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At least 19 recordsLinked to original sources
PubMedDate not supplied

Nondifferentiated-type small cell sarcoma of kidney in a young woman.

A case of rapidly fatal small cell sarcoma of kidney in a young woman is described. Histologic, immunohistochemical, and ultrastructural studies demonstrated some features not reported previously in the literature, such as the arrangement of vimentin intermediate filaments in small irregular masses and the presence of various types of cytoplasmic projections. These findings, together with the observation of rare neoplastic elements with immunophenotypic and subcellular aspects of histiocytic and myofibroblastic differentiation, suggest that this tumor should be included in a group of small cell sarcomas of kidney lacking features of differentiation and should be considered a new variant occurring in adulthood.

Adult↗
PubMed1998

Molecular features in a biphenotypic small cell sarcoma with neuroectodermal and muscle differentiation.

We report a case of a 13-year-old girl with soft tissue sarcoma of the hand, which showed muscle and neuroectodermal immunophenotypes. Molecular studies were performed on RNA collected from fine-needle aspiration (FNA) cytology and peripheral blood samples by nested reverse transcriptase-polymerase chain reaction (RT-PCR) and Southern blot analysis. This biphenotypic tumor showed simultaneous expression of EWS-FLI1 and PAX3-FKHR transcripts, specific of Ewing family tumors and alveolar rhabdomyosarcoma, respectively. Although childhood sarcomas with simultaneous muscle and neural differentiation have been described to have EWS-FLI1 transcripts, there are no reports of tumors with both transcripts. Cytological specimens are a good source of RNA for molecular studies.

Adolescent↗
PubMed1997

[Small-cell sarcoma of the esophagus as a fourth malignancy. Its palliative therapy with argon gas coagulation].

HISTORY: A prostatic carcinoma, an early gastric carcinoma and a colon carcinoma had occurred over 15 years in a now 82-year-old patient. He was now admitted because of severe dysphagia. INVESTIGATIONS: Gastroscopy revealed an exophytic tumour of the oesophagus, histologically identified as a small-cell sarcoma. It had caused a 12 cm long severe eccentric stenosis of the oesophagus. Tissue from the previous three tumours were examined immunohistochemically for p-53 gene mutation, but only the oesophageal sarcoma gave positive results. TREATMENT AND COURSE: After part of exophytic tumour had been ablated by argon gas coagulation a prosthetic tube was implanted. Bleeding from erosion of a large metastasis in the gastric fundus was successfully treated by argon gas coagulation 4 months after the previous discharge, but the patient died of the malignancy 1/1 and half months later. CONCLUSIONS: The consecutive occurrence of four different malignant tumours is rare even in advanced age. In this case the malignancies were presumably unrelated and it demonstrates the possibility of removing an eccentric tumour stenosis by argon gas coagulation before implanting a prosthesis.

Aged↗
PubMed2005

Type II collagen as specific marker for mesenchymal chondrosarcomas compared to other small cell sarcomas of the skeleton.

Mesenchymal chondrosarcoma is a rare, usually highly malignant chondrogenic neoplasm. The diagnosis of mesenchymal chondrosarcoma can be challenging, it nonetheless has important therapeutic and diagnostic implications. Thus, biopsies of mesenchymal chondrosarcomas without conspicuous cartilaginous differentiation cannot be safely distinguished from other small cell mesenchymal neoplasms such as Ewing's sarcoma and peripheral neuroendrocrine tumors, synovial sarcomas and hemangiopericytomas, because all of these neoplasms might show overlapping histological features, and so far, there have been no safe immunohistochemical markers available in order to differentiate these neoplasms. In our study on a large series of mesenchymal chondrosarcomas (n=30) and other small cell sarcomas (Ewing's sarcomas (n=12), synovial sarcomas (n=6), hemangiopericytomas (n=5), small cell osteosarcomas (n=3), and desmoplastic small round cell tumors (n=1)), we could establish the presence of type II collagen in the extracellular tumor matrix of the small cell areas of mesenchymal chondrosarcomas as a specific and sensitive marker to identify mesenchymal chondrosarcomas and to exclude other small cell neoplasms (except chondroblastic areas in small cell osteosarcomas). In contrast, the S-100 protein was less sensitive and vimentin and total collagen content unspecific for discriminating these neoplasms. Thus, the presence of type II collagen in the extracellular tumor matrix significantly facilitates the diagnosis of mesenchymal chondrosarcomas in the absence of histologically visible chondroid matrix formation.

