Cases reported "Osteosarcoma"

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1/29. Restoration of immunocompetency by lymphocyte transfusion.

    A female patient with metastatic osteosarcoma was given a lymphocyte transfusion from her brother, cured of the same histologic type of tumor ten years earlier. Prior to the transfusion the patient was anergic. Following lymphocyte transfer cutaneous reactivity to DNCB was restored. skin reactivity to mumps and varidase antigens was specifically transferred. Whole lymphocyte transfer is a feasible method for restoring immune competence in the anergic cancer patient.
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2/29. 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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3/29. Extraskeletal osteosarcoma of the scalp.

    A rare case of extraskeletal osteosarcoma of the scalp in a 56-year-old woman is described. At presentation she was found to have an 8-cm diameter, tender, firm, exophytic scalp tumor. MRI scan confirmed absence of underlying skeletal origin and showed extension along the subcutaneous plane. The tumor was excised and the patient received post-operative chemotherapy. Histologically, the tumor showed classical features of an osteogenic osteosarcoma with focal fibroblastic areas. In addition, there were rhabdoid cells present, which showed paranuclear cytoplasmic immunoreactivity for epithelial membrane antigen. The patient developed metastatic disease 6 months after surgical excision.
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4/29. 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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5/29. Osteogenic sarcoma of the prostate.

    A 76-year-old man was treated with bilateral orchiectomy, estramustine phosphate and pelvic irradiation for prostate cancer. Osteogenic sarcoma of the prostate developed 18 months after the treatment. Postmortem examination revealed that the tumor was 8 cm in diameter and had infiltrated into the bladder and rectal walls and had resulted in peritoneal dissemination. There was no distant metastasis. Macroscopically, the tumor was ashen, firm and relatively homogenous and diffusely spread. Histologically, it was composed of spindle and pleomorphic cells, which were making osteoid with calcification. There was no ordinary tubular formation as shown in adenocarcinoma of the prostate. No positive immunostaining for prostate-specific antigen, epithelial membrane antigen and cytokeratin (AE-1, AE-3) were confirmed. Positive immunostaining for nonepithelial marker vimentin was confirmed. The ultimate diagnosis was osteogenic sarcoma of the prostate.
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6/29. Tissue-specific expression of SV40 in tumors associated with the li-fraumeni syndrome.

    Inactivation of wild-type p53 tumor suppressor function is the primary mechanism of tumor initiation in li-fraumeni syndrome (LFS) individuals with germline p53 mutations. Tumors derived from LFS patients frequently retain the normal p53 allele, suggesting that alternative mechanisms in addition to gene deletion must be involved in inactivating wild-type p53 protein. dna tumor viruses, such as SV40, target p53 for inactivation through the action of viral oncoproteins. We studied the probands from two unrelated LFS families, each of whom presented with multiple malignant neoplasms. Patient 1 developed an embryonal rhabdomyosarcoma (RMS) and a choroid plexus carcinoma (CPC), while patient 2 developed a CPC and subsequently presented with both an osteosarcoma (OS) and renal cell carcinoma (RCC). We utilized DNA sequence analysis and immunohistochemistry to determine p53 gene status in the germline and tumors, as well as evidence for SV40 T-antigen oncoprotein expression. Each patient harbored a heterozygous germline p53 mutation at codons 175 and 273, respectively. In patient 1, the normal p53 gene was lost while the mutant p53 allele was reduced to homozygosity in the RMS. Both normal and mutant genes were maintained in the CPC. In patient 2, normal and mutant p53 alleles were retained in both the CPC and RCC. Both specific PCR and immunostaining detected SV40 T-antigen in both CPCs and the RCC. In addition to chromosomal alterations, epigenetic mechanisms may disrupt p53 function during tumorigenesis. In two LFS patients, we found SV40 DNA sequences and viral T-antigen expression that could account for inactivation of the normal p53 protein. Inactivation of p53 or other tumor suppressors by viral proteins may contribute to tumor formation in specific tissues of genetically susceptible individuals.
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7/29. Chromophobe renal cell carcinoma with osteosarcoma-like differentiation.

