SEARCH · PubMed Health

Results for “Sarcoma, Small Cell”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 721 records · Page 40Linked to original sources

PubMed1990

Ifosfamide and mesna in combination with other cytostatic drugs in the treatment of patients with advanced cancer.

Fifty patients with histologically confirmed advanced malignancies were treated with ifosfamide and mesna plus other cytostatics. The other cytostatic drugs added to the treatment regimen were cisplatin (36 pts), etoposide (31 pts) and doxorubicin (20 pts). Among previously untreated patients objective response was documented in 12 of 19 pts with ovarian cancer, 9 of 14 with small cell cancer of the lung and in all 3 pts with Ewing's sarcoma. Among patients with disease refractory to prior cytostatic treatment, objective response was documented in 8 of 9 with testicular cancer, 1 of 3 with high grade lymphoma and 0 of 2 with osteosarcoma. Side-effects due to the combination of ifosfamide plus mesna and other cytostatics were acceptable. No life-threatening or lethal toxicities occurred. Most commonly encountered toxicities were leukopenia (64%), nausea and vomiting (84%), alopecia (63%), CNS toxicity (30%) and renal toxicity (12%).

Adolescent↗

PubMed1985

Differential transformation of C3H10T1/2 cells by v-mos: sequential expression of transformation parameters.

Extremely small quantities of the product of the transforming gene v-mos of Moloney murine sarcoma virus are able to efficiently transform cells. Recent data indicate the existence of a threshold level for v-mos transformation of NIH3T3 cells. Using mouse mammary tumor virus long terminal repeat sequences or hybrid promoters consisting of mouse mammary tumor virus and Moloney murine sarcoma virus long terminal repeat elements to express v-mos in C3H10T1/2 cells, we established cell lines representing different stages of morphological transformation in vitro. The threshold level for v-mos transformation was considerably lower than that for NIH3T3 cells, because no treatment with dexamethasone or primary selection other than transformation was necessary during standard transfection procedures. Using the cell lines mentioned we established an association of the level of v-mos expression with the transformation parameters examined, but not with p53 levels. Furthermore, the characterization of the different promoters showed (i) that the distal binding site confers hormone responsiveness to Moloney murine sarcoma virus promoter elements and (ii) that artifactual transcription initiation sites can be detected in mouse mammary tumor virus-Moloney murine sarcoma virus hybrid promoters which are, however, not regulated by the hormone.

Animals↗

PubMed2004

[Experience and discussion on treatment of high-grade soft tissue sarcomas of crura in youth].

OBJECTIVE: To summarize the experience and investigate better method on treatment of high-grade soft tissue sarcomas of crura in youth. METHODS: Six young cases suffered from high-grade soft tissue sarcomas of crura were treated by interpolatory chemotherapy using vindesine 4mg or vincristine 2 mg combined with cisplatin 40 - 60 mg and 4'-Epidoxorubicin (4'-epi-ADM) 50 - 80 mg. There were 3 males and 3 females, and their ages rang from 12 to 23. The primary tumor locations were: calf in 4, thigh in 1, popliteal fossa in 1. The pathological type of these 6 cases were: 1 small round cell tumor from soft tissue, one rabdomyosarcoma, one alveoliar soft part sarcoma, one synovial sarcoma, one primitive neuroectodermal tumor and one without classification. All cases received interpolatory chemotherapy twice. Before their receiving interpolatory chemotherapy they had no tumor metastases. The maximum diameters of tumors rang from 4 - 15 cm. One patient's tumor disappeared after interpolatory chemotherapy and she received no further therapy. The other 5 cases received operations and one of them received additional pre-operational radiotherapy using Co(60) and pre-operational systemic chemotherapy using ifosfamide and dacarbazine. RESULTS: The tumors dwindled in size of 1 - 8 cm after interpolatory chemotherapy. All cases saved their crura and had normal functions. After interpolatory chemotherapy, all patients suffered from edema and ache of crura. One patient suffered from lymphatitis. All these patients were followed up for 6 - 20 months. All of them were alive and had pigmentations on their crura. A lung metastasis was found in 1 patient by CT 2 months after surgery, then he was in hospital and treated by chemotherapy. CONCLUSIONS: For treatment of high-grade soft tissue sarcomas of crura which are difficult to resect in youth, interpolatory chemotherapy is a good choice.

