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PubMed1990

Synovial sarcoma: an immunohistochemical and ultrastructural study.

Thirty-nine primary synovial sarcomas (15 biphasic, 24 monophasic), and 19 metastatic synovial sarcomas were studied with a battery of antibodies directed to keratin, epithelial membrane antigen (EMA), carcinoembryonic antigen (CEA), vimentin, desmin, muscle-specific actin, smooth muscle actin, S-100 protein, Leu-7, chromogranin A, laminin, collagen IV, Ulex europaeus agglutinin I (UEAI), and the HMB-45 antimelanoma antibody. Twenty-two primary and 18 metastatic synovial sarcomas were also examined by electron microscopy. Epithelial and/or spindle cells in every biphasic tumor, primary and metastatic, reacted for keratin and EMA, but only six primary tumors (five biphasic and one monophasic) showed weak reactivity for CEA which, in the biphasic tumors, was confined to the epithelial component. Of the monophasic tumors, 15 primary (63%) and four metastatic (25%) stained for keratin, whereas seven primary (29%) and two metastatic (13%) tumors reacted for EMA. Only one primary monophasic synovial sarcoma stained for CEA. Tumors that stained for EMA or CEA also stained for keratin which is, therefore, the most useful epithelial marker. Immunostaining for epithelial markers, UEAI, collagen IV, and laminin serves to delineate the epithelial component when it is obscure in routine sections. Electron microscopy facilitates the diagnosis when epithelial markers are not expressed and aids in separating monophasic synovial sarcomas from other sarcomas that they resemble by light microscopy.

Adolescent↗
PubMed1992

Risk of Kaposi's sarcoma and sexual practices associated with faecal contact in homosexual or bisexual men with AIDS.

The causal agent of Kaposi's sarcoma is unknown. That the disorder is ten times more common in homosexual or bisexual men with the acquired immunodeficiency syndrome (AIDS) than in other human immunodeficiency virus (HIV) transmission groups suggests that a certain aspect of their behaviour exposes them to the agent or facilitates its spread. We therefore assessed social and demographic characteristics, including sexual behaviour, of 65 homosexual or bisexual men with AIDS from London. Sexual practices in which there was contact with partner's faeces before AIDS developed were the main determinants of Kaposi's sarcoma risk. Risk increased with frequency of insertive "rimming" (oral-anal contact): Kaposi's sarcoma developed in 18% of the men with AIDS who reported never having practised insertive rimming compared with 50% who practised it less than once a month, 73% between once a week and once a month, and 75% or more once a week (two-sided exact p-value for trend less than 0.001). 45 men had been interviewed about their sexual practices before AIDS developed, and 20 were interviewed at the time the syndrome developed. The findings were similar and statistically significant when each group was analysed separately. The men with Kaposi's sarcoma also tended to be more sexually active and were more likely to engage in other sexual activities that entailed contact with faeces than were the men who had other features of AIDS only. Other behaviours and exposures, including the use of "poppers" (nitrite inhalants), were not related to Kaposi's sarcoma risk, after taking into account whether the subjects had practised insertive rimming. The data suggest that faecal-oral contact is the main route of transmission of the agent of Kaposi's sarcoma in homosexual or bisexual men with AIDS.

Acquired Immunodeficiency Syndrome↗
PubMed1989

Neopterin and alpha-interferon in patients affected by Kaposi's sarcoma from Africa.

The presence of circulating alpha-interferon and neopterin was investigated in sera of 47 patients affected by African Kaposi's sarcoma, both HIV-seropositive (13 patients) and HIV-seronegative (34 patients). For comparison, analyses were also performed in 20 HIV-seropositive symptomatic African subjects as well as in 20 African and 20 Italian healthy individuals. Alpha-interferon and neopterin levels appeared significantly higher in comparison with healthy control groups (P less than 0.001) but not with HIV-seropositve African individuals without Kaposi's sarcoma. Moreover, alpha-interferon and neopterin levels were significantly higher in progressive Kaposi's sarcoma (27 patients) than in regressive Kaposi's sarcoma (20 patients) (P less than 0.001). A significant correlation between alpha-interferon and neopterin was observed (r = 0.57; P less than 0.01). Furthermore, alpha-interferon levels of HIV-seropositive Kaposi's sarcoma patients resulted significantly higher in comparison with the seronegative ones (P less than 0.05). It is concluded that alpha-interferon and neopterin may be reliable prognostic markers in Kaposi's sarcoma patients.

