Cases reported "Femoral Neoplasms"

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1/10. Malignant fibrous histiocytoma after allogeneic bone marrow transplantation.

    A 24-year-old woman with CML underwent allogeneic BMT in August 1995 from a one-antigen HLA mismatched brother. Conditioning included BuCy2 and CsA and MTX were used to prevent GVHD. In July 1997 she developed right leg pain, lytic bone lesions of distal femur and a solid mass of soft tissue. Histological diagnosis of malignant fibrous histiocytoma was made. Despite treatment with surgery and chemotherapy (doxorubicin and ifosfamide), the patient died 1 year later with local recurrence of the tumor and liver, lung and brain metastases. The CML was in CR.
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2/10. Transfer of in vitro cytotoxicity against osteogenic sarcoma cells.

    Evidence of lymphocyte cytotoxicity against osteosarcoma, suggesting a degree of cell-mediated immunity, was found in the mother of a patient with osteogenic sarcoma. The mother was found to be HL-A identical to the patient and therefore was an ideal donor for whole lymphocytes. lymphocytes were obtained from the mother by leukophoresis and were administered to the patient. Lymphocytotherapy transferred or re-established a delayed hypersensitivity response to mumps antigen and transferred the ability of killing osteogenic sarcoma cells in vitro. There was slight improvement in the patients' clinical condition coincident with the establishment of lymphocytoxicity in the patient. Loss of this capacity coincided with a rapid deterioration of the patient's condition.
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3/10. Human leukocyte antigen (HLA) phenotypes in siblings with osteosarcoma.

    osteosarcoma was detected in two siblings. Their human leukocyte antigen (HLA) phenotypes were completely identical, although they were different from those of osteosarcoma patients in previous reports. Despite an extensive search of family and past history, no significant background related to the induction of cancer could be found. These cases suggest that genetic similarity may influence the development of osteosarcoma. Ascertainment of the HLA phenotypes in siblings with osteosarcoma might be a useful strategy to facilitate the early diagnosis of this tumor.
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4/10. Pleomorphic ("dedifferentiated") chondrosarcoma. Report of a case initially examined by fine needle aspiration biopsy.

    Fine needle aspiration (FNA) biopsy of a predominantly radiolucent, destructive lesion of the right distal femoral metaphysis of a 69-year-old man produced smears containing spindle-shaped cells with cytologic features consistent with a malignant fibrous histiocytoma. This initial diagnosis was supported by immunoperoxidase staining, which was strongly positive for vimentin and alpha-1-antichymotrypsin, focally positive for S-100 protein and negative for desmin, muscle-specific actin, keratin, carcinoembryonic antigen and epithelial membrane antigen. Subsequent surgical resection revealed a lesion with a predominance of malignant fibrous histiocytoma-type regions; however, focal microscopic areas contained a low-to-medium-grade cartilaginous component. The final diagnosis rendered was thus pleomorphic or so-called "dedifferentiated" chondrosarcoma. This rare lesion should be included in the differential diagnosis of malignant spindle-cell lesions of bone assessed by FNA biopsy.
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5/10. Characteristics of a cell line established from a patient with multiple osteosarcoma, appearing 13 years after treatment for bilateral retinoblastoma.

    An osteosarcoma cell line, OHS, was established from a patient with multiple skeletal manifestations of osteosarcoma, developing after bilateral retinoblastoma. The tumor cells expressed sarcoma-associated antigens and showed rapid growth in monolayers and as multicellular spheroids. They formed distinct colonies in soft agar, and subcutaneous tumors in nude mice. Morphological studies indicated that OHS cells had retained important characteristics of the cells of origin. No deletion of the retinoblastoma genes on chromosome 13q14 could be demonstrated with the banding techniques used. However, cytogenetic studies revealed double minute chromosomes, as evidence of gene amplification, as well as translocations involving chromosomes 1,6,11 and 13. The OHS line can be used to study the genetic basis of tumor initiation and growth, and to elucidate factors predisposing for second primary cancers in retinoblastoma patients.
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6/10. The characterization of alpha-subunit of glycoprotein hormone produced by undifferentiated carcinoma.

    The glycoprotein hormone alpha-subunit was extracted and purified from the urine of a patient with undifferentiated carcinoma producing isolated alpha-subunit. Its final specific immunoactivity was 0.92 (mg alpha-subunit/mg protein). The alpha-subunit exhibited virtually identical immunoantigenicity to hCG-alpha antiserum with standard hCG-alpha. The molecular weight of the alpha-subunit determined by gel chromatography on Sephadex G-100 was greater than that of standard hCG-alpha dissociated by urea in vitro. By SDS disc electrophoresis, however, the alpha-subunit moved faster than hCG-alpha separated by mercaptoethanol reduction. The amino acid composition of the alpha-subunit was quite similar to that of standard hCG-alpha. In the isoelectric focusing, the major components of the alpha-subunit from undifferentiated carcinoma and the alpha-subunit from urine of normal pregnant women (third trimester) were distributed over the range from pH 3.5 to 6.0, while standard hCG-alpha was distributed in the fractions ranging from pH 6.0 to 8.0. The result of a combination study in vitro indicated that both alpha-subunits from undifferentiated carcinoma and from urine of normal pregnant women did not actively combine with hCG-beta. These results suggest that the alpha-subunit secreted by undifferentiated carcinoma is virtually identical with standard hCG-alpha as the protein moiety but differs in regard to carbohydrate moiety, and also suggest that the excess of alpha-subunit, which is not associated with beta-subunit, may have undergone some intracellular modification, and consequently, the electric charge of the freely secreted alpha-subunit changes and it no longer has the ability to combine with the beta-subunit.
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7/10. Ewing's sarcoma in female siblings. A clinical report and review of the literature.

