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PubMed2004

Abdominal small round cell tumor with osteoid and EWS/FLI1.

In this report, we described a case of multiple intraperitoneal tumors. Histologically, the tumors were composed of small round cells with malignant phenotype, necrotic areas, and islands of osteoid matrix in the stroma. In immunohistochemical and molecular analyses, the tumors expressed CD99 and EWS-Fli1 fusion gene. Production of osteoid by small round tumor cells was consistent with the histologic criteria of small-cell osteosarcoma, whereas expression of EWS-Fli1 was a characteristic genetic feature of Ewing's sarcoma family of tumor. Such tumors have been limited to a case in which histologically proven small-cell osteosarcoma of the scapula showed a chromosomal translocation, t(11;22)(q24;q12).

12E7 Antigen↗

PubMed2006

Suppression of KCMF1 by constitutive high CD99 expression is involved in the migratory ability of Ewing's sarcoma cells.

High CD99 expression levels and rearrangements of the EWS gene with ETS transcription factor genes characterize the Ewing's sarcoma family of tumors (ESFT). CD99 is a cell surface glycoprotein whose engagement has been implicated in cell proliferation as well as upregulation and transport of several transmembrane proteins in hematopoietic cells. In ESFT, antibody ligation of CD99 induces fast homotypic cell aggregation and cell death although its functional role in these processes remains largely unknown. Here, using an RNAi approach, we studied for the first time the consequences of modulated CD99 expression in six different ESFT cell lines, representing the most frequent variant forms of EWS gene rearrangement. CD99 suppression resulted in growth inhibition and reduced migration of ESFT cells. Among genes whose expression changes in response to CD99 modulation, the potassium-channel modulatory factor KCMF1 was consistently upregulated. In a series of 22 primary ESFT, KCMF1 expression levels inversely correlated with CD99 abundancy. Cells forced to express ectopic KCMF1 showed a similar reduction in migratory ability as CD99 silenced ESFT cells. Our results suggest that in ESFT, high CD99 expression levels contribute to the malignant properties of ESFT by promoting growth and migration of tumor cells and identify KCMF1 as a potential metastasis suppressor gene downregulated by high constitutive CD99 expression in ESFT.

12E7 Antigen↗

PubMed1990

[A clinico-experimental study of patients with mycosis fungoides and Kaposi's sarcoma].

Twenty-six cultures were prepared by the cultivation method. Continuous cells lines (IK-10 and IK-12) were obtained in two cases, in the rest short-lived cell cultures were prepared. All the isolated cell cultures were studied by transmission electron microscopy. Abnormal lymphoblasts were detected in some cell cultures isolated from the blood and lymph nodes of mycosis fungoides (IJ-137, IK-140, IK-142, IK-143) and Kaposi's sarcoma patients (IK-145). Cell population consisted of small, medium, and large lymphocytes. Electron microscopic examination has revealed retroviruses in IK-143 culture, isolated from the lymph node of a patient with mycosis fungoides. Morphologically these particles were typical of type C oncoviruses, 120-140 nm in diameter, with a spherical core in the center of the virion.

Adolescent↗

PubMed1978

Glucocorticoids induce focus formation and increase sarcoma viral expression in a mink cell line that contains a murine sarcoma viral genome.

Dexamethasone (3 X 10(-10) to 3 X 10(-6) M) induced foci of morphologically transformed cells in a small proportion of a mink cell line that contains the Moloney murine sarcoma viral genome (S+L-). The induction was glucocorticoid specific, since other steroids with glucocorticoid activity (prednisolone, cortisol, and aldosterone) induced foci with an efficiency that paralleled their glucocorticoid activity, and steroids lacking glucocorticoid activity (17B-estradiol, testosterone, and progesterone) failed to induce foci. Viral antigen, as measured by specific immunofluorescence, was localized to the foci. The induction of foci by dexamethasone (3 X 10(-7)) was accompanied by an approximately 10-fold increase in intracellular Moloney murine sarcoma virus-specific RNA and viral p30 antigen. Removal of dexamethasone was followed by the disappearance of foci and a decrease in viral RNA and p30. In this cell system, therefore, glucocorticoids can affect the intracellular levels of type C viral RNA and protein.

Animals↗

PubMed1991

Immunohistochemical analysis of Ewing's sarcoma cell surface antigen p30/32MIC2.