Adult↗
PubMed1989

Inhibitory properties of low molecular mass cysteine proteinase inhibitors from human sarcoma.

Elevated activities of cysteine proteinases such as cathepsins B and L and cancer procoagulant have been linked to tumor malignancy. In the present study we examined the hypothesis that these elevated activities could be due to impaired regulation by the endogenous low molecular mass cysteine proteinase inhibitors (cystatins). Inhibitors from human sarcoma were compared to those from human liver, a normal tissue in which the inhibitors had been characterized previously. An extract of cystatins from sarcoma was less effective against papain and cathepsin B (liver or tumor) than was an extract from liver. This reduced inhibitory capacity in sarcoma was not due to a reduction in either the concentrations or specific activities of the cystatins or an absence of any family or isoform of cystatins. We purified two members of the cystatin superfamily (stefin A and stefin B) to homogeneity and determined their individual inhibitory properties. Stefins B from liver and sarcoma exhibited comparable inhibition of papain and cathepsin B. In contrast, stefin A from sarcoma exhibited a reduced ability to inhibit papain, human liver cathepsins B, H and L and human and murine tumor cathepsin B. The Ki for inhibition of liver cathepsin B by sarcoma stefin A was 10-fold higher than that for inhibition of liver cathepsin B by liver stefin A, reflecting a reduction in the rate constant for association and an increase in the rate constant for dissociation. Cancer is now the third pathologic condition reported to be associated with alterations in cystatins, the other two being amyloidosis and muscular dystrophy.

Chromatography, Affinity↗
PubMedDate not supplied

Moritz Kaposi: idiopathic pigmented sarcoma of the skin.

Moritz Kaposi first published a description of the entity that bears his name in 1872, calling it "idiopathic multiple pigmented sarcoma of the skin." For many years thereafter, Kaposi's sarcoma was considered to be a relatively rare, slow-growing malignancy, most commonly seen in middle-aged and elderly men. This changed in 1981, with Alvin Friedman-Kein's report of what eventually proved to be HIV-associated (epidemic) Kaposi's sarcoma. Kaposi's sarcoma is now one of the diagnostic markers of the acquired immunodeficiency syndrome, as well as the most common neoplastic complication of that disease. HIV-associated Kaposi's sarcoma tends to disseminate widely to mucous membranes and the viscera. Almost all of the cases reported in the United States have occurred in homosexual and bisexual men, and a number of hypotheses have been suggested to explain this association. Over the past several years, however, the incidence of HIV-associated Kaposi's sarcoma has declined, and the reasons for this are also uncertain.

Acquired Immunodeficiency Syndrome↗
PubMed2005

Expression profiles of mouse dendritic cell sarcoma are similar to those of hematopoietic stem cells or progenitors by clustering and principal component analyses.

We isolated and screened two tumor cell clones DD1 and DG6 with different capacity of metastasis from the same parent cell line, a mouse dendritic cell (DC) sarcoma, using limited dilution method. The genome-wide expressions of DD1 and DG6 cells were detected by Affymetrix's MOE-430A microarray. The expression profiles related with mouse DC development were downloaded from GEO at NCBI and ArrayExpress at EBI database. In order to compare the expression of DC sarcoma and DC developmental arrays which was performed by MG-U74av2, we had screened the best matched probesets between MOE-430A and MG-U74av2 according to the probe identities from Affymetrix technical annotation. After the normalization of 11 housekeeping genes across the 34 arrays (2 DC sarcoma and 32 DC developmental arrays), all these expression profiles were analyzed by the methods of hierarchical clustering, principal component analysis, nearest-neighborhood, and self-organizing maps. The results indicate that expression profiles of DC sarcoma are closer to those of the DC progenitors and hematopoietic stem cells from bone marrow compared with the sorted DCs from spleen. The results support the hypothesis that cancers (tumors or sarcomas) arise from stem cells. It is suggested that the DC sarcomas are more similar to the DC progenitors and hematopoietic stem cells than the relative mature DCs in gene expressions on the large-scale.