Adolescent↗

PubMed1994

MIC2 analysis in pediatric lymphomas and leukemias.

Monoclonal antibodies to the glycoprotein product of the MIC2 gene strongly and reliably stain primitive neuroectodermal tumors and Ewing's sarcomas, and are negative in neuroblastomas and most rhabdomyosarcomas. Therefore, these antibodies are helpful in the diagnosis of small round cell tumors of childhood (SRCT). Lymphomas also are in the differential diagnosis of SRCT, but few have been studied with respect to MIC2 protein expression. In the present study we used the 12E7 antibody to assess MIC2 expression in 82 pediatric non-Hodgkin's lymphomas. Forty lymphoblastic, 22 small noncleaved, and 20 large cell lymphomas were studied. Strong immunoreactivity was found in 37 of the 40 (93%) lymphoblastic lymphomas, whereas only one of the 22 (5%) small noncleaved lymphomas was 12E7 positive. Four of the 20 (20%) large cell lymphomas also were immunoreactive. Three 12E7+ lymphoblastic lymphomas were primary in bone and were of B-progenitor lineage; Ewing's sarcoma was included in the initial differential diagnosis of these cases. Evaluation of 125 pediatric acute lymphocytic leukemia (ALL) cases for MIC2 expression showed similar results, with all 36 T-cell ALLs showing strong expression, one of eight B-cell (Burkitt-like) ALLs showing 12E7 expression, and 62 of 81 B-progenitor ALLs showing 12E7 positivity. We conclude that among the SRCTs, MIC2 expression is not limited to Ewing's sarcoma and primitive neuroectodermal tumors, but also shows strong and reliable expression in lymphoblastic lymphomas and related leukemias. MIC2 analysis continues to be helpful in the diagnosis of SRCT, provided that a panel of antibodies is used. In addition, the possibility that MIC2 analysis may aid in the distinction of lymphoblastic lymphomas from small noncleaved lymphomas needs to be further addressed.

12E7 Antigen↗

PubMed2001

Wilms tumor and the WT1 gene.

Wilms tumor or nephroblastoma is a pediatric kidney cancer arising from pluripotent embryonic renal precursors. Multiple genetic loci have been linked to Wilms tumorigenesis; positional cloning strategies have led to the identification of the WT1 tumor suppressor gene at chromosome 11p13. WT1 encodes a zinc finger transcription factor that is inactivated in the germline of children with genetic predisposition to Wilms tumor and in a subset of sporadic cancers. When present in the germline, specific heterozygous dominant-negative mutations are associated with severe abnormalities of renal and sexual differentiation, pointing to the essential role of WT1 for normal genitourinary development. The role of this tumor suppressor in normal organ-specific differentiation is also supported by the highly restricted temporal and spatial expression of WT1 in glomerular precursors of the developing kidney and by the failure of kidney development in wt1-null mice. Of two major alternative splicing products encoded by WT1, the (-KTS) isoform appears to mediate transcriptional activation of genes implicated in cellular differentiation, possibly also repressing proliferation-associated genes. The (+KTS) isoform, whose DNA-binding domain is disrupted by the insertion of three amino acids, may be involved in some aspect of mRNA processing. In addition to its function in genitourinary development, a role for WT1 in hematopoiesis is suggested by its aberrant expression and/or mutation in a subset of acute human leukemias. WT1 is also expressed in mesothelial cells; a specific oncogenic chromosomal translocation fusing the N-terminal domain of the Ewing sarcoma gene EWS to the three C-terminal zinc fingers of WT1 underlies desmoplastic small round cell tumor, an abdominal tumor thought to arise from the peritoneal lining. Understanding the distinct functional properties of WT1 isoforms and tumor-associated variants will provide unique insight into the link between normal organ-specific differentiation and malignancy.