Monoclonal antibody (MAb) HBA71, which was raised against Ewing's sarcoma cells, recognizes a cell-surface glycoprotein, p30/32MIC2, that is encoded by the MIC2 gene in the pseudoautosomal region of human chromosomes X and Y. This immunohistochemical study evaluates the specificity and sensitivity of MAb HBA71 for tumor diagnosis. Frozen and paraffin-embedded tissues of more than 300 tumors of diverse histologic type, including more than 100 small round cell tumors of childhood and adolescence, were tested with this MAb by the avidin-biotin immunoperoxidase procedure. The authors found HBA71 immunoreactivity in 61 of 63 Ewing's sarcomas studied and 9 of 11 primitive neuroectodermal tumors and peripheral neuroepitheliomas. HBA71-negative tumors included neuroblastomas (0 of 24), melanomas (0 of 13), an esthesioneuroblastoma, small cell osteosarcomas (0 of 2), a malignant ectomesenchymoma, desmoplastic SRCT (0 of 5), and medulloblastomas (0 of 5). Heterogeneous expression of HBA71 immunostaining was found in some embryonal rhabdomyosarcomas (3 of 14) and astrocytomas (4 of 7), and in a few neuroendocrine tumors (4 of 26), carcinomas (3 of 94), and lymphomas (6 of 30). Because Ewing's sarcomas are consistently HBA71 positive, the authors searched for antigen-positive normal cells that may represent precursors for these tumors; however, no obvious candidate for the elusive cell of origin for Ewing's sarcoma was identified in the normal fetal tissues tested. Their findings indicate that HBA71 is a highly restricted cell-surface antigen of Ewing's sarcomas and primitive neuroectodermal tumors, and immunohistochemistry employing this antibody may be of value in the differential diagnosis of selected small round cell tumors in childhood and adolescence.

12E7 Antigen↗

PubMed1989

A monoclonal antibody specifically reactive with Ewing's sarcoma.

We have developed a mouse monoclonal antibody 5C11 (IgG2a) against cell surface antigen of Ewing's sarcoma (ES). 5C11 specifically reacted with ESs but not with other small round cell tumours in childhood, i.e. neuroblastomas, primitive neuroectodermal tumours (PNETs), rhabdomyosarcomas and malignant lymphomas. 5C11 did not react with any other tumours in children except for hepatoblastomas. No reactivity has been identified in normal tissues with the exception of fetal hepatocytes. Immunoelectron microscopically, 5C11 reactive antigen was located on cell membrane of ES cells. Biochemically, 5C11 immunoprecipitated a cell surface protein having molecular weight of 81,000 Da. 5C11 is the first antibody which can clearly distinguish ES from neurogenic tumours, especially from PNETs which were recently reported to have common features to ESs regarding chromosal abnormality and proto-oncogene expression but show evident differentiation into neurogenic direction. The results strongly indicate the usefulness of 5C11 not only for diagnostic purpose when no specific marker is available but also for studying the histogenesis of ES. In addition, no reactivity in normal tissue implies its potential application as a therapeutic reagent when the management of ES patients is still a great problem in clinical field.

Adolescent↗

PubMed2001

Spindle-cell B-cell lymphoma presenting in the skin.

Lymphomas with predominant spindle-cell morphology have only been previously reported in small numbers; such lesions are potentially mistaken for sarcoma or other spindle-cell tumours. We describe a 73-year-old woman who presented with a tumour on the scalp of a few months' duration. Biopsy showed a B-cell lymphoma of follicle centre cell origin with a sarcomatoid appearance and a CD20+, CD10+, CD21+, bcl-6+ immunophenotype. This case highlights the very rare occurrence of spindle-cell B-cell lymphoma presenting in the skin. An awareness of this phenomenon is essential to avoid confusion with a variety of other cutaneous spindle-cell neoplasms and to enable a correct diagnosis to be made, thereby ensuring that appropriate treatment is initiated.

Aged↗

PubMed1987

Malignant bone tumors in children: Ewing's sarcoma.

Ewing's sarcoma is a rare, malignant, small, round-cell tumor of bone. Most tumors arise in the diaphyses of long bones, but any bone can be involved. Chemotherapy is essential for long-term survival. Multiagent protocols are superior to single-agent therapies, but the ideal regimen has not been established. Both radiation therapy and surgery have been employed for local control of primary tumors. Control with either modality is significantly better when used in conjunction with effective chemotherapy. Patients who present with metastatic disease or pelvic primaries have a worse prognosis. Autologous bone marrow transplantation may improve disease-free survival for this group.

Adolescent↗

PubMed1980

Large cell lymphocytoma.