Animals↗
PubMed2005

Spindle cells and their role in Kaposi's sarcoma.

Spindle cells represent the main cell type of the advanced final nodular stage of Kaposi's sarcoma lesions. Despite some clinical and epidemiological differences, the four Kaposi's sarcoma forms (classic, endemic, post-transplant and epidemic) display very similar histopathological features, with the proliferation of spindle cells (considered as the Kaposi's sarcoma tumor cells) associated with inflammation and neo-angiogenesis. Electron-microscopy and immuno-histochemistry studies have led to the consensus that the spindle cells originated from the endothelial lineage. However, only recently, studies that used specific lymphatic immunological markers (such as podoplanin) and molecular features (gene expression microarrays) strongly linked Kaposi's sarcoma spindle cells to the endothelium lymphatic cell lineage. Both hybridization and immuno-histochemistry techniques have demonstrated that human herpesvirus 8 also known as Kaposi's sarcoma associated herpesvirus was present in spindle cells at all stages of the disease (patch, plaque, nodule). Interestingly, while the human herpesvirus 8 latent genes are expressed in nearly all tumor spindle cells, only a small fraction of them expresses markers of viral lytic replication. Recent findings showing that nodular Kaposi's sarcoma lesions display all patterns of human herpesvirus 8 clonality support the model according to which this tumor begins as a polyclonal disease with a subsequent evolution to a mono/oligoclonal process involving infected spindle cells. Spindle cells appear to be the central masterpiece in KS tumorigenesis, however the exact respective role of each human herpesvirus 8 gene, in the initiation and the disease progression is still under investigation and the question of whether or not this tumor is a reactive process or a true malignant proliferation of spindle cells remains yet unclear.

Cell Lineage↗
PubMed2004

Multicolor fluorescence in situ hybridization analysis of a synovial sarcoma of the larynx with a t(X;18)(p11.2;q11.2) and trisomies 2 and 8.

Approximately 10% of all mesenchymal malignancies are classified as synovial sarcomas, which show a balanced translocation t(X;18)(p11.2;q11.2) at the cytogenetic level. The occurrence of this neoplasm in the head and neck region is rare, and its lowest frequency is found in the larynx. When synovial sarcomas are present in such unusual locations, diagnosis based solely on histologic features might be problematic, and to our knowledge, cytogenetic data have been reported so far in only one case of synovial sarcoma of the larynx. Because of the rarity and shortage of consistent prognostic markers, there is no clear consensus for the treatment of these patients. Cytogenetic analysis of a primary case of synovial sarcoma of the larynx was performed by using a 48-color fluorescence in situ hybridization technique that allows differential staining of short and long chromosome arms to establish the karyotype. We report here the molecular cytogenetic analyses of a synovial sarcoma of the larynx harboring the diagnostic t(X;18), as well as trisomies 2 and 8. The karyotypic information on synovial sarcomas of the larynx is scarce, and our data might add to the diagnosis and prognosis of this tumor.

Chromosomes, Human, Pair 18↗
PubMed2006

[Soft tissue sarcomas: update on molecular data].

Soft tissue sarcomas are rare and may be a source of problems for diagnosis and treatment. Four types of genetic disorders can be distinguished: translocations, gene amplifications, mutations and complex genetic imbalances. Detection of these disorders may help in diagnosis and in determining prognosis. Detection of specific translocation is recommended in synovial sarcoma, alveolar rhabdomyosarcoma or PNET diagnosis because of therapeutic consequences; in case of rarer histologic type (low grade fibromyxoid sarcoma, clear cell sarcoma, infantile fibrosarcoma...), it may confirm the diagnosis. In some cases, some translocations have a prognostic value (alveolar rhabdomyosarcoma) whereas it is discussed in others (synovial sarcoma). The techniques used to detect these translocations are very sensitive so it may be used to detect microscopical metastasis (bone marrow metastasis of alveolar rhabdomyosarcoma for example). Detection of MDM2 and CDK4 genes amplifications (FISH or quantitative PCR) may be sometimes useful in well differentiated and dedifferentiated liposarcomas diagnosis. Mutation detection of KIT or PDGFRA may help in GIST diagnosis and type of mutation is predictive of response to treatment. Study of complex genomic imbalances in sarcomas is not used in routine practice but remains useful in research.

