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)

Grading of myxofibrosarcoma (MFS) is contentious and based on a variety of factors, such as the percentage of myxoid or solid areas, tumor necrosis, mitotic counts, and so on. These factors are often used in combination for different grading schemes, which have not been uniformly evaluated by consistent criteria for patients treated and prospectively followed up at a single institution. Because only a subset of low-grade (LG) MFS will progress to high grade and metastasize after relentless local recurrences, we analyze various histologic parameters and grading methods to identify prognostic predictors of the LGMFS. Forty-nine cases were classified as LGMFS after review, by using > or =30% of myxoid component, but < or =20% of solid areas and only focal, < or =10%, of tumor necrosis as cutoffs, as modified from a 2-tier system that is used in our hospital. These cases were also graded in parallel by French Federation of Cancer Centers Sarcoma Group (FNCLCC) 3-tier and 4-tier grading schemes. The study cohort consisted of 26 men and 23 women, with a median age of 60.5 years. Nineteen cases were superficial, and 30 were deep seated, with the most common site being the lower limb (57%), followed by the upper limb (31%), trunk (8%), and head and neck (4%). The primary tumors ranged from 1.5 to 24 cm in size. Solid areas (5% to 20%) were seen in 23 cases, tumor necrosis (5% to 10%) was observed in 4 cases, and a predominant myxoid area (> or =75%) was noted in 22 cases. Mitotic activity ranged from 0 to 16 (median, 2) per 10 HPF. Comparing FNCLCC 3-tier versus 4-tier grading, respectively, 26 versus 10 tumors were classified as grade I, and 23 versus 39, as grade II, with 16 cases (33%) graded discordantly by 2 schemes. A median follow-up of 55 months in 49 patients (range, 9 to 171 months) revealed local recurrence occurring in 28 patients (57%), 15 and 7 of which developed multiple local recurrences and distant metastases, respectively. There was only 1 case with pulmonary metastasis without a prior local recurrence. Currently, 33 patients are alive with no evidence of disease, 4 are alive with disease, 9 are dead of disease, and 3 are dead of unknown causes. The 5-year recurrence-free survival, metastasis-free survival (MeFS), and disease-specific mortality (DSM) rates were 41%, 90%, and 4.4%, respectively. Size of larger than 5 cm (P = 0.032), tumor necrosis (P = 0.033), and < 75% of myxoid area (P = 0.042) were significant risk factors for DSM; the former two (P = 0.011 for size larger than 5 cm, P = 0.038 for necrosis) were also significantly related to MeFS. Both FNCLCC 3-tier and 4-tier schemes failed to show a significantly better outcome in grade I LGMFS than grade II lesions with respect to all 3 endpoints. In conclusion, our data statistically validated the previous impression that even the blandest LGMFS still carries a recurrent potential that cannot be foreseen by either different grading schemes or other clinicopathologic parameters. However, DSM rate is significantly related to tumor necrosis, large size, and decreased myxoid area. Tumors having necrosis or exceeding 5 cm are at significant risk of metastatic relapse.
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PMID:Low-grade myxofibrosarcoma: a clinicopathologic analysis of 49 cases treated at a single institution with simultaneous assessment of the efficacy of 3-tier and 4-tier grading systems. 1513 37

Metastases are the most frequent tumors of the heart even though they seldom are recognized. Most primary cardiac tumors are benign. The main role of imaging is to differentiate a cardiac tumor from thrombus and rare pseudotumors: tuberculoma, hydatid cyst. Echocardiography is the fist line imaging technique to detect cardiac tumors, but CT and MRI are useful for further characterization and differential diagnosis. Myxoma of the left atrium is the most frequent benign cardiac tumor. It usually is pedunculated and sometimes calcified. Sarcoma is the most frequent primary malignant tumor and usually presents as a sessile infiltrative tumor. Lymphoma and metastases are usually recognized by the presence of known tumor elsewhere of by characteristic direct contiguous involvement. Diagnosing primary and secondary pericardial tumors often is difficult. Imaging is valuable for diagnosis, characterization, presurgical evaluation and follow-up.
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PMID:[Cardiac tumors]. 1521 45