We studied nine cases of cutaneous lymphoproliferative disease that shared a distinct histologic pattern. The lesions consisted of red-to-violaceous, single or clustered, deep papules or nodules. They proved to be benign on long-term evaluation. Nodular infiltrates of large, pleomorphic lymphocytes, sometimes appearing to be histiocytic and often associated with frequent mitoses, led to the diagnosis of lymphoma or reticulum cell sarcoma in seven of the cases. Sharply marginated, dense clusters of small lymphocytes surrounded or infiltrated the large cell component, a juxtaposition that characterizes large cell lymphocytoma. This pattern was not found in patients with progressive malignant lymphoma. At times, multiple biopsies must be done to establish the diagnosis. All patients are alive, and none has malignant lymphoma. The follow-up periods for six of the patients have been five years or longer.

Adult↗

PubMed1988

Effect of the mixed bacterial vaccine on the immune response of patients with non-small cell lung cancer and refractory malignancies.

Since 1984, 13 patients were entered into our study and 12 patients have completed one or more cycles of treatment with mixed bacterial vaccine (MBV), a natural biologic response modifier derived from Streptococcus pyogenes and Serratia marcescens. Eight patients with refractory malignancy were treated with MBV only (0.1 ml intravenously [IV]) twice weekly for 3-16 weeks (colorectal cancer, pancreatic cancer, chronic lymphatic leukemia, hepatoma [two patients], sarcoma [three patients]). Four patients with advanced non-small cell lung cancer were treated with MBV in combination with low-dose cyclophosphamide, day 1; cisplatin, day 15; and MBV, 0.1 ml IV, days 5, 7, and 9. Two patients in this study received cyclophosphamide and cisplatin alone. The cycle was repeated every 28 days. Plasma interferon levels, interleukin-2 production by peripheral lymphocytes, and lymphocyte subpopulations were monitored. Interferon levels and interleukin-2 production showed increased or sustained values in general. In some patients, B-cells and helper T-cell populations increased, whereas T-suppressor cell numbers declined. With one exception, side effects were mild and consisted of fever greater than 37.8 degrees C (nine of 13), chills (11 of 13), increased respiratory rate (nine of 13), minor changes in blood pressure (seven of 13), and nausea (three of 13). One patient with non-small cell lung cancer had a partial response. Two patients with non-small cell lung cancer and one patient with refractory malignancy had stable disease and performance status at the end of 8 weeks of treatment; one patient with refractory malignancy was stable at the end of 4 weeks of treatment. In this pilot study, cancer patients treated with MBV showed objective evidence of immune stimulation with acceptable toxicity.

Adult↗

PubMed1996

The EWS-ATF-1 gene involved in malignant melanoma of soft parts with t(12;22) chromosome translocation, encodes a constitutive transcriptional activator.

Molecular characterization of malignant melanoma of soft parts or soft tissue clear cell sarcoma which shares t(12;22) chromosome translocation revealed fusion of EWS with a transcriptional factor gene ATF-1. The EWS gene, which encodes an RNA binding protein, was also shown to be involved in Ewing sarcoma, related primitive neuroectodermal tumors and desmoplastic small round cell tumors. In order to understand the functional role of EWS-ATF-1 chimeric protein in human solid tumors, we have cloned the aberrant human ATF-1 (EWS-ATF-1) cDNA and studied its DNA binding, transcriptional activation properties and compared with normal ATF-1 protein. Our results demonstrate that EWS-ATF-1 binds weakly to DNA in vitro but functions as an efficient constitutive transcriptional activator unlike the normal ATF-1 which needs to be induced with cAMP. Deletion analysis revealed that EWS-fusion domain functions as a regulatory domain for the transcriptional activation properties of EWS-ATF-1 chimeric protein. Deletion of leucine zipper domain results in a loss of transcriptional activation of EWS-ATF-1 chimeric protein suggesting that protein-protein interaction play a role in the transcriptional activation properties of EWS-ATF-1. We demonstrate that EWS-fusion domain negatively regulates the DNA binding activity of EWS-ATF-1 chimeric protein. Therefore replacement of part of the amino-terminal kinase regulatory domain of ATF-1 protein with EWS regulatory domain results in an altered DNA binding, protein-protein interactions and transcriptional activation properties of EWS-ATF-1 causing deregulated gene expression which may be responsible for the genesis of t(12;22) chromosome translocation-bearing human solid tumors. Targeting the transcriptional cofactors (CBP, etc) by EWS-fusion proteins could be one of the mechanisms of activation of EWS-fusion proteins in human neoplasia.