Humans↗
PubMedDate not supplied

Review of the M.D. Anderson experience in the treatment of bladder sarcoma.

OBJECTIVE: To assess the histologic subtypes, clinical presentations, treatment approaches, and treatment-related outcomes of patients with bladder sarcoma. METHODS: Between January 1985 and July 2004, 19 patients (12 men and 7 women) with primary bladder sarcoma were evaluated at the University of Texas M.D. Anderson Cancer Center. Median follow-up duration was 72 months (range 3-141). RESULTS: The median age of patients at presentation was 57 years (range 22-94). The histologic subtypes of bladder sarcoma were leiomyosarcoma (N = 14), angiosarcoma (N = 3), and unclassified sarcoma (N = 2). The clinical presentation consisted of gross, painless hematuria in 79% of patients, lower urinary tract symptoms in 16%, and microhematuria in 5%. The primary treatment modalities used were surgery in 16 (84%) patients, chemotherapy in 2 (11%), and palliation in 1 (5%). The rate of local and distal recurrence was 16% and 53%, respectively. The most common sites of distant metastases were the lungs, bone, brain, and liver. The 5-year disease-specific survival rate was 59%, with a median survival duration of 6 years. There was no statistically significant difference in disease-specific survival between patients with bladder leiomyosarcoma compared to other sarcoma subtypes (P = 0.149). Lymphovascular invasion (P = 0.03) and lymphatic metastasis (P = 0.03) were associated with disease-specific survival, and surgical margin status was associated with recurrence-free (P = 0.04), disease-specific (P = 0.03), and overall survival (P = 0.005). CONCLUSIONS: Bladder sarcoma is a highly aggressive malignancy, regardless of its histologic subtype. Surgical margin status is an important determinant of survival.

Adult↗
PubMed2005

Systemic therapy for advanced uterine sarcoma: a systematic review of the literature.

OBJECTIVE: To conduct a systematic review of the literature regarding the systemic treatment of advanced uterine sarcoma and provide an evidence-based summary of the available literature. METHODS: MEDLINE, EMBASE, and the Cochrane Library databases were searched. "Uterine sarcoma," "leiomyosarcoma," "mixed mesodermal tumor," "chemotherapy," and "systemic therapy" were combined with the search terms for study designs. RESULTS: Three randomized controlled trials and 24 prospective phase II trials were included in the systematic review. In a randomized trial of doxorubicin versus doxorubicin plus cyclophosphamide for advanced or recurrent uterine sarcoma, doxorubicin produced an overall response rate (RR) of 19% and median survival of 11.6 months, which was similar to the response with combination chemotherapy (RR 19%, median survival 10.9 months). A randomized trial comparing ifosfamide plus cisplatin versus ifosfamide alone in mixed mesodermal tumors showed a significant improvement in RR and progression-free survival with the combination compared with ifosfamide alone, however, the combination was associated with increased toxicity including death. A randomized trial comparing doxorubicin to doxorubicin with dacarbazine in women with advanced or recurrent uterine sarcoma demonstrated a significantly higher RR with the combination (P < 0.05), but no significant difference in survival. CONCLUSIONS: Offering palliative chemotherapy to patients with advanced, unresectable uterine sarcoma who are symptomatic from this disease is a reasonable decision. Doxorubicin is an option for women with advanced uterine sarcoma. The combination of cisplatinum and ifosfamide is also an option for women with metastatic mixed mesodermal tumors; however, this combination is associated with significant toxicity when compared to ifosfamide alone.

Clinical Trials, Phase II as Topic↗
PubMed2005

A case of monophasic synovial sarcoma presenting as a vulvar mass.

BACKGROUND: Synovial sarcoma is the fourth most commonly occurring sarcoma, accounting for 8-10% of all sarcomas. They arise from unknown pleuripotent stem cells that are capable of differentiating into mesenchymal and/or epithelial structures. Synovial sarcoma is characterized by specific chromosomal translocation t (X; 18)(p11, q11). CASE: We report the first case of monophasic synovial sarcoma arising in soft tissues of the vulva in a 33-year-old female. Complete excision of the mass was possible with tumor-free margins. CONCLUSION: Only four previous cases of biphasic synovial sarcoma arising in the vulva have been reported. The finding of the SYT-SSX2 translocation is generally associated with a better prognosis, besides tumor negative margins after excision offer the possibility of a better outlook for this patient.