Acute Disease↗

PubMed2006

Valproate inhibition of histone deacetylase 2 affects differentiation and decreases proliferation of endometrial stromal sarcoma cells.

Covalent modifications of histone proteins, in particular deacetylation of lysine residues, are important for the regulation of gene transcription both in normal and malignant cells. These processes are controlled by histone acetyltransferases and histone deacetylases (HDAC) and have up to now not been described in solid mesenchymal tumors. The present study shows differences in the HDAC1 and HDAC2 expression in endometrial stromal sarcomas (ESS) and a cognate cell line (ESS-1) compared with nonneoplastic endometrial stroma. We show for the first time that HDAC2 expression is consistently increased in ESS. In contrast, HDAC1 expression is generally lower than HDAC2 both in nonneoplastic stroma and in ESS, suggesting that these two proteins, although closely related, are regulated in different ways. In vitro experiments with an ESS cell line showed that valproate, an inhibitor of the class I HDACs, led to significant HDAC2 decrease and to cell differentiation. HDAC2 inhibition in ESS-1 cells caused significant changes in the cell cycle by inhibiting G1-S transition and influencing expression of p21WAF1 and cyclin D1. Moreover, in ESS-1 cells, increased expression of the p21WAF1 was associated with reduction of HDAC2 expression after transfection with small interfering RNA directed against HDAC2. Our results suggest that HDAC2 might be considered as potential drug target in the therapy of ESS and that HDAC inhibitors should be further evaluated in clinical trials in ESS.

Cell Differentiation↗

PubMed2005

Epithelioid sarcoma with SYT-SSX1 fusion gene expression: molecular and cytogenetic analysis.

Epithelioid sarcoma is a rare malignant soft tissue tumor of unknown histogenesis, characterized by the epithelioid morphology of tumor cells and co-expression of epithelial and mesenchymal lineage proteins. A common epithelial and mesenchymal immunophenotype is also found in another soft tissue tumor, the biphasic subtype of synovial sarcoma. Furthermore, the presence of a breakpoint at 18q11 in epithelioid sarcoma, similar to that found in synovial sarcoma, has been reported. These facts would support the hypothesis that both tumors may share a common histogenetic background. However, expression of the SYT-SSX fusion genes has been investigated in epithelioid sarcoma with negative results. We report, to our knowledge, the first epithelioid sarcoma in which the presence of SYT-SSX1 fusion gene has been detected by means of conventional RT-PCR analysis and sequencing, real-time RT-PCR and FISH. The SYT-SSX1 gene fusion was identified in a small proportion of tumoral cells. This finding supports a likely common histogenetic background for both epithelioid sarcoma and synovial sarcoma.

Adolescent↗

PubMed1995

Characterization of the genomic breakpoint and chimeric transcripts in the EWS-WT1 gene fusion of desmoplastic small round cell tumor.

Desmoplastic small round cell tumor is a recently recognized distinctive tumor shown to be associated with a recurrent translocation, t(11;22)(p13;q12), and rearrangement of the genes for Ewing sarcoma (EWS) and Wilms tumor (WT1). A genomic DNA fragment containing the EWS-WT1 gene fusion has been isolated from a desmoplastic small round cell tumor, and the breakpoint has been characterized. The breakpoints involve the intron between EWS exons 7 and 8 and the intron between WT1 exons 7 and 8. Chimeric transcripts corresponding to the fusion gene were detected in four of six cases studied. Analysis of these transcripts show an in-frame fusion of RNA encoding the amino-terminal domain of EWS to both alternatively spliced forms of the last three zinc fingers of the DNA-binding domain of WT1. Desmoplastic small round cell tumor represents the third tumor type associated with translocation of EWS and the first tumor associated with consistent translocation of WT1. The chimeric products are predicted to modulate transcription at WT1 target sites and contribute to development of this unique tumor.

Abdominal Neoplasms↗

PubMed1986

Comparative activity of ifosfamide and cyclophosphamide.