Biomarkers, Tumor↗
PubMedDate not supplied

[Immunohistochemical study of small cell sarcomas and factors(nm23, CD44) predicting metastatic spreading in children].

An immunohistochemical study was made of 43 tumors from children, which were classified as small blue round cell tumors (13 neuroblastomas, 13 rhabdomyosarcomas, 14 Ewing sarcomas/PNET, 2 undifferentiated sarcoma, 1 rhabdoid tumor). The use of a wide panel of antibodies confirmed (in 74% of the cases) and corrected (in 26%) the diagnosis of a diversity of small cell sarcomas established by routine methods. In neuroblastoma, the occurrence of distal metastases was associated with the loss of nm 23 protein. Increase in CD44 expression was more frequently observed in neuroblastomas with favorable prognostic signs and embryonic rhabdosarcomas that are not accompanied by metastases. This needs further study.

Adolescent↗
PubMed1993

Differential expression of leukocyte differentiation antigens in small round blue cell sarcomas.

BACKGROUND: Small round blue cell sarcomas (SRBCS) comprise a group of cytomorphologically poorly differentiated neoplasms that are characterized by different histogenesis and biologic behavior. METHODS: Twenty-seven well-characterized SRBCS were examined immunohistochemically to detect the expression of a panel of leukocyte differentiation (CD) antigens and class I (HLA-A,B,C) and class II (HLA-DR) major histocompatibility complex antigen. RESULTS: Although various cell surface antigens were detectable in SRBCS, the pan-leukocytic-histiocytic CD53 antigen was absent in the neoplastic population of all tumors studied; this finding allowed the authors to discriminate these lesions from lymphomas and leukemias. Some antigens had a differential pattern of expression in the SRBCS group, in particular in the undifferentiated tumor cell populations. In most instances, neuroblastomas (NB) and ganglioneuroblastomas (GNB) were CD9+/CD24+/CD56+ but CD40-/HLA-A,B,C-. Rhabdomyosarcomas (RMS) were CD56+ in all specimens and CD9+ in many samples; generally, they showed CD24-/CD40-/HLA-A,B,C-. Ewing sarcomas (ES) and peripheral primitive neuroectodermal tumors (pPNET) were HLA-A,B,C+/CD40+ but CD9-/CD24-/CD56- in most instances. Thus, with few exceptions, the expression of CD9 and CD56 and the simultaneous absence of HLA-A,B,C and CD40 differentiated GNB, NB, and RMS from ES and pPNET. GNB, NB, and RMS differed in regard to their CD24 expression. CONCLUSIONS: These data show that various types of SRBCS have different patterns of cell surface antigens. Therefore, these antigens are considered to be helpful in the immunophenotypic subclassification of SRBCS: The immunophenotypic similarities between ES and pPNET, however, might be an additional argument for a close relationship between these two lesions.

Antigens, CD↗
PubMed1989

[Angiotensin II-induced hypertension chemotherapy of bone and soft-tissue sarcomas].

We treated 14 patients with high grade sarcomas by angiotensin II-induced hypertension chemotherapy. The chemotherapy protocol described by Rosen was selected according to histological classification of sarcomas (small cell sarcoma, spindle cell sarcoma, pleomorphic sarcoma). The level of angiotensin-induced hypertension was one and half times as high as blood pressure at rest. Induced hypertension was maintained for 30-60 minutes. In three cases of 5 primary osteosarcomas, induced hypertension resulted in the increase of tumor stain and/or vascularity angiographically, and chemotherapeutic effects were CR or PR. The six cases with soft-tissue sarcomas were 2 cases each of CR, PR, and NC. The decrease of relative tumor blood flow under the condition of angiotensin II-induced hypertension was detected in 5 cases of 6 soft-tissue sarcomas by 133Xe clearance method. In the case of rhabdomyosarcoma, the decrease of tumor stain and vascularity by induced hypertension was observed on angiogram. As the side effects accompanying induced hypertension, nausea and chest oppression were noted in 2 cases, respectively. In this study it was suggested that angiotensin II-induced hypertension chemotherapy was effective for osteosarcoma, but that it might be ineffective for soft-tissue sarcomas.