ONYX-015 is a provisionally replication competent adenovirus with oncolytic activity in cells with malfunctioning p53. Sarcomas represent a rational target for this approach given the high frequency of p53 mutations (40-75%) and MDM-2 amplification (10-30%). We, therefore, undertook a phase I/II study of ONYX-015, days 1-5 every month administered intratumorally under radiographic guidance, in combination with MAP (mitomycin-C, doxorubicin, cisplatin) chemotherapy in patients with advanced sarcoma. Six patients were treated. Injected lesions included liver metastases in four patients and chest wall metastases in two patients. Sarcoma histologies were gastrointestinal stromal tumors (GIST, two patients), leiomyosarcoma (two patients), liposarcoma (one patient), and malignant peripheral nerve sheath tumor (1 patient). Dose escalation was performed from 10(9) plaque forming units (PFU)/dose (total dose of 5 x 10(9) PFU/cycle) to 10(10) PFU/dose (total dose of 5 x 10(10) PFU/cycle) without dose-limiting toxicity being encountered. Immunohistochemistry of the metastatic lesions prior to treatment showed that five out of six patients were positive for p53, while two patients also had mdm-2 overexpression. Adenoviral replication was detected in two out of six patient biopsies on day 5 of the first cycle, by in situ hybridization (ISH). Both patients were treated at the highest dose level. ONYX-015 viral DNA was detected by quantitative PCR in the plasma of 5/6 patients on day 5 of the first cycle, and up to day 12 (7 days after the last viral dose) in one patient who had extended sampling for viral kinetics performed, suggesting viral replication in sarcoma tissue. One patient with p53 mutation and MDM-2 amplification achieved a partial response to treatment that lasted 11 months. In conclusion, intratumoral administration of ONYX-015 in combination with MAP chemotherapy is well tolerated with no significant toxicity due to ONYX-015 being encountered. Detection of viral DNA in post treatment tumor specimens by ISH and detection of the ONYX-015 genome in the peripheral blood by quantitative PCR, up to 7 days after the last viral dose provide evidence for adenoviral replication. There was evidence of antitumor activity in one out of six patients. Further investigation of this approach in patients with recurrent sarcomas is warranted.
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PMID:Phase I-II trial of ONYX-015 in combination with MAP chemotherapy in patients with advanced sarcomas. 1564 67

We report a case of bilateral breast metastases from Ewing sarcoma of the femur. A 40-year-old woman presented with Ewing sarcoma of the left thigh, treated by complete surgical exeresis and chemotherapy. Secondary, a large tumor appeared in the left breast. Bone scintigraphy, chest, and abdominal computed tomographic scan were normal. A breast biopsy found a malignant tumor composed of small round cells consistent with the initial diagnosis. After the first cycle of chemotherapy, a tumor was discovered in the controlateral breast. After 5 cycles, residual tumors persisted in the 2 breasts. Tumor exeresis was performed and found bilateral breast metastases of Ewing sarcoma. Because of the early recurrence of the left breast tumor, segmentectomy of the right breast and left mastectomy were performed. The histopathological analysis confirmed Ewing sarcoma metastases in the left breast. Despite local radiotherapy, the clinical course was marked by lumbar bone metastasis, local chest evolution, and progression of the disease. Metastases to the breast by extramammary malignant neoplasms are unusual. Sarcoma is an extremely rare cause of breast metastases and our case is the first report of breast metastases from Ewing sarcoma.
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PMID:Bilateral breast metastases from ewing sarcoma of the femur. 1568 43

Sarcoma is a relatively rare malignant disease with high mortality, bad prognosis and response to conventional therapy. Two possible models of this disease were tested: the K2 rat sarcoma cell line, which was described previously, and the new rat R5-28 cell line derived from a spontaneously growing rat neoplasm with sarcoma morphology. While all rats inoculated with K2 cells developed tumours at 22th-25th day after inoculation (D = 22-25), only 60%-75% of R5-28-inoculated rats were affected by tumours. The frequency and progress of the disease depended on the number of inoculated cells. No metastases were detected in both cases. All affected animals showed large splenomegaly. A possible response of some immune system components to tumours was tested. No tumour-infiltrating lymphocytes were revealed in the tumour tissue. Anti-tumour antibodies were not found in tumour-bearing animal sera. Appropriate changes in peripheral blood lymphocyte subsets were explored. While the relative numbers of both NK cells and Tc were impaired, no changes were noted in numbers of CD4+CD8- T helper cells. Leukocytosis with highly increased numbers of CD11b+ myeloid cells displaying variable expression of CD4 was detected in terminal stages of the disease.
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PMID:Immune characterization of the Lewis rats inoculated with K2 sarcoma cell line and newly derived R5-28 malignant cells. 1641 9

Sarcoma is generally a rare disease in the US, with poor survival in patients with both primary angiosarcoma and metastatic disease from sarcoma and GIST. In order to determine if liver transplantation for sarcoma is a realistic option, we examined records of all patients in the US component of the Israel Penn International Transplant Tumor Registry were reviewed. Those patients with liver failure from primary or metastatic liver sarcoma were evaluated. Patient outcome analysis was then performed. Patient and tumor demographics were reviewed as well as patient survival after transplantation. 19 patients are identified having received liver transplantation after treatment for sarcoma of the liver, 6 patients with primary hepatic sarcoma and 13 patients with metastatic sarcoma of the liver. Recurrence was almost universal in 18 of 19 patients (95%) after a median interval of 6 months. Survival for the group as a whole was 47% for 1-year, 15% for 3-years and 5% for 5-years. Given the early recurrence of tumor and meager 1-year survival outcome, liver transplantation is a poor therapeutic choice for patients with either primary or metastatic liver sarcoma, including high-grade leiomyosarcoma (GIST) regardless of primary site or primary therapy.
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PMID:Liver transplantation for primary or metastatic sarcoma to the liver. 1642 26

