 and 10), rhabdomyosarcoma, meningioma, lymphoma, and metastasis (Figs. 11 and 12). A history of prior orbital irradiation for retinoblastoma or other tumors may raise the possibility of radiation-induced secondary tumors, such as MFH, fibrosarcoma, and osteosarcoma. Determining the extent of orbital involvement remains the primary goal of the radiologist. The final diagnosis still rests with the pathologist.
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PMID:Fibrous histiocytoma and fibrous tissue tumors of the orbit. 1002 37

Between the years 19890-1998 in I ENT Department of Silesian Medical Academy were treated surgically 15 patients suffering from soft tissue sarcomas of head and neck region. Patomorphological examination showed: fibrosarcoma (4 cases), haemangioendothelioma malignum (2 cases), rhabdomyosarcoma (2 cases), schwannoma malignum (2 cases), fibrohistiocytoma malignum (2 cases), tumor mixtus mesenchymalis (1 case), haemangiopericytoma malignum (1 case), extrasceletal osteosarcoma (1 case). Sarcomas were localised in paranasal sinuses (7 cases), orbita (5 cases), parapharyngeal space (1 case), parotid gland (1 case) and bucca (1 case). All those patients were treated surgically and most of them were irradiated postoperatively. The authors have presented the diagnostic and methods of treatment of soft tissue sarcomas. According to our experience and data of the literature the method of choice in treatment soft tissue sarcomas should consist of surgery and radiation therapy postoperatively.
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PMID:[Histological and clinical analysis of mesenchymal malignant neoplasms (soft tissue sarcomas) of head and neck region in the data of I ENT Department of Silesian Medical Academy]. 1091 56

Primary malignant tumours should be resected with wide margins. This may be difficult to apply to lesions of the spine. We undertook total vertebrectomy on seven patients, four males and three females with a mean age at operation of 26.5 years (6.3 to 45.8). The mean follow-up was 52.3 months. Histological examination revealed a Ewing's sarcoma in two patients and osteosarcoma, leiomyosarcoma, spindle-cell sarcoma, chondrosarcoma and malignant schwannoma in one each. In five patients, histological examination showed that a wide resection had been achieved. At follow-up there was no infection and a permanent neurological deficit was only seen in those patients in whom the surgical procedure had required resection of nerve roots. Despite the high demands placed on the surgeon and anaesthetist and the length of postoperative care we consider total vertebrectomy to be an appropriate procedure for the operative treatment of primary malignant lesions of the spine.
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PMID:Total vertebrectomy for primary malignant tumours of the spine. 1218 90

Synovial sarcomas are rare soft tissue malignancies that most commonly affect the extremities in the vicinity of large joints. These malignancies typically occur in adolescents and young adults between the ages of 15 and 40 years.(1,2) Historically they are believed to originate from primitive pluripotent mesenchyme capable of synovial differentiation. This belief is consistent with the malignancy's origin from sites devoid of normal synovium, such as the pleural cavity. A variety of pleural cavity sarcomas have been described, including liposarcoma,(3) chondrosarcoma,(4) osteosarcoma,(5) and malignant schwannoma.(6) Pleural synovial sarcoma, however, is a much rarer entity. In fact, pleural synovial sarcoma was first described only 6 years ago(7) and has not yet been reported in the surgical literature. Because of its rarity, pleural synovial sarcoma is often mistaken for the histologically similar malignant mesothelioma, the most common of the pleural neoplasms. This is a critical distinction, because synovial sarcoma may be extremely aggressive. Studies in the last 10 years have shown it to be extremely sensitive to ifosfamide-based chemotherapy, and survival of patients with synovial sarcoma has recently increased with chemotherapy, with 5-year survivals now as high as 57%.(8-10) In this report, we describe 3 cases of synovial sarcoma of the pleura. Clinical findings are correlated with pathologic features, including immunohistochemical stains and fluorescence in situ hybridization (FISH) for the identification of the diagnostic chromosomal translocation, t(X;18)(p11.2;q11.2). This delineation of the clinical and pathologic aspects of this rare, newly recognized tumor should increase awareness among the surgical community.
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PMID:Synovial sarcoma of the pleura: a clinical and pathologic study of three cases. 1232 43


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