Adolescent↗
PubMed1994

EWS/FLI-1 rearrangement in small round cell sarcomas of bone and soft tissue detected by reverse transcriptase polymerase chain reaction amplification.

Recent cloning of the t(11;22) region has led to the detection of a number of sequences involved in the breakpoints by substituting a sequence which encodes a putative RNA binding domain for that of the DNA binding domain of the human homologue of murine FLI-1. Several tumours display consistent translocation at t(11;22) (q24;q12), a finding that suggests these fusion transcripts could be expressed and detected by reverse transcriptase polymerase chain reaction amplification. To date, only a small number of Ewing's sarcomas (Es) and peripheral neuroectodermal tumours (pPNET) of bone have been tested with this novel molecular biology approach. In this study, we confirmed the presence of the three putative chimaeric transcripts on 7 cases of Es and pPNET sarcomas of bone and soft tissue, providing 100% positivity for the tested tumours. For comparative purposes, a number of other neuroectodermal tumours were analysed with negative results: esthesioneuroblastoma, retinoblastoma, Schwannoma. A primitive soft tissue sarcoma (ectomesenchymoma) with a 22 chromosome rearrangement did not express any transcript, nor did a number of non-neuroectodermal small round cell sarcomas of soft tissue (rhabdomyosarcomas) and bone (microcellular osteosarcoma), conventional bone sarcomas, leiomyosarcomas, malignant fibrous histiocytomas and synovial sarcomas. These results reinforce the value of molecular biology techniques for the correct assessment of histology difficult evaluable neoplasms, such as the group of small round cell tumours within the Es family.

Animals↗
PubMedDate not supplied

Small cell osteogenic sarcoma of the ribs: cytological, immunohistochemical, and ultrastructural study with literature review.

Small cell osteosarcoma (OS) is a rare variant of OS that is composed of small cells resembling those of Ewing's sarcoma (ES) with recognizable osteoid. This type of tumor often creates difficulty in making a diagnosis when tissue samples do not include osteoid. The frequent sites are long bones and until now there have been no reported cases arising in the ribs. A case is reported here of small cell OS occurring in the ribs of a 37-year-old female with its aspiration cytologic and electron microscopic characteristics. In the cytologic smear, the small round neoplastic cells were individually scattered or arranged in small nests. The nuclei were hyperchromatic and oval with no visible nucleoli. Ultrastructurally, the nuclei had a round or oval euchromatic chromatin pattern and occasional nucleoli. The scanty cytoplasm contained a small quantity of organelles including either tubular or dilated cisternae of RER, a few mitochondria, and free or polyribosomes. Other organelles were absent. Although the electron microscope sample of this case did not include bone mineral (hydroxy apatite), the electron microscopic features of the tumor cells were unique and useful for exclusion of other small round cell neoplasms.

Adult↗
PubMedDate not supplied

Differentiating small round cell sarcomas of the soft parts by an innovative immunogold labeling method: an ultrastructural study.

A new immunoelectron microscopy procedure was developed by remaking the fixed-frozen tissue specimens into LR White resin blocks suitable for postembedding colloidal gold immunolabeling, and used to examine 16 cases of small round cell soft tissue sarcomas. In rhabdomyosarcoma, ultrastructural double-immunogold staining demonstrated a coexpression of muscle specific actin and desmin in the same tumor cell. In both Ewing's sarcoma and peripheral neuroepithelioma, the heterogeneous expression of MIC2 gene product (p30/32MIC2) in each tumor cell was demonstrated as well. In peripheral neuroepithelioma, the colloidal gold immunolabeling for neurofilament demonstrated the intermediate filaments surrounding microtubules. The procedure for ultrastructural colloidal gold immunolabeling using fixed-frozen tissue is thus considered to be useful not only for tumor diagnosis, but also for investigating various subcellular structures.