    The third reported case of Ewing's sarcoma in siblings is presented. All reported siblings to date have been female. Because of the most unusual occurrence, clues for environmental and genetic factors in the origin of the tumors were sought. Human leukocyte antigen (HL-A) genotyping, routine chromosomal, and prophase chromosomal studies were obtained. Despite extensive study, the existence of an infectious agent, exposure to a carcinogen, or genetic abnormality could not be defined.
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8/10. CD3- CD56 non-Hodgkin's lymphomas with an aggressive behavior related to multidrug resistance.

    CD56 expression has been reported previously in some non-Hodgkin's lymphoma (NHL) characterization. They principally involve the nasopharynx, are related to Epstein-Barr virus (EBV), and may be classified as either T- or non-T-natural killer (NK) cells according to CD3/T-cell receptor (TCR) status at the genomic or protein level. The present study reports three cases of non-nasal NK-NHL with the following characteristics: an agressive clinical behavior, heterogenous morphological data evoking pleomorphic T-cell malignant lymphoma, a non-T-NK phenotype using flow cytometry, and immunochemistry. The three cases were CD56 without membrane expression of specific T markers (CD3, CD5, and TCR). Heterogenous results were observed concerning different antigens: CD2, CD4, CD8, CD16, CD94, CD122, TiA1, perforin, and granzyme B. There was no evidence of detectable clonal TCR gene rearrangement with polymerase chain reaction. No NK activity was detected in the two tested cases, and no relation was found with EBV. Multidrug resistance investigations suggest that agressive clinical findings could be related to MDR1 gene expression as confirmed by MDR1 mRNA detection, MDR1 gene product (Pgp) expression, and a functional multidrug resistance study using rhodamine efflux by flow-cytometry.
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9/10. Bone allografts are immunogenic and may preclude subsequent organ transplants.

    The authors report a case of a 41-year-old woman with diabetes and chronic renal failure in whom antihuman leukocyte antigen antibodies developed after she received a frozen bone allograft that limited her access to organ donors. The patient had a chondrosarcoma of the right distal femur. A wide resection with segmental total knee arthroplasty was followed by a revision using a composite bone allograft prosthesis. After revision, broadly reactive lymphocytotoxic antibodies developed in the patient. The patient's panel reactive antibody level rose from 28% to a peak of 70%. Panel reactive antibody expresses the percentage of a panel of human leukocyte antigen type T lymphocytes from 40 individuals (representative of all human leukocyte antigen Class I histocompatibility antigens) to which antihuman leukocyte antigen Class I lymphocytotoxic antibodies have developed in the recipient as measured by the antiglobulin crossmatch method. The specificity of the patient's primary antibody is found in 45% of donors available in illinois since 1988 (N = 1606). Because a positive crossmatch precludes kidney and pancreas transplantation, at least 45% of cadaver organ donors were excluded from use for this patient. This is an unusual case that focuses on the potential impact of bone allografts in patients who may need subsequent organ transplantation.
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keywords = antigen
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10/10. Malignant glomus tumor: a case report and review of the literature.

    This report concerns a malignant glomus tumor, a rare soft tissue tumor that was examined immunohistochemically and ultrastructurally. It occurred in a 44-year-old male patient who had suffered from dull pain and stiffness in the right thigh for 10 months. Radiographic examination revealed a well-defined osteolytic lesion in the diaphysis of the right femur. Hypervascularity of the tumor was observed angiographically. Computed tomographic and magnetic resonance examinations showed an intramuscular mass invading the marrow space of the femur. Wide resection was performed after open biopsy. Histologically, round to polygonal tumor cells revealed a uniform appearance of round to ovoid nuclei with single large nucleoli and slightly eosinophilic cytoplasm, forming solid sheets of cells interrupted by vessels of varying size. A few mitotic figures and vascular invasion were observed. Immunohistochemically, vimentin and alpha-smooth muscle actin were stained intensely, and muscle actin was positive for tumor cells of the perivascular area. Tumor cells were negative for desmin, factor viii-related antigen, S-100 protein, neurofilament, cytokeratin, and epithelial membrane antigen. Ultrastructurally, tumor cells were characterized by many cytoplasmic processes, pinocytotic vesicles, plasmalemmal dense plaques, and scattered microfilaments in the cytoplasm. Few cell junctions and focal basement membrane-like structures were observed. No recurrence or metastasis was noted 57 months after operation. This case was considered to be a malignant glomus tumor, that is, a glomangiosarcoma arising de novo.
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