Adult↗
PubMed2003

Classification and subtype prediction of adult soft tissue sarcoma by functional genomics.

Adult soft tissue sarcomas are a heterogeneous group of tumors, including well-described subtypes by histological and genotypic criteria, and pleomorphic tumors typically characterized by non-recurrent genetic aberrations and karyotypic heterogeneity. The latter pose a diagnostic challenge, even to experienced pathologists. We proposed that gene expression profiling in soft tissue sarcoma would identify a genomic-based classification scheme that is useful in diagnosis. RNA samples from 51 pathologically confirmed cases, representing nine different histological subtypes of adult soft tissue sarcoma, were examined using the Affymetrix U95A GeneChip. Statistical tests were performed on experimental groups identified by cluster analysis, to find discriminating genes that could subsequently be applied in a support vector machine algorithm. Synovial sarcomas, round-cell/myxoid liposarcomas, clear-cell sarcomas and gastrointestinal stromal tumors displayed remarkably distinct and homogenous gene expression profiles. Pleomorphic tumors were heterogeneous. Notably, a subset of malignant fibrous histiocytomas, a controversialhistological subtype, was identified as a distinct genomic group. The support vector machine algorithm supported a genomic basis for diagnosis, with both high sensitivity and specificity. In conclusion, we showed gene expression profiling to be useful in classification and diagnosis, providing insights into pathogenesis and pointing to potential new therapeutic targets of soft tissue sarcoma.

Adult↗
PubMed2000

Radiation-induced gastrointestinal stromal sarcoma of the esophagus.

Esophageal sarcoma is an uncommon tumor, sporadically reported in the literature. Radiation therapy is frequently employed in the treatment of carcinoma of the esophagus, and the increased risk of development of sarcoma arising in irradiated fields is well known. However, to our knowledge, the occurrence of radiation-associated sarcoma of the esophagus after radiation therapy for carcinoma of the esophagus has not been reported. We therefore report the case of a 43-year-old female who developed a gastrointestinal stroma sarcoma 9 years following radiation therapy for esophageal squamous cell carcinoma. The patient underwent resection of her gastrointestinal stromal sarcoma by transhiatal esophagectomy with cervical anastomosis and is doing well 18 months later. The increasing use of radiation therapy for esophageal carcinoma suggests that radiation-associated sarcoma of the esophagus may be seen more frequently in the future.

Adult↗
PubMed2006

[Intraoperative therapeutic intensification techniques in locally advanced abdominal sarcomas].

INTRODUCTION: Sarcomas are rare tumors that develop from mesenchymal cells. Their management is difficult due to their changing histology, location, and behavior. In this article, we discuss the use of two intraoperative therapeutic intensification techniques, intraoperative radiotherapy (IORT) and hyperthermic intraoperative intraperitoneal chemotherapy (HIIC), in the treatment of locally advanced abdominal sarcomas and peritoneal sarcomatosis. MATERIAL AND METHODS: We analyzed a series of 20 consecutive patients diagnosed with advanced abdominal sarcoma and 5 patients with a diagnosis of peritoneal sarcomatosis who were evaluated and treated in our department from December 1996 to October 2005. In advanced abdominal sarcoma, we performed complete or maximal resection followed by IORT. In peritoneal sarcomatosis we performed massive cytoreduction followed by HIIC. RESULTS: The survival rate in advanced abdominal sarcomas without sarcomatosis was 65% at 26 months. Among the 5 patients diagnosed with peritoneal sarcomatosis, 3 were alive, and 2 were without recurrence at 20 months of follow-up. CONCLUSIONS: IORT associated with radical surgery seems to improve local control and survival in advanced abdominal sarcomas. Maximal cytoreduction plus HIIC used as treatment of peritoneal sarcomatosis is a feasible technique that offers a therapeutic option with curative intent.

Abdominal Neoplasms↗
PubMed1995

Gastroscopic findings in Mediterranean Kaposi's sarcoma (non-AIDS).