Activating Transcription Factor 1↗

PubMed1999

Neurotensin receptors in human neoplasms: high incidence in Ewing's sarcomas.

Receptors for regulatory peptides, such as somatostatin or vasoactive intestinal peptide (VIP), expressed at high density by neoplastic cells, can be instrumental for tumor diagnosis and therapy. Little is known about the expression of neurotensin receptors in human tumors. In the present study, 464 human neoplasms of various types were investigated for their neurotensin receptor content by in vitro receptor autoradiography on tissue sections using 125I-[Tyr3]-neurotensin as radioligand. Neurotensin receptors were identified and localized in tumor cells of 11/17 Ewing's sarcomas, 21/40 meningiomas, 10/23 astrocytomas, 5/13 medulloblastomas, 7/24 medullary thyroid cancers and 2/8 small cell lung cancers. They were rarely found in non-small cell lung cancers and breast carcinomas; they were absent in prostate, ovarian, renal cell and hepatocellular carcinomas, neuroendocrine gut tumors, pituitary adenomas, schwannomas, neuroblastomas and lymphomas. When present, the receptors bound with nanomolar affinity neurotensin and acetyl-neurotensin-(8-13), with lower affinity neuromedin N, diethylenetriamine penta-acetic acidneurotensin-(8-13) and SR 48692, but not neurotensin-(1-11). They were all of the NT1 type, without high affinity for levocabastine. Further, in 2 receptor-positive Ewing's sarcomas, neurotensin mRNA was detected by in situ hybridization techniques. Since neurotensin is known to stimulate cell proliferation, the presence of neurotensin receptors in human neoplasia may be of biological relevance, possibly as an integrative part of an autocrine feedback mechanism of tumor growth stimulation.

Animals↗

PubMed1990

[Effects of the method and regimen of administration on the carcinogenic effect of N-methyl-N'-nitro-N-nitrosoguanidine in rats].

Peculiarities of carcinogenic effect of N-methyl-N'-nitro-N-nitrosoguanidine administered through a stomach tube on rats were studied in 30 rats given 1-2 ml MNNG dissolved in distilled water (5 mg/ml) through a gastric tube for 2-3 days. The procedure is repeated every 4-10 days. This intermittent carcinogen administration continued until week 20; the animals were killed on week 25. All effective 26 (100%) rats had multiple papillomas and squamous cell carcinomas of the forestomach, 5 (19.2%) had adenomatous hyperplasias and adenocarcinomas of the glandular stomach, 7 (26.9%) had adenocarcinomas and sarcomas of the small intestine.

Adenocarcinoma↗

PubMed1994

Chemotherapy dose intensity facilitated by use of lenograstim--implications for quality of life and survival.

Increasingly more aggressive chemotherapeutic regimens are being used in pursuit of better tumour response and patient survival. Studies in advanced breast cancer, small cell lung cancer (SCLC), urothelial cancer and sarcoma indicate that recombinant human granulocyte colony stimulating factor (rHuG-CSF) (lenograstim) facilitates modest dose intensification (20-30%), but at high intensity doses, chemotherapy-related toxicity remains a problem. Survival outcome in SCLC patients is still uncertain although in one randomised trial lenograstim facilitated a 20% increase in chemotherapy dosage which resulted in a 25% increase in complete remissions. In studies of breast cancer, an initially improved response rate was not maintained. However, increased dose intensity of chemotherapeutic agents in aggressive lymphoma was possible using lenograstim. Studies to date with haematopoietic growth factors have not been designed to specifically address the issue of survival and/or quality of life, although they have inferred that this is possible through dose intensification. The use of haematopoietic growth factors will allow the design of such studies, but a modest chemotherapy dose increase may be insufficient to improve survival. The future for dose intensification in cancer therapy may lie in the use of lenograstim-primed peripheral blood progenitor cells (PBPC) transplants to enhance haematopoietic recovery after high-dose sequential or myeloablative chemotherapy.

Adjuvants, Immunologic↗

PubMed1998

Carbon-11-thymidine and FDG to measure therapy response.

