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

The pathologic material from 56 patients diagnosed initially as Ewing's sarcoma of the distal extremity and treated on National Cancer Institute protocols between 1968 and 1984 was reviewed and correlated with clinical outcome. Histologically, the tumors were categorized, based on recent pathologic criteria, into three diagnostic groups: (1) typical Ewing's sarcoma, (2) atypical Ewing's sarcoma, and (3) other (predominantly peripheral neuroepithelioma [PN]). Thirty-two patients (57%) had typical Ewing's, 13 (23%) were atypical, and 11 (20%) were in the "other" diagnostic category (seven [13%] PN, two primitive rhabdomyosarcoma, one primitive sarcoma of bone, and one synovial cell sarcoma). No cases of metastatic neuroblastoma, osteosarcoma, or lymphoma were found. Forty-five patients had localized disease at diagnosis; 11 had metastases. Patients with typical Ewing's sarcoma were less likely to have metastatic disease at the time of diagnosis. Only two of 32 patients with typical Ewing's sarcoma had metastases compared with nine of 24 patients in the two other groups. The pattern of relapse was also different in these other groups compared with typical Ewing's patients; five patients developed lymph node metastases and two patients developed brain metastases. Although the presence of metastatic disease at diagnosis was a strong negative prognostic factor, our histologic grouping was independently prognostic of clinical outcome in patients with localized disease. Patients with typical osseous Ewing's sarcoma had an improved overall survival (P2 = 0.03) and patients with other tumors (neither typical nor atypical Ewing's sarcoma) had a poorer disease-free survival (P2 = 0.02). Since no generally accepted histopathologic prognostic criteria exist for Ewing's sarcoma, the potential value of our proposed classification should be evaluated in a larger retrospective and a prospective study.
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PMID:Prognostic value of histopathology in Ewing's sarcoma. Long-term follow-up of distal extremity primary tumors. 198 13

In pediatric malignancies, particularly sarcomas, alkylating agents play an important role in the curative, combination chemotherapy approach. Since high doses of ifosfamide, the structural isomer of cyclophosphamide, were made tolerable with mesna uroprotection, high-dose ifosfamide (5 to 10 g/m2) has replaced conventional-dose cyclophosphamide (900 to 1,500 mg/m2) in some combination chemotherapy regimens. In Ewing's sarcoma and soft tissue sarcoma, the response rate and proportion of patients surviving disease free have been increased (at least for poor-prognosis patients) by 15% to 20% with an ifosfamide-containing regimen, as used in ongoing trials of the German Society of Pediatric Oncology. In germ cell tumors, the combination of ifosfamide and etoposide has proved to be an effective salvage regimen in patients resistant to vinblastine, bleomycin, and cisplatin. In stage IV disseminated neuroblastoma, however, the introduction of ifosfamide-containing regimens has not altered the poor prognosis. Results reflecting the value of ifosfamide in osteosarcoma, Wilms' tumor and Hodgkin's and non-Hodgkin's lymphomas are pending. It is not yet known whether high doses of akylating agents will increase the risk of late sequelae in cured patients.
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PMID:Ifosfamide in pediatric malignancies. 264 85

Seven children underwent magnetic resonance imaging (MRI) of the bone marrow: results showed that it is technically feasible to obtain good MR images of marrow in children. MR has detected abnormality in the bone marrow of a child who had metastatic neuroblastoma. The extent of abnormality in the femur correlated well with findings of a bone marrow isotope scan. In one child who had idiopathic aplastic anemia, diseased marrow could not be distinguished from normal marrow on MR images. MRI identified abnormality of the marrow in osteogenic sarcoma, and demonstrated change in response to chemotherapy. It displayed marrow spread of tumor as well as CT. MRI showed marrow abnormality in four children who had leukemia.
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PMID:Magnetic resonance imaging of bone marrow disease in children. 658 59

Autologous bone marrow transplantation is used for children with cancers which are only partially responsive to bone marrow toxic doses of cancer chemotherapy and irradiation. The use of megatherapy and autologous bone marrow transplantation instead of conventional doses for children has yielded substantial benefits in B-cell lymphoma, relapse of leukaemia, disseminated neuroblastoma and germ cell tumors. In the case of Wilms' tumor, rhabdomyosarcoma, osteosarcoma and Ewing's sarcoma with a partial initial response or a relapse, autologous bone marrow transplantation has been used to a limited extent. Based on the best results from conventional therapy and autologous bone marrow transplantation, respectively, the present need for autologous bone marrow transplantation in Denmark is estimated to be 24 transplantations a year.
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PMID:[Autologous bone marrow transplantation in children]. 831 84