All cases of high-grade osteosarcoma (OS) (n = 196) and Ewing's sarcoma of bone (ES) (n = 56) treated at the Norwegian Radium Hospital in the period 1980-1999 were analyzed retrospectively. They were allocated to consecutive ten-year periods by their time of diagnosis. Patient and tumour characteristics have been relatively stable. Eighty percent of all patients received surgical treatment and the amputation rate decreased from 64% to 23%. The percentage of patients receiving chemotherapy has remained around 80%. The use of radiotherapy in primary treatment decreased gradually from 33% to 18%. Sarcoma specific survival (SSS) at five years for all patients increased significantly from 39% to 53%. Similar trends for improvement were seen for both OS and ES. In multivariate analysis, independent prognostic factors for improved SSS were non-metastatic disease at diagnosis, age under 40, extremity tumours, small tumours and treatment from 1995 onwards. No major new treatment options have emerged over these 20 years. The improved outcome appears partly to be due to refinements in the use of existing modalities and improved quality and integration of multidisciplinary approaches. Improved formalized organisation of the sarcoma group and annual audited reports of its patient and research activity may also have contributed to improved focus and performance.
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PMID:Management of high-grade bone sarcomas over two decades: the Norwegian Radium Hospital experience. 1646 94

Treatment of retroperitoneal soft tissue sarcomas is a difficult clinical problem. Despite the improvement in resection rates in the most recent surgical series, local control still remains the main problem because of the high incidence of local recurrences after surgery. Postoperative radiation therapy has not been always successful because of dose-tolerance of surrounding normal structures, which prevent the delivery of adequate doses of radiation. To overcome this limitations, new therapeutic approaches including external-beam radiation and intraoperative radiation therapy (IORT) have been evaluated at some Institutions. The results of IORT with or without external-beam radiation are reviewed and our experience with preoperative radiation and IORT is reported. As treatment of retroperitoneal sarcomas has evolved into combined modalities including preoperative radiation, maximum surgical resection and IORT, a possible improvement in local control rates has been achieved. However, locoregional failures and the incidence of distant metastases remain a challenge, emphasising the need for further improvement in local and distant treatment. The new phase II trial, activated within the Italian Sarcoma Group, with preoperative concurrent chemo-radiation therapy and IORT is presented.
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PMID:Intraoperative radiation therapy for retroperitoneal soft tissue sarcomas. 1676 23

Background. Multifocal osteosarcoma is usually described as the occurrence of the tumour at two or more sites in a patient without pulmonary metastases and may be synchronous or metachronous. Case report. A previously well 21-year old male, who presented with a swollen, painful right knee with no history of trauma, was found to have a high-grade osteosarcoma of the distal tibia and proximal femur. He underwent resection and prosthetic replacement of the distal femur and proximal tibia and remains well 19 months after diagnosis. Discussion. Multifocal osteosarcoma is a rare condition with a poor prognosis. There is debate about whether it represents multiple primary tumours or metastatic disease.
Sarcoma 2006
PMID:Synchronous multifocal osteosarcoma: case report and literature review. 1725 58

Ewing sarcoma is the second most frequent primary bone cancer affecting children or young adults. Advances in molecular biology have revealed common chromosomal translocations such as EWS-FLI 1 among Ewing sarcoma and related diseases such as primitive neuroectodermal tumor (PNET), so these are considered as Ewing sarcoma family tumor (ESFT). Although fewer than 10% of patients with ESFT survived before establishment of modern multiagent chemotherapy, the multimodal therapeutic regimens including combination chemotherapy, radiotherapy, and surgery can cure 60% of patients with localized disease, due to the collaborative research in European-American or the international trials. The standard chemotherapy for localized ESFT now comprises vincristine, actinomycin D, cyclophosphamide and doxorubicin (VACD) in Europe or vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide (VDC-IE) in North America. Meanwhile, those with metastatic disease have a much worse outcome with an approximately 10-30% 5-year event-free survival rate. New American-European collaborative trials such as EURO-E.W.I.N.G.99 are in progress for further improvement of the cure rate in localized and metastatic ESFT. In Japan, Japan Ewing Sarcoma Study Group (JESS) phase II clinical trial for localized ESFT, and some clinical trials including new drugs are ongoing and waiting for results.
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PMID:[Ewing sarcoma]. 1730 23


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