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)

Peripheral neuroectodermal tumours (PNET) of bone are rare and mimic those seen in soft tissue (peripheral neuroepithelioma of soft tissue). Their differential diagnosis from Ewing's sarcoma (Es) is extremely difficult by optical means. Here we report 14 new cases of PNET of bone (other than Askin's neoplasm) located primarily in the limbs, pelvic girdle and scapula. Clinically and radiologically they displayed Ewing's sarcoma-like features: mean age was 14.4 years, male/female ratio being 3:11. Metastasis was present in 6 cases at diagnosis (5 with bone metastasis). Prognosis was poor; thirteen patients died; only one with a metatarsal located tumour is alive and free of disease. The mean survival rate was 25 months following diagnosis and treatment with radio- and multimodal chemotherapy. Histologically the 14 cases displayed Homer-Wright rosettes and pseudorosette-like structures, as well as a fibrillary background and lobular pattern. Immunohistochemistry revealed positivity in a number of neural markers when using paraffin-embedded material: NSE, B-2-microglobuline, HNK-1 (leu-7) and E-36 antibodies. At EM level the cell cytoplasms evidenced dense-core granules with neurosecretion, neurotubules and intermediate filaments like those seen in peripheral neuroepithelioma.
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PMID:Malignant peripheral neuroectodermal tumours of bone other than Askin's neoplasm: characterization of 14 new cases with immunohistochemistry and electron microscopy. 283 33

A retrospective analysis, from 1965 to 1987, of 22 patients less than 18 years of age undergoing thoracotomy for pulmonary metastases from previously diagnosed malignancy, was performed. There were 15 males and seven females whose ages ranged from 8 months to 17, years. Ten patients had primary osteogenic sarcoma, five had Wilms' tumor, and seven had miscellaneous other tumors. A total of 41 thoracotomies were performed with no mortality. The overall survival rate was 54.5%, with an average survival of 6.2 years after initial diagnosis. The osteosarcoma group had a 50% survival rate after an average of 62 months from initial diagnosis, while the Wilms' tumor group had an 80% survival rate with a 100-month average. The remaining seven patients had a 29% average survival rate 62 months after diagnosis. Of the 12 patients undergoing wedge resections, two died upon follow-up 20 and 21 months after initial diagnosis. As opposed to the survivors in this group, both required more than four wedge resections upon initial thoracotomy. Two patients requiring extended resections, one for Ewing's sarcoma and one for hepatoblastoma, died 35 and 3 months after diagnosis, respectively. Of the eight patients undergoing lobectomy and/or segmentectomy, 75% died an average of 31.3 months after diagnosis. Ten patients had two or more thoracotomies for an average of 2.9, with a 40% survival rate. Of the 27% who presented with initial bilateral lung metastases, 33% survived. Forty-five percent of patients had a tumor-free interval of less than 12 months prior to thoracotomy, resulting in a 60% mortality rate.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pulmonary metastases in children: results of surgical treatment. 284 44

Using 31P-MR spectroscopy spectra with good signal-to-noise ratio were obtained in five different types of tumours (Ewing's sarcoma, osteosarcoma, malignant melanoma, metastases from a squamous cell carcinoma, parotid adenoma). Surface coils were used. Short- and long-term follow-up after chemotherapy was possible in some cases. In the short-term follow-up, changes in the phosphocreatine and inorganic phosphate resonances could be observed within minutes after the start of the infusion. In the longer follow-ups, changes in phosphodiester and phosphomonoester resonances were observed within two days. There were no significant changes in tissue pH during treatment, but increased pH values were observed in all tumours.
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PMID:[In vivo 31 phosphorus spectroscopy of tumors: pre-, intra- and post-therapy]. 284 3