While advances in the treatment of pediatric cancers have increased cure rates, children with metastatic or recurrent solid tumors have a dismal prognosis despite initial transient responses to therapy. Autologous hematopoietic stem cell transplantation takes advantage of the steep dose-response relationship observed with many chemotherapeutic agents. While clearly demonstrated to improve outcomes in patients with metastatic neuroblastoma, autologous hematopoietic stem cell transplantation is also frequently used to treat patients with other high-risk diseases such as Ewing sarcoma, osteosarcoma, rhabdomyosarcoma, Wilms' tumor, retinoblastoma, germ cell tumors, lymphomas and brain tumors. Most published experience consists of retrospective, single-arm studies; randomized clinical trials are lacking, due in part to the rarity of pediatric cancers treatable by autologous hematopoietic stem cell transplantation. These published literature demonstrate that autologous hematopoietic stem cell transplantation results in most cases in equivalent or superior outcomes when compared with conventional therapies. However, patient heterogeneity, patient selection, graft characteristics and processing and the varied conditioning regimens are additional factors to consider. Since the inception of autologous hematopoietic stem cell transplantation, regimen-related toxicity has markedly decreased and the vast majority of treatment failures are now due to disease recurrence. Prospective clinical trials are needed to identify specific high-risk patient populations, with randomization (when possible) to compare outcomes of patients undergoing autologous hematopoietic stem cell transplantation with those receiving standard therapy. In addition, investigators need to better define the role of autologous hematopoietic stem cell transplantation in these solid tumors, particularly in combination with other therapeutic modalities such as immunotherapy and novel cell processing methodologies.
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PMID:Autologous hematopoietic stem cell transplantation for pediatric solid tumors. 1622 Oct 53

Facial swelling is a common clinical problem in pediatric patients. The causes of swelling are diverse, and knowledge of the typical clinical and imaging manifestations and the most common sites of occurrence of these conditions is needed to formulate a differential diagnosis. The general clinical manifestations may be classified into the following four groups: (a) acute swelling with inflammation, (b) nonprogressive swelling, (c) slowly progressive swelling, and (d) rapidly progressive swelling. Conditions that may account for acute swelling accompanied by inflammation include lymphadenitis, sinusitis, odontogenic infection, and abscess. Contrast-enhanced computed tomography is the modality of choice for detection of abscesses requiring surgical drainage. Nonprogressive midfacial swelling is suggestive of a congenital anomaly (eg, a cephalocele, nasal glioma, or nasal dermoid or epidermoid cyst). Slowly progressive swelling may indicate the presence of a neurofibroma, hemangioma, lymphangioma, vascular malformation, or pseudocyst, or of fibrous dysplasia. The differential diagnosis for rapidly progressive facial swelling in association with cranial nerve deficits should include rhabdomyosarcoma, Langerhans cell histiocytosis, Ewing sarcoma, osteogenic sarcoma, and metastatic neuroblastoma.
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PMID:Causes of facial swelling in pediatric patients: correlation of clinical and radiologic findings. 1641 50

Although neuroblastoma is a relatively common malignancy of childhood and its dissemination to distant organs is often seen, metastasis to the mandible is rare. A 3-year-old boy which a mandibular soft tissue mass was the initial presenting symptom of disseminated neuroblastoma is reported. The results of biopsy were inconclusive and the differential diagnosis from the imaging studies included lymphoma, soft tissue sarcoma, and osteosarcoma. A metastatic work-up disclosed neuroblastoma of the adrenal gland origin with osseous and bone marrow metastases. Urinary catecolamines were also increased. Regarding the widespread features of the tumor and lack of adequate treatment at this stage of disease, a palliative chemotherapy was conducted, and the patient died one month after starting treatment. This case illustrates that neuroblastoma at a young age, with bone metastases and bone marrow involvement are predictive of the poor outcome of the disease. Therefore, detecting early stage metastasis is one of the essential factors for improving treatment of neuroblastoma patients.
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PMID:Metastatic neuroblastoma to the mandible in a 3-year-old boy: a case report. 1746 14

In the pediatric population, malignant osseous tumors of the spine include osteosarcoma, Ewing sarcoma, lymphoma, and metastatic neuroblastoma. Although these tumors are rare, prompt diagnosis and recognition are critical to the overall prognosis. Improved understanding of the natural history of spine deformity, combined with advances in imaging, surgical technology, radiation therapy, and chemotherapeutic regimens, has improved survival rates and decreased rates of local recurrence-especially recurrence of low-grade lesions. Prognosis for patients with high-grade lesions with distant metastasis on presentation remains exceedingly poor. Recognition of these spine tumors and prompt referral to a tertiary care center that specializes in oncology can optimize patient outcomes.
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PMID:Malignant osseous tumors of the pediatric spine. 2302 94

Ganglioside GD2 is a tumor-associated surface antigen found in a broad spectrum of human cancers and stem cells. They include pediatric embryonal tumors (neuroblastoma, retinoblastoma, brain tumors, osteosarcoma, Ewing sarcoma, rhabdomyosarcoma), as well as adult cancers (small cell lung cancer, melanoma, soft tissue sarcomas). Because of its restricted normal tissue distribution, GD2 has been proven safe for antibody targeting. Anti-GD2 antibody is now incorporated into the standard of care for the treatment of high-risk metastatic neuroblastoma. Building on this experience, novel combinations of antibodies, cytokines, cells, and genetically engineered products all directed at GD2 are rapidly moving into the clinic. In this review, past and present immunotherapy trials directed at GD2 will be summarized, highlighting the lessons learned and the future directions.
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PMID:GD2-targeted immunotherapy and radioimmunotherapy. 2544 Jun 5