1/451. bone marrow metastases in disseminated alveolar rhabdomyosarcoma: case report with ultrastructural study and review.A case of desseminated alveolar rhabdomyosarcoma in an 18-year-old male with leuco-erythroblastic anaemia is described. Numerous bizarre malignant cells, including frequent multinucleated giant cells, were seen in bone marrow aspirates, and osteolytic lesions appeared late in the clinical course. The primary site of the neoplasm remained undertermined during life and also at necropsy, which revealed minute pulmonary metastases and extensive lymph nodal, pleural and skeletal deposits. The diagnosis was confirmed on necropsy tissue by ultrastructural examination which demonstrated numerous thin (5 nm) and thick (15 nm) intracytoplasmic filaments in tumour cells, sometimes organized in bundles; scattered dense Z-band-like bodies, and rod-shaped structures were also seen. The fine structure of the rhabdomyosarcoma in the present case is compared with previous ultrastructural studies. Elongated, thick intracytoplasmic filaments whose diameter corresponds to that of myosin myofilaments are strong evidence for rhabdomyoblastic differentiation and are considered to be the sine qua non of a positive electron microscopic diagnosis of rhabdomyosarcoma. Orgaized bundles of filaments and Z-band-like dense bodies are usually present, and rod-shaped structures are found infrequently, but none of these are necessary for the ultrastructural diagnosis.- - - - - - - - - - ranking = 1keywords = sarcoma (Clic here for more details about this article) |
2/451. Molecular assessment of clonality leads to the identification of a new germ line TP53 mutation associated with malignant cystosarcoma phyllodes and soft tissue sarcoma.Cystosarcoma phyllodes (CSP) is a rare breast neoplasm composed of stromal and epithelial elements. It usually runs a benign course but it may metastasize. In a 31-year-old patient with recurring CSP, a mesenchymal tumor in the leg developed. The question arose whether the latter tumor could be a metastasis from the CSP, which would have major treatment consequences. The problem was addressed using molecular methods, i.e., comparison of the pattern of polymorphic repeat markers on chromosome 17p as well as single strand conformation polymorphism analysis and sequencing of exons 5 to 8 of the TP53 gene in both tumor and normal tissue. An identical pattern of loss of heterozygosity in both breast tumors was demonstrated, but a different pattern was shown in the tumor in the leg. This led to the conclusion that the latter tumor had to be a new primary tumor. A mutation in codon 162 of the TP53 gene was found in the tumor tissue as well as in the normal tissue of this patient. This germ line mutation leads to the replacement of isoleucine by asparagine and most likely has functional consequences. In all four examined tumors of this patient, the normal TP53 allele was lost. This is strong evidence that this germ line TP53 mutation causes the genesis of these two rare primary mesenchymal tumors in this young patient. The current study exemplifies the power of molecular diagnostic methods in investigating the specific clinical problem of clonal relation between two separate tumors. The germ line mutation found in codon 162 of the TP53 gene and the association with cystosarcoma phyllodes have not been described previously.- - - - - - - - - - ranking = 1.4285714285714keywords = sarcoma (Clic here for more details about this article) |
3/451. meningioma with sarcomatous change and hepatic metastasis.A 72-year-old patient had a meningotheliomatous meningioma that invaded through the skull and into temporalis muscle. One year following craniotomy for removal of the neoplasm, he developed headaches, diplopia, and proptosis of the left eye. biopsy of the orbital contents revealed a malignant supporting tissue neoplasm having a resemblance to the previous meningioma. No curative therapy was possible and the patient died 33 months after diagnosis. autopsy examination showed extensive residual intracranial neoplasm and a 3-cm metastasis in the liver. The metastatic tumor appeared similar to the meningioma and did not appear malignant histologically. The case illustrates the distinct histologic variations in meningiomas and the difficulties in predicting their biologic activity. Aggressive local behavior may indicate possible malignant areas in the neoplasm. Therefore, examination of the neoplasm should be thorough. Such a correlation may suggest malignant biologic potential.- - - - - - - - - - ranking = 0.57142857142857keywords = sarcoma (Clic here for more details about this article) |