UNLABELLED: This study was performed to determine if PET imaging with 11C-thymidine could measure tumor response to chemotherapy early after the initiation of treatment. Imaging of deoxyriboneucleic acid biosynthesis, quantitated with 11C-thymidine, was compared with measurements of tumor energetics, obtained by imaging with 18F-fluorodeoxyglucose (FDG). METHODS: We imaged four patients with small cell lung cancer and two with high-grade sarcoma both before and approximately 1 wk after the start of chemotherapy. Thymidine and FDG studies were done on the same day. Tumor uptake was quantified by standardized uptake values (SUVs) for both tracers by the metabolic rate of FDG and thymidine flux constant (K(TdR)) using regions of interest placed on the most active part of the tumor. RESULTS: In the four patients with clinical response to treatment, both thymidine and FDG uptake markedly declined 1 wk after therapy. Thymidine measurements of SUV and K(TdR) declined by 64% +/- 15% and 84% +/- 33%, respectively. FDG SUV and the metabolic rate of FDG declined by 51% +/- 9% and 63% +/- 23%, respectively. In the patient with metastatic small cell lung cancer who had disease progression, the thymidine SUV decreased by only 8% (FDG not done). In a patient with abdominal sarcoma and progressive disease, thymidine SUV was essentially unchanged (declined by 3%), whereas FDG SUV increased by 69%. CONCLUSION: Images show a decline in both cellular energetics and proliferative rate after successful chemotherapy. In the two patients with progressive disease, thymidine uptake was unchanged 1 wk after therapy. In our limited series, K(TdR) measurements showed a complete shutdown in tumor proliferation in patients in whom FDG showed a more limited decrease in glucose metabolism.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1992

Ewing's sarcoma and extracranial primitive neuroectodermal tumors.

Ewing's sarcoma and primitive neuroectodermal tumor of bone and soft tissue are rare tumors, considered with the small round blue cell tumors of childhood. Recent advances link Ewing's sarcoma and primitive neuroectodermal tumor, and support the concept that these tumors represent stages of differentiation in a neoplasm of neuroectodermal origin. Advances in chemotherapy have resulted in the survival of the majority of patients presenting with these tumors. This review briefly outlines the year's literature regarding the histogenesis, biology, and treatment of this tumor.

Adolescent↗

PubMed1995

The emerging molecular genetics of sarcoma translocations.

Many types of sarcomas are characterized by specific chromosomal translocations which are likely to be of etiologic significance. The recent elucidation of the molecular structure of the several of these translocations has revealed some striking similarities. Nearly all appear to result in the production of novel, tumor-specific chimeric transcription factors. Furthermore, six of the translocations, namely the t(11;22), t(21;22), and t(7;22) of Ewing's sarcoma, the t(12;22) of clear cell sarcoma, the t(12;16) of myxoid liposarcoma, and the t(11;22) of desmoplastic small round cell tumor, achieve this following a peculiar pattern, consisting in the fusion of a gene with an RNA-binding domain (EWS or TLS) with a transcription factor gene (FLI1, ERG, ETV1, ATF-1, CHOP, or WT1). The observation that the different translocation partners of the EWS gene are specifically associated with several distinct types of primitive sarcomas suggests a model in which the translocation partner supplying the DNA-binding domain confers the target specificity of the transcriptional activation mediated by these chimeric proteins, whereas the partner supplying the N-terminal domain and promoter region determines their transactivation potential and expression level. Further analysis of the normal functions and expression patterns of these genes should yield insights into the histogenesis of these different tumor types and into normal tissue development and differentiation. Clinically, our new understanding of the molecular structure of these translocations opens new avenues for molecular diagnosis and investigative therapeutics.

Base Sequence↗

PubMed2006

Peripheral primitive neuroectodermal tumor/Ewing's sarcoma of the craniospinal vault: case reports and review.

The peripheral primitive neuroectodermal tumor/Ewing's sarcoma family tumor (pPNET/ESFT) group includes small round cell tumors of the bone, soft tissue, and nerve with morphological attributes of the germinal neuroepithelium. Peripheral PNETs/ESFTs also occur within the craniospinal vault, a region including the central nervous system, the meninges, and the cranial and spinal nerve roots. Gene rearrangements between the EWS gene on chromosome 22q12 and members of the ETS gene family are common in and specific to pPNETs/ESFTs. Another defining characteristic of pPNETs/ESFTs is their membranous expression of the MIC2 gene product. We describe 2 cases of pPNETs within the craniospinal vault. An intradural tumor arising from the nerve roots of the cauda equina was discovered in a 32-year-old man presenting with radiculopathic back pain and lower-extremity weakness. An intracranial pPNET that mimicked a meningioma was found in a 21-year-old man presenting with headache and visual disturbances. MIC2 gene product expression and EWS/ETS gene rearrangement were detected in both case patients. The literature with regard to pPNETs/ESFTs arising within the craniospinal vault is reviewed.

12E7 Antigen↗