Antitumor activity (increase in lifespan and cure) was greater for ifosfamide (IFO) in several experimental tumors, some of which were primarily resistant to cyclophosphamide (CYC). IFO has been shown to be active in anthracycline-resistant and in adriamycin/cisplatin-resistant sublines of an Ehrlich ascites tumor, as well as in tumor cells primarily resistant to CYC. The few comparative controlled clinical trials available suggest superior single-agent activity of IFO compared with CYC in soft tissue sarcoma and ovarian cancer. Combination chemotherapy with IFO has been effective in second-line treatment of sarcomas, malignant lymphomas, lung cancer, and testicular cancer, most of them pretreated with or refractory to CYC. Although it is difficult to obtain clinical proof that there is no cross-resistance between IFO and CYC, IFO has been shown to be active in multirefractory malignant lymphomas, in small cell lung cancer not responding to adriamycin, vincristine, and etoposide, and in soft tissue and bone sarcomas. Testicular cancer and pancreatic cancer are some of the tumors in which IFO activity is currently being evaluated and in which CYC has so far failed to show sufficient clinical activity. More comparative controlled clinical trials are needed in ovarian cancer, breast cancer, malignant lymphomas, sarcomas and cervical cancer, in which IFO has already shown sufficient single-agent activity. Due to its lower level of cross-resistance with a variety of heterocyclic products, but also with other alkylating agents, in addition to its use in induction chemotherapy, IFO is an important second-line agent in many clinical situations.

Animals↗

PubMed1989

Pseudosarcomatous stromal reaction in primary and metastatic urothelial carcinoma. A source of diagnostic difficulty.

We report five urothelial carcinomas (one primary and four metastatic) with pseudosarcomatous stromal reaction. The exuberant stromal reaction led to a histologic misdiagnosis in three of the original small biopsy specimens. The differential diagnoses of primary spindle cell lesions of urinary tract include spindle cell carcinoma, carcinosarcoma, sarcoma, and benign pseudosarcomatous lesions. The distinction between those conditions and urothelial carcinomas with pseudosarcomatous stromal reaction is obviously of great clinical significance. In an initial small biopsy specimen, it may be difficult to make such a distinction. Immunostaining for cytokeratin and examination of more material should be performed. In our study of metastatic urothelial carcinomas with pseudosarcomatous stromal reaction, clinicopathologic correlations along with immunostaining for keratin proved to be useful. The stromal inductive capability of transplanted urothelium, which has been established in animal experiments, has occasionally also been reported in humans. The malignant urothelium in humans similarly appears to be capable of inducing stromal reaction.

Aged↗

PubMedDate not supplied

Primary intracranial peripheral primitive neuroectodermal tumor/Ewing's sarcoma presenting with acute intracerebral hemorrhage.

OBJECTIVE: To report two cases of intracerebral hemorrhage due to primary intracranial peripheral primitive neuroectodermal tumor (pPNET)/Ewing's sarcoma (ES) and review of related literatures. MATERIAL: Two cases of 17-year-old patients presented with acute increased intracranial pressure one of which also had left hemiparesis. METHOD: On neuroimaging studies, the first patient had an intraparenchymal hematoma with a size of 4 cm at the right fronto-parietal junction adjacent to tumor infiltrating the superior sagittal sinus. The second patient had a large left temporal tumor with intraventricular hemorrhage. Both patients underwent craniotomy with complete removal of tumor and hematoma. RESULTS: Pathological examination in both cases revealed numerous small round tumor cells with stippled chromatin pattern and scanty cytoplasm. Tumor cells strongly expressed CD99. Vimentin immunoreactivity was observed. The final diagnosis of pPNET/ES was rendered. There was no evidence of extracranial disease in both cases. Both patients were doing well without evidence of recurrent disease at 12 and 24-month follow-up respectively. CONCLUSIONS: Peripheral primitive neuroectodermal tumor (pPNET)/Ewing's sarcoma (ES) is a malignant small round cell tumor, commonly arising in soft tissue of the trunk and lower extremity. Those occurring in the intracranium are rare, and most patients present with progressively increased intracranial pressure and/or cranial nerve deficit. The occurrence of intracerebral hemorrhage due to primary intracranial pPNET/ES is exceedingly rare. The role of adjuvant therapy in this condition is yet to be investigated.

