DISSOCIATION AND RECONSTITUTION OF AN ENDOTOXIN.
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OBJECTIVE: To assess the feasibility of detecting SYT-SSX fusion transcripts in paraffin-embedded tissues of synovial sarcoma by reverse transcription-polymerase chain reaction (RT-PCR). METHODS: RT-PCR was used to amplify the SYT-SSX fusion transcripts using archival formalin-fixed paraffin-embedded tumor specimens from a series of 37 synovial sarcoma cases. To investigate the specificity of the SYT-SSX fusion transcripts, a variety of non-synovial sarcoma tumors were included in the study as negative controls. The detected messages derived from fusion genes were confirmed by subsequent sequence analysis. RESULTS: SYT-SSX fusion transcripts were detected in 33 of 37 (89.2%) synovial sarcomas. None of the 34 cases of non-synovial sarcoma tumors showed amplified products of SYT-SSX fusion transcripts, although PBGD mRNA was detected in all specimens. Among 33 SYT-SSX-positive synovial sarcomas, 22 tumors had an SYT-SSX 1 fusion transcript, whereas 6 tumors had an SYT-SSX2 fusion transcript. Fusion types can not be distinguished in the remaining 5 cases. There was a significant relationship between SYT-SSX fusion type and histologic subtype. All 10 biphasic synovial sarcomas had the SYT-SSX1 fusion, whereas all tumors with SYT-SSX2 were of monophasic morphology (P < 0.05). CONCLUSIONS: RT-PCR can be applied to archival formalin-fixed paraffin-embedded tumor tissues as a sensitive and reliable technique for the diagnosis and differential diagnosis of synovial sarcoma. There is an association between SYT-SSX fusion type and histological subtype. SYT-SSX2 fusion transcripts can only be found in monophasic synovial sarcoma.
Thirty-five dogs with 37 soft tissue sarcoma tumors that were incompletely excised and treated with radiotherapy in the postoperative, adjuvant setting were reviewed. Variables evaluated included age, sex, tumor site, tumor histology, total tumor radiation dose, radiotherapy field size. time to recurrence, and survival. The majority of tumors were fibrosarcomas and hemangiopericytomas, but small numbers of other tumor types were also represented. Total tumor radiation dose ranged from 42 to 57 Gy given in 3- to 4.2-Gy daily fractions on a Monday through Friday schedule. Overall median survival was 1,851 days. Median time to local recurrence was greater than 798 days. Soft-tissue sarcoma tumors at oral sites had a statistically significant lower median survival (540 days) as compared to other tumor sites (2,270 days). Radiotherapy may be a useful adjuvant therapy for incompletely excised soft-tissue sarcomas with a reasonable expectation for long-term patient survival.
Monoclonal antibodies against the mouse sarcoma FS9 were prepared. Antibodies recognizing a 37,000 dalton glycoprotein on FS9 tumor cells inhibit invasion by FS9 sarcoma cells of chicken heart fibroblasts in vitro as assessed by Abercrombie's confronted explant assay. Antibodies to other membrane proteins of FS9 tumor cells failed to inhibit invasiveness of FS9 sarcoma cells. The 37,000 dalton glycoprotein, which is neither a histocompatibility antigen nor a gp37 glycoprotein of Rous sarcoma virus nor the MEP described by Gottesman, is present on the surface and in the cytoplasm of FS9 sarcoma cells. The plasma membrane of the non-invasive mouse cell line L929 contains only low concentrations of the 37,000 dalton antigen. Hence, a relationship apparently exists between the increased concentration of this protein on the surface of FS9 cells and their invasiveness.
BACKGROUND: Isolated limb perfusion (ILP) with melphalan is used in the treatment of advanced in-transit melanoma but has no real efficacy for irresectable soft tissue sarcomas arising in the extremities. The addition of tumor necrosis factor (TNF)-alpha may increase response rates for bulky melanoma and for sarcoma, but the potential for major systemic toxicity has limited its use. METHODS: Between October 2000 and April 2004, 49 ILPs were performed with melphalan and TNF-alpha. All procedures were performed with continuous leakage monitoring and regional hyperthermia. RESULTS: Forty-nine ILPs were performed for melanoma (n = 30), sarcoma (n = 16), or other tumors (n = 3). The most common indications were widespread in-transit disease for melanoma (n = 29) and irresectable primary disease for sarcoma (n = 9). Complete and partial responses for melanoma were 40% and 37%, and for sarcoma they were 20% and 33%. At a median follow-up of 14 months, 66% of melanoma patients who responded had not experienced local progression, compared with only 37% of sarcoma patients. Progression-free survival was significantly less for patients with sarcoma than melanoma (P = .0476). Four of 16 patients with sarcoma subsequently required amputation for progressive disease. CONCLUSIONS: ILP with melphalan and TNF-alpha is a valuable treatment for advanced in-transit melanoma. Significant response rates were also seen in irresectable sarcoma, although the duration of response was limited.