Pulmonary nodules morphologically indistinguishable from bronchioloalveolar carcinoma (BAC) were found in two adolescent cancer patients postchemotherapy. A solitary nodule was noted at thoracotomy for a single computerized tomography (CT) scan lesion in a 16-yr-old male, 6 yr after diagnosis of Ewing's sarcoma. A similar nodule was found in a 19-yr-old male coincident with resection of multiple lung metastases of a testicular germ cell tumor. Both lesions were discrete nodular masses (1 cm and 0.5 cm) consisting of atypical epithelial cells with a papillary and lepidic growth pattern and surrounded by histologically normal appearing lung. Immunohistochemistry of both cases was positive for laminoorganel (LO) antigen, which is found in normal type II pneumocytes, and one nodule showed carcinoembryonic antigen (CEA) staining. Quantitative DNA analysis in one case indicated aneuploidy. Thus the morphology, immunohistochemistry, and DNA content of these lesions suggest that they may represent early lung cancers despite the highly unusual clinical context. The extreme rarity of BAC in this age group makes this report significant in light of heightening concern about second malignancies in pediatric cancer patients and reports of chemically induced pulmonary adenomas in mice. It also underscores the importance of basing therapeutic decisions on a histologic diagnosis of lung nodules in cancer patients rather than assuming the presence of metastatic disease.
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PMID:Pulmonary nodules resembling bronchioloalveolar carcinoma in adolescent cancer patients. 285 63

A case of a young man with Ewing's sarcoma of the os ilium is demonstrated. The tumor is removed by resection of the os ilium and the adjacent musculature. The bony stability is reconstructed by means of a double autogenous free fibula graft in combination with a broad plate, braced between os sacrum and the roof of the acetabulum. Full weight bearing is possible, at present there is no evidence of a local recidive or of metastases.
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PMID:[Stable osteosynthesis following ilial resection in intraosseous Ewing sarcoma]. 290 44

One hundred and ninety six thoracotomies were performed on 152 patients with pulmonary metastases up to 1988 in the Second Surgical Department, University of Vienna. Age ranged from 2 to 78 years, 13 patients were younger than 18 years. The primary tumour was carcinoma in 103 cases, sarcoma in 38 cases and melanoma in 11 cases. The primary tumour in young patients was osteosarcoma in 7 cases, Ewing sarcoma in 2 and Wilms tumour in 2 cases. With a minimal follow-up period of 2.5 years the actuarial 5 years survival rate of 37% was observed for carcinoma, and 29% for sarcoma patients. A statistical difference was found between the carcinoma and sarcoma groups with respect to survival rate; the prognosis for patients with melanoma was markedly worse. A prognostic factor was the duration of disease-free interval in carcinoma patients. Actuarial post-thoracotomy survival in patients with osteogenic sarcoma was 32% at 5 years and only 10% in the soft-tissue sarcoma group. Size of lesions, vitality of the metastases and disease-free interval correlated with survival in the osteogenic group, whilst the number of lesions was of importance in the soft-tissue group. On account of the lesser functional morbidity and the ability to assess both lungs for exploration, palpation and resections, the importance of median sternotomy is constantly increasing for the treatment of pulmonary metastatic disease and the results justify an aggressive approach. In those cases which the primary tumour is sensitive to chemotherapy the procedure of metastatic resection must be incorporated into the general scheme of oncological therapy.
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PMID:[Resection of pulmonary metastases: indications, surgical technic, results and prognostic factors]. 291 41

Conventional methods of treatment having failed in 17 children (aged 9/12 to 16 5/12 years) with incurable solid malignant tumours underwent whole-body hyperthermia (41.8-42.0 degrees C, for 2-3 h), hyperglycaemia (20-25 mmol/l) and polychemotherapy. Five children had neuroblastoma (stage 4), three Wilm's tumour (stage 4 or 5, unfavourable histology), five skeletal sarcoma with metastases, three inoperable malignant liver tumour, and one brainstem tumour of unknown histology. Whole-body hyperthermia was induced by extracorporeal blood warming in an haemodialysis apparatus under neuroleptic analgesia, thermistors measuring the temperature in the oesophagus, rectum, trachea and skin. There were on average four treatment sessions (between one and ten, total 58), a week apart. The result could be assessed in 12 children: one persisting complete remission (19 months-metastasising renal rhabdoid tumour), eight partial or incomplete remissions, and three nonresponders (osteogenic sarcoma; Ewing sarcoma; brainstem tumour). If the risk can be satisfactorily judged the method is useful and of bearable toxicity. The results point to a high antitumour effectiveness of combined hyperthermia and chemotherapy.
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PMID:[Treatment of otherwise incurable tumor diseases in childhood using whole-body hyperthermia and chemotherapy]. 291 80