The frequency and degree of gastrointestinal involvement in patients with Mediterranean Kaposi's sarcoma (non-AIDS), a newly recognized form of Kaposi's sarcoma, is unknown. Eighty-seven patients with Mediterranean Kaposi's sarcoma proven by skin and/or nodal biopsy underwent endoscopic study of the upper gastrointestinal tract. Of these, 71 (81.6%) had gastrointestinal lesions. All these patients had lesions in the stomach. Additional lesions were detected in the esophagus in 19 patients and in the proximal duodenum in 8 patients, whereas additional lesions in both the esophagus and duodenum were identified in 2 patients. The lesions were classified into 4 types according to their size, shape, and color. Most types of lesions showed characteristic discoloration, but lesions with the appearance and color of normal mucosa that histologically were shown to be Kaposi's sarcoma were also identified. The high prevalence of gastrointestinal involvement in patients with Mediterranean Kaposi's sarcoma (non-AIDS) suggests that an endoscopic examination of the upper gastrointestinal tract may be useful in non-AIDS-related forms of Kaposi's sarcoma.

Adult↗
PubMed2003

Epidermal growth factor receptor expression in follicular dendritic cells: a shared feature of follicular dendritic cell sarcoma and Castleman's disease.

The factors regulating the growth of follicular dendritic cell (FDC) sarcoma are currently unknown. Using cDNA microarray analysis, we found that the epidermal growth factor receptor (EGFR) is expressed in FDC sarcoma. We immunohistochemically examined the expression of EGFR in a larger series of FDC sarcomas and in nonneoplastic FDCs. This included 8 cases of FDC sarcoma, 12 cases of hyaline vascular Castleman's disease (CD), 5 cases of human herpesvirus 8 (HHV8)-positive plasma cell CD, 7 cases of HHV8-negative plasma cell CD, 13 cases of reactive lymph nodes, 3 cases of reactive tonsils, 10 cases of follicular lymphoma, 6 cases of nodular mantle cell lymphoma, and 6 cases of angioimmunoblastic T-cell lymphoma. EGFR was expressed in tumor cells in 7 of 8 cases (88%) of FDC sarcoma (strongly in 4 cases and moderately in 3 cases). The single EGFR-negative case had an anaplastic appearance and a more aggressive clinical behavior. EGFR was also expressed by FDC in all types of CD (strongly in 4 cases, moderately in 16 cases, and weakly in 4 cases). Immunostaining results were negative or only weakly positive for EGFR in FDC of reactive lymph nodes and tonsils, and in the FDC networks of follicular lymphoma, mantle cell lymphoma, and angioimmunoblastic lymphoma. The up-regulation of EGFR in FDC of CD was paralleled by an increase in EGFR expression in the surrounding perifollicular fibroblastic reticulum cells suggesting coordinate regulation. These findings identify a differentially expressed growth regulatory receptor common to both FDC sarcoma and CD, identifying a target for possible therapy in unresectable or refractory cases.

Adult↗
PubMed1984

Keratin proteins and carcinoembryonic antigen in synovial sarcomas: an immunohistochemical study of 24 cases.

Twenty-four synovial sarcomas were examined for the presence of keratin proteins by an indirect immunoperoxidase method with paraffin-embedded tissues. Keratin proteins were identified in 16 of 24 cases (67 per cent). Both the pseudoglandular and spindle cell areas of all eight of the biphasic synovial sarcomas and the spindle cells of eight of the 16 monophasic synovial sarcomas contained keratin proteins. In spindle cell areas, staining was observed in single cells and small cords and clusters of cells in the absence of cleft formation or other evidence of a pseudoglandular component. The predominant cytologic staining pattern in all cases was peripheral, with localization of staining to the cell membrane or adjacent areas, but diffuse and focal cytoplasmic staining patterns were also observed. No staining for keratin proteins was seen in 101 control cases, including 52 sarcomas of various types. Carcinoembryonic antigen was also identified in four of the 24 synovial sarcomas by an indirect immunoperoxidase technique. The identification of keratin proteins may be helpful in the pathologic diagnosis of synovial sarcoma, particularly the spindle cell monophasic variant.

Adult↗