4/451. dna ploidy alterations detected during dedifferentiation of periosteal chondrosarcoma.dna ploidy of a case with dedifferentiated periosteal chondrosarcoma was analyzed by dna cytofluorometry. The diagnosis of primary periosteal chondrosarcoma was made on the basis of the radiographic and histological findings. At 4 years after marginal resection, the tumor recurred locally and metastasized to various organs. The patient died of disease 2 years later. Histologically, there were two components, chondrosarcoma and malignant fibrous histiocytoma, in the recurrent and metastatic tumors. dna ploidy analysis of multiple samples revealed that the primary lesion was composed of many diploid cells with some tetraploid and octaploid cells, whereas in the dedifferentiated area, there were many aneuploid cells which were not recognized in any area of the primary tumors. This case illustrated that dna ploidy alteration of euploidy to aneuploidy is closely correlated with the process of dedifferentiation in chondrosarcoma.- - - - - - - - - - ranking = 1.1428571428571keywords = sarcoma (Clic here for more details about this article) |
5/451. Low-grade malignant perineurioma of the paravertebral column, transforming into a high-grade malignancy.A demarcated 6 x 5 cm right paravertebral tumor at the level of T6 in a 39-year-old male was removed surgically. Histologically, the tumor consisted of monomorphous benign-looking, low-cellular spindle cells embedded in desmoplastic stroma. Ten years later, the tumor recurred locally with metastasis to systemic organs, including the occipital skin. Malignancy was histologically evident by the increased cellularity, cellular atypia and mitotic activity. The patient died of respiratory failure at the age of 49. Retrospectively reviewed, the primary lesion was low-grade fibrosarcoma-like spindle cell tumor, with secondary transformation into a highly malignant form. The differential diagnoses included sclerosing epithelioid fibrosarcoma, low-grade fibromyxoid sarcoma and malignant peripheral nerve sheath tumor. Immunohistochemically, the spindle cells in the primary and recurrent tumors consistently expressed epithelial membrane antigen, vimentin, type 4 collagen and laminin. The tumor cells in the present case showed a differentiation toward perineurial cells, which are normally positive for these immunohistochemical markers. Hence, the appropriate diagnostic term should be 'malignant perineurioma', a subtype of malignant peripheral nerve sheath tumor.- - - - - - - - - - ranking = 0.42857142857143keywords = sarcoma (Clic here for more details about this article) |
6/451. Benign metastasizing leiomyoma of the uterus: histologic and immunohistochemical characterization of primary and metastatic lesions.Benign uterine leiomyoma metastasizing to the lung is a recognized entity that has been reported infrequently in the medical literature. There is persisting controversy regarding the pathogenesis and biology of these lesions. We report a well-studied and well-characterized case of benign leiomyoma metastasizing to the lung. The patient was a 72-year-old woman with an enlarged uterus that contained several leiomyomas with usual histology. Areas of fibrosis, hyalinization, edema, and focal infarction together with small foci with mildly increased cellularity and minimal nuclear pleomorphism were seen. Careful and repeated mitotic counts ranged from 0 to 2 mitoses per 10 high-power fields. In summary, based on histopathologic criteria, the neoplasm was determined to be a focally cellular benign leiomyoma. Four years later, the patient underwent surgical resection of a single nodule in the lung, which had been detected on routine radiographs. Histopathologic evaluation showed a low-grade leiomyosarcoma with moderate nuclear pleomorphism, necrosis, and brisk mitotic activity. Immunohistochemical studies performed on both neoplasms showed them to be of mesenchymal derivation with smooth muscle differentiation. Both neoplasms expressed estrogen receptors with moderate to strong intensity. The patient received no further treatment and, to date, shows no evidence of recurrent disease. The diagnosis of benign metastasizing leiomyoma can only be made with certainty after careful and extensive sampling of the primary tumor to exclude small foci of sarcoma and of the pulmonary tumor to rule out a primary neoplasm. Although it is biologically peculiar, benign metastasizing leiomyoma should continue to be recognized as a distinct entity because current morphologic criteria do not allow primary myometrial tumors to be reclassified as leiomyomas of uncertain malignant potential even if they have metastasized to the lung.- - - - - - - - - - ranking = 0.28571428571429keywords = sarcoma (Clic here for more details about this article) |
7/451. Prognostic factors in osteosarcoma. A review of 20 year's experience at the University of Pittsburgh health Center hospitals.The histologic and clinical characteristics of 54 patients with osteosarcoma are reviewed. The association of rapid linear bone growth with the occurrence of this tumor is confirmed, and evidence for increased growth in these adolescent patients is presented. A significant increase in female survival is seen in this study, and the literature relevant to gender and survival is reviewed. A histologic characterization of six predominant tumor patterns is presented with correlation to survival. Increased survival is seen with two specific histologic patterns, but there is great variability in the histology and sampling of osteosarcomas, and the series is small.- - - - - - - - - - ranking = 3.0125340063869keywords = osteosarcoma, sarcoma (Clic here for more details about this article) |
8/451. "Skip" metastases in osteosarcoma.Careful