Acute Disease↗

PubMed1982

Selective hormonal activation of cyclic AMP-dependent protein kinase isoenzymes in normal and malignant osteoblasts.

The pattern of cyclic AMP-dependent protein kinase isoenzyme response to acute hormonal activation has been studied in cultured cells derived from rat osteogenic sarcoma and osteoblast-rich cells grown from newborn rat calvaria. Using multiple small anion exchange columns and a batch elution technique, a rapid method of separating the isoenzymes of cyclic AMP-dependent protein kinase was developed and the acute activation by parathyroid hormone and prostaglandin E2 of each isoenzyme was studied. Activation was rapid, being detectable at 5 s, maximal at 15-30 s, and persisting for up to 6 h. Both hormones showed a dose-dependent activation of each isoenzyme in both cell types, but the patterns of response differed. Parathyroid hormone predominantly stimulated isoenzyme I in the clonal osteogenic sarcoma cells but showed equivalent activation of each isoenzyme in calvarial cells. Prostaglandin E2 also predominantly stimulated isoenzyme I in the malignant cells, whereas in the calvarial strain there was a major effect on isoenzyme II with almost no stimulation of isoenzyme I. Half-maximal stimulation of cyclic AMP-dependent protein kinase in the malignant cell strain was achieved for both hormones at concentrations an order of magnitude lower than those in the normal strain. These studies demonstrate selective activation of cyclic AMP-dependent protein kinase isoenzymes by hormones. Furthermore, the nature of the response differs between the normal and the corresponding neoplastic cell types for the same hormone stimulus.

Animals↗

PubMed2003

Granulocytic sarcoma of the small intestine with CBFbeta/MYH11 fusion gene: report of an aleukaemic case and review of the literature.

Granulocytic sarcomas (GS) are rare extramedullary tumours composed of immature myeloid cells. Inversion of chromosome 16 [inv(16)] is a cytogenetic marker for M4Eo subtype of acute myeloid leukaemia (AML). The possibility of an association between the development of granulocytic sarcoma of the small intestine (GSSI) and the M4Eo subtype of AML was suggested in nine previous case reports. Here we report an aleukaemic case of GSSI with inv(16) and its molecular equivalent, the CBFbeta/MYH11 fusion gene, detected by reverse transcriptase-polymerase chain reaction (RT-PCR), that after treatment with conventional AML chemotherapy followed by autologous bone marrow transplantation, achieved complete haematological and molecular remission on bone marrow examination. After chemotherapy, a thickened ileum wall positive for CBFbeta/MYH11 on tumour mass samples was still observed on computed tomography (CT) studies, raising the question of residual GS representing a reservoir of malignant cells. This case demonstrates the critical need of multidisciplinary diagnosis and follow-up of this entity combining immunopathologic, cytogenetic and molecular studies, reinforcing the potentiality of risk-adapted therapy strategies, as it is increasingly claimed for patients with overt AML.

Adult↗

PubMed1981

Lumbar epidural Ewing sarcoma. Light and electron microscopic investigation.

The clinicopathological findings in a child with extraskeletal Ewing sarcoma are described. The patient complained of pain in the lower back and difficulty walking. An extraskeletal, epidural, friable tumor, 2-3 cm long was removed from the epidural space. It had no relationship with the bone structures. Light and electron microscopic examination of the tumor led to the diagnosis of Ewing sarcoma. The morphological aspects of this neoplasia and the problem of the differential diagnosis with other small cell tumors of the epidural space are discussed.

Adolescent↗

PubMed2006

The role of genetic testing in soft tissue sarcoma.