Adolescent↗
PubMed1989

Establishment and characterization of a small round cell sarcoma cell line, SCCH-196, with t(11;22)(q24;q12).

A cell line designated SCCH-196 was established from an extraskeletal small round cell sarcoma developed in a 16-year-old Japanese girl. The cells grew as a monolayer, and have been continuously propagated by serial subcultures during the past 26 months. Cells from the primary tumor and those from the SCCH-196 cell line at passage 10 both showed the same karyotype, 51,XX, +8, +20, +21, t(11;22)(q24;q12), +i(1q), +i(1q). Histologically the primary tumor was difficult to classify as either Ewing's sarcoma (ES) or peripheral neuroepithelioma (NE). Neuron-specific enolase-positive cells in the primary tumor and the occurrence in the upper extremity were in favor of NE, while positive reaction of SCCH-196 cells to an ES-specific monoclonal antibody 5C11 suggested a diagnosis of ES. The SCCH-196 cell line may be useful for basic studies on differentiation of neuroectodermal tumors, and for future cloning of still unidentified genes which may be located at the breakpoints of the 11;22 translocation.

Adolescent↗
PubMed1990

Acridine orange--RNA histofluorescence of sarcomas and small round cell tumors of childhood.

Forty-nine pediatric malignant neoplasms were stained with acridine orange (AO) fluorochrome to qualitatively evaluate cytoplasmic RNA content. The application of AO as a supplementary stain in surgical pathologic diagnosis is based on the premise that specific neoplastic cell types characteristically and consistently contain few or many cytoplasmic ribosomes. Primitive tumors such as Ewing's sarcoma and primitive neuroectodermal tumors showed negative or low-intensity AO-RNA cytoplasmic staining. Differentiated sarcomas such as rhabdomyosarcomas and lymphomas exhibited moderate to strong AO-RNA cytoplasmic fluorescence. Acridine orange--RNA staining provides an easy, convenient, and inexpensive adjunct in the histopathologic differential diagnosis of sarcomas. It is particularly useful for distinguishing Ewing's sarcomas from other small round cell sarcomas of childhood.

Acridine Orange↗
PubMed1991

Development of small-cell osteogenic sarcoma after ulnar ostectomy in a dog.

A 5-year-old male Saint Bernard was evaluated for vague forelimb lameness. Four and a half years earlier, the dog had undergone left ulnar ostectomy to correct a valgus deformity. Focal bone lysis was evident radiographically at the ostectomy site. Four months later, marked bony swelling was apparent and the dog was bearing partial weight. The dog was euthanatized, and at necropsy, a bone tumor with lung metastasis was found. Microscopically, features of the tumor were compatible with those of small-cell osteogenic sarcoma.

Animals↗
PubMed2000

A novel zinc finger gene is fused to EWS in small round cell tumor.

Ewing sarcoma family of tumors share recurrent translocations that fuse EWS from 22q12 to five different members of transcription factors namely FLI-1, ERG, ETV1, E1AF and FEV. Different classes of DNA binding proteins, ATF1, WT1 and CHOP are fused to EWS generating distinct tumor phenotypes: clear cell sarcoma, desmoplastic small round cell tumor, and myxoid liposarcoma, respectively. We have cloned a novel gene located at 22q12 fused to EWS by a submicroscopic inversion of 22q in a small round cell sarcoma showing a translocation (t(1;22)(p36.1;q12). The gene, designated ZSG (Zinc finger Sarcoma Gene), is a putative Cys2-His2 zinc finger protein which contains a POZ transcriptional repressor-like domain at the N-terminus. The rearrangement involves intron 8 of EWS and exon 1 of ZSG creating a chimeric sequence containing the transactivation domain of EWS fused to zinc finger domain of ZSG. This product lacks the transcriptional repressor domain at the N-terminus of ZSG. A rearrangement of the second ZSG allele was also found in tumor cells. This is the first example of an intra-chromosomal rearrangement of chromosome 22, undetectable by cytogenetics, activating EWS in soft tissue sarcoma.

Adolescent↗