The clinical presentation of the disease and the results of treatment in 48 patients with metastases at diagnosis of Ewing's sarcoma, entered into the Cooperative Ewing's Sarcoma Studies (CESS) 1981 and 1986 of the German Society of Pediatric Oncology (GPO), were analysed. The period of observation ranged from 1 to 82 months, the median relapse-free time was 26 months. There was a male predominance of 35 to 13, which was even more pronounced in patients older than 15 years. The predominant localization of the primary tumor was the pelvic region, followed by the extremities, the chest wall, and the spine. The most common site of primary metastases were the lungs, followed by bone and bone marrow. Nine patients presented with combined metastases. The disease-free survival according to Kaplan-Meier life-table analysis was 18% after 7 years. Best results were obtained in patients with pulmonary metastases only, who underwent surgical resection of the primary tumor and received radiation to the lungs. Without bone marrow transplantation the prognosis of patients with bone metastases was extremely poor.
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PMID:[Results of treatment of primary metastatic Ewing sarcoma. A retrospective analysis of 48 patients]. 306 62

One hundred twenty-four children and young adults with recurrent tumors, predominantly sarcomas, were treated with the combination of ifosfamide, etoposide, and the uroprotector, mesna (2-mercaptoethane sulphonate), in a phase II trial. The treatment regimen consisted of 12 cycles of therapy administered every 3 weeks. After evaluation of the tumor response to chemotherapy alone, radiation or surgery was used to eradicate residual sites of metastatic disease where possible. At the present time, 77 patients are evaluable for response to the chemotherapy; 43 of the patients have experienced a significant reduction in the tumor size in response to the chemotherapy alone (39 partial responses [PR] and four complete responses [CR]). Sixteen of 17 patients with Ewing's sarcoma, nine of 13 with rhabdomyosarcoma, four of eight with peripheral neuroepithelioma, three of eight with osteosarcoma, and 11 of 31 with other tumors have responded with a PR or CR. The toxicity of the regimen was acceptable. Moderate or severe toxicity evaluated on a per cycle basis included: neutropenia, 97%; thrombocytopenia, 32%; nephrotoxicity, less than 1%; mucositis, 1%; neurologic toxicity, 2%; nausea and vomiting, 13%; hemorrhagic cystitis, less than 1%. Fever was present after 33% of cycles and sepsis following 7%. One patient died due to sepsis and pancytopenia. At the present time, only seven of the 43 patients who responded to the chemotherapy regimen have relapsed, with a median follow-up of 10 weeks after the response. This drug combination is highly active in the treatment of recurrent sarcomas and other tumors in children and young adults.
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PMID:Ifosfamide with mesna uroprotection and etoposide: an effective regimen in the treatment of recurrent sarcomas and other tumors of children and young adults. 311 35

This is a retrospective analysis of bone scans in 72 patients with Ewing's sarcoma. Sites of primary disease were found to be evenly distributed among the axial skeleton and the proximal and distal extremities. Primary disease sites in the axial skeleton were more frequently associated with metastases. Fifty-three of these patients had their first scans done at presentation; among them, 25 were found to have metastases. Unsuspected metastases were picked up in 28 of all the patients. Metastases at presentation were seen to be associated with a poorer prognosis.
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PMID:Bone scanning in Ewing's sarcoma. 315 74


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