Soft tissue tumours represent a heterogeneous group of mesenchymal lesions and their classification continues to evolve as a result of incorporating advances in cytogenetic and molecular techniques. In the last decade traditional diagnostic approaches were supplemented with a significant number of reliable molecular diagnostic tools, detecting tumour type-specific genetic alterations. In addition, the successful application of some of these techniques to formalin-fixed paraffin-embedded tissue made it possible to subject a broader range of clinical material to molecular analysis. Thus, molecular genetics has already become an integral part of the work-up in some tumours, such as paediatric small blue round cell tumours, which demonstrate characteristic translocations. Several lines of evidence suggest that sarcomas can be divided into two major genetic groups: (i) sarcomas with specific genetic alterations and usually simple karyotypes, such as reciprocal chromosomal translocations (e.g. FUS-DDIT3 in myxoid liposarcoma) and specific oncogenic mutations (e.g. KIT mutation in gastrointestinal stromal tumours); and (i) sarcomas with non-specific genetic alterations and complex unbalanced karyotypes. Some of these genetic abnormalities, including chromosomal numerical changes, translocations, gene amplifications or large deletions can be apparent at the cytogenetic level (karyotyping, fluorescence in situ hybridization), while others, such as small deletions, insertions or point mutations, require molecular genetic techniques (polymerase chain reaction and sequence analysis). This review focuses on the applicability of genetic testing in the diagnosis and prognosis of soft tissue sarcomas, and gives a realistic appraisal of the ancillary role of molecular techniques, including its advantages and limitations.

Base Sequence↗

PubMed1986

Ultrastructural studies in the preoperative cytologic diagnosis of soft tissue tumors.

A consecutive series of 100 patients operated on for lesions that were assumed to be soft tissue tumors, all of whom had been the subject of fine-needle aspiration in the preoperative investigation, is described. A correlative study of smears and the light- and electron-microscopic findings of embedded fine-needle aspirates and the histopathology of the surgical specimens was performed. Eighty of the lesions were found to be genuine soft tissue tumors, of which 51 were sarcomas. The other 20 cases were either metastatic carcinoma, malignant melanoma, or malignant lymphoma. The embedding technique produced additional light-microscopic information about tissue structure and growth pattern, and electron-microscopic information about tissue and cell differentiation of importance to the diagnosis. In the case of certain types of soft tissue tumor, such as lipoma, neurilemmoma, liposarcoma, and malignant fibrous histiocytoma, and for well-differentiated metastatic carcinoma and pigmented malignant melanoma, the diagnosis may be strongly suggested by the appearance of the smears; the embedding technique serves to further support the diagnosis. In the case of small round-cell malignancies, the ultrastructural examination proved to be of special value, ie, in the distinction of rhabdomyosarcoma, poorly differentiated metastatic small cell carcinoma and malignant melanoma, and occasional cases of malignant lymphoma. Spindle cell sarcomas, such as leiomyosarcoma when well differentiated, biphasic synovial sarcoma when it includes glandular structures, and malignant hemangiopericytoma, could be recognized ultrastructurally, although electron-microscopy generally failed to reach a definite diagnosis as to the subtype in most cases of poorly differentiated spindle-cell sarcoma.

Adult↗

PubMed1982

B cell lymphoproliferation in spontaneously diabetic BB Wistar rats.

Ninety-six spontaneously diabetic BB Wistar rats were maintained for their natural life span and, at death, were autopsied together with 86 age-and sex-matched non-diabetic BB control rats. A 15% incidence of abdominal B cell lymphoproliferative lesions was documented in the diabetic rats compared with 1% incidence in the non-diabetic rats (p less than 0.005). The B cell lymphoproliferative process included minute mesenteric and omental aggregates of plasma cells and small lymphocytes (one rat), atypical partially fibrotic lymphoproliferative mesenteric nodules (three rats), and malignant lymphoma with features of immunoblastic sarcoma (eight rats) or plasma cell lymphoma (two rats). Cytoplasmic immunoglobulin was demonstrated in two of the four lymphomas examined by the peroxidase-antiperoxidase technique, thus confirming their B cell derivation. The striking incidence of B cell lymphoproliferation in this diabetic population is additional evidence of altered immunity in this animal model of insulin-dependent diabetes mellitus.

Animals↗

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↗