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The alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor which usually occurs in the lower extremity of young girls. The incidence of metastasis is said to be highest in the lung (38%), and second highest in the bone and brain (33%). This report describes two cases of metastatic intracerebral alveolar soft part sarcoma, originating in the lower extremity. A female patient noticed a painless swelling in her right leg at the age of 11, and 10 years later she underwent total removal of the tumor. The diagnosis was alveolar soft part sarcoma. At 37 she was admitted to our service with a diagnosis of cerebral metastasis in the left frontal lobe. Since then she has undergone surgical removal 4 times for recurrent cerebral metastasis and twice for lung metastasis. Now she is 55 years old and doing well except for mild left hemiparesis. She survives without cerebral or general metastasis 44 years following the onset of the sarcoma in her right leg and 18 years following the onset of the metastatic brain tumor. A 30-year-old man, who noticed a painless swelling in his left thigh in January 1991, underwent total removal of the tumor and the diagnosis was alveolar soft part sarcoma. He was admitted to our service with no neurological deficits in October 1992, but a CT scan showed a metastatic brain tumor in the left parieto-occipital region. Total removal of this metastatic brain tumor was successfully performed in November 1992. However, he died because of multiple brain and lung metastasis in February, 1994.(ABSTRACT TRUNCATED AT 250 WORDS)
The clinical records and autopsy data of 75 patients dying with AIDS were reviewed to determine the frequency of individual diseases diagnosed premortem and postmortem, the significance of pulmonary processes found in the lungs at autopsy, and the clinical and pathologic causes of death. Cytomegalovirus (CMV) infection was identified histologically either premortem or postmortem in 81% of patients. The lungs and adrenal glands were infected most commonly. Only one-half of CMV infections were recognized premortem. Pneumocystis pneumonia and Kaposi sarcoma occurred in 68% and 59% of patients, respectively, but were not unsuspected premortem in any patient. Visceral involvement with Kaposi sarcoma, however, was frequently recognized only at autopsy. While disseminated M. avium-intracellulare infection was common (31% of patients), histologically documented pulmonary disease was uncommon (3% of patients). Cryptococcal infection, diagnosed in 10 patients, was confined to the central nervous system in only 1 patient. Toxoplasma, in contrast, infected the brain of only 6 patients. All 75 patients had one or more disease processes identified in their lungs or pleurae at autopsy. These processes included opportunistic infections in 76% of patients, neoplasms in 37% (Kaposi sarcoma in 36% and lymphoma in 3%), and other processes in 60%. The most prevalent pathogen, CMV was found in pulmonary tissue from 44 patients and caused significant disease in 21 patients. Five patients died due to CMV pneumonia. Pneumocystis carinii was found at autopsy in 24 patients. In spite of treatment, pneumocystis pneumonia was fatal in 11 patients. One patient died with concomitant CMV and pneumocystis pneumonia. Kaposi sarcoma, identified in the lungs of 23 patients, led to death in 5 patients via upper airway obstruction, hemorrhage, or parenchymal destruction. Other fatal pulmonary processes included bacterial pneumonia in 9 patients, idiopathic diffuse alveolar damage in 5, cryptococcosis in 2, and pulmonary hemorrhage in 1. Specific clinical criteria were used to determine the cause of death due to organ system failure. Fifty-one percent of patients died due to respiratory failure; 16% from neurologic disease; 17% from hypotension that was not caused by respiratory, neurologic, or cardiac disease; and 3% from cardiac dysfunction. Thirteen percent of deaths did not meet the clinical criteria defining these 4 categories. This clinical assessment was combined with autopsy data to identify specific diseases as causes of death.(ABSTRACT TRUNCATED AT 400 WORDS)