    Sarcomatoid differentiation in renal cell carcinoma is thought to be the result of the dedifferentiation of the parent tumor, and it can be found in the chromophobe renal cell carcinoma just as other subtypes. We report a case of chromophobe renal cell carcinoma, which showed osteosarcoma-like differentiation. This is the first known case ever to be clearly identified as such. The patient was a 74-year-old man, and the CT scan revealed a huge retroperitoneal mass, which protruded from the lower half of the kidney and directly invaded the colon. Intraabdominal dissemination and metastases to the liver and lungs were also found. The resected tumor histologically showed sarcoma-like spindle cell proliferation and partly produced massive osteoid, which simulated the osteosarcoma. In addition, a typical histology of chromophobe renal cell carcinoma was found in part of the tumor. Immunohistochemically, spindle cells were reactive for epithelial membrane antigen, cytokeratin, and vimentin. The cell nests that were labeled by epithelial membrane antigen and cytokeratin were also found in the osteosarcoma-like area. We think that these phenomena were the result of "dedifferentiation" and metaplasia of the chromophobe renal cell carcinoma.
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8/29. Synovial sarcoma after chemotherapy for osteosarcoma: a case report.

    A 23-year-old man had eight cycles of adjuvant chemotherapy including doxorubicin, cisplatin, methotrexate, and ifosfamide and radical surgery for biopsy-proved osteosarcoma of the right fibular shaft. Two years after the initial diagnosis, he noticed a mass in the medial aspect of his right knee. magnetic resonance imaging scans revealed a soft tissue tumor measuring 2 x 2 cm in the pericapsular region of the right knee. Histologically, this soft tissue tumor was composed of spindle cells with occasional atypical mitoses and without matrix formation. Immunohistochemically, the tumor cells were positive for vimentin, cytokeratin, and epithelial membrane antigen, and negative for alpha smooth muscle actin. A fusion gene, SYT-SSX was detected with reverse transcription-polymerase chain reaction. From the results, the secondary tumor was diagnosed as a synovial sarcoma. The current case of double sarcomas is rare. Both sarcomas were diagnosed accurately using immunohistochemical and molecular procedures. This case suggests a positive association between a second tumor and chemotherapy including intraarterial perfusion of doxorubicin.
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9/29. Second primary osteosarcoma with rosette-like structure in a patient with retinoblastoma.

    A Japanese male patient developed bilateral retinoblastomas at the age of 1 year, but remained continuously disease-free after enucleation of the left eye and radiation therapy to the right eye. He noticed a painless hard mass around the right temporal bone when he was 25 years old. biopsy specimen showed a small multi-nodular proliferation of tumor cells with prominent rosette-like structures. Eosinophilic material with focal mineralization was seen in the center of the rosettes. Immunostaining of the tumor cells showed positive reactions for epithelial membrane antigens CD 56 and CD 99. The patient was treated with systemic chemotherapy, and the tumor partially diminished. It is well known that a few osteosarcomas show a rosette-like appearance with production of osteoid in the center, but this is the first case of second primary osteosarcoma with prominent rosette-like features.
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10/29. Non-myeloablative allogenic peripheral blood stem cell transplantation in a patient with refractory osteosarcoma.

    The prognosis of patients with relapsed osteosarcoma is dismal despite the use of intensive chemotherapy. We describe a patient with refractory osteosarcoma who underwent non-myeloablative peripheral blood stem cell transplantation (PBSCT) from an human leukocyte antigen (HLA)-identical sibling during a third complete remission. The patient suffered pulmonary relapse after the transplantation. Cyclosporin A withdrawal induced a graft-vs.-osteosarcoma effect and graft-vs.-host disease, but eventually the tumor progressed. Although our experience in this case suggested the presence of a graft-vs.-osteosarcoma effect during non-myeloablative allogenic PBSCT, this strategy might have limited value for refractory osteosarcoma with rapid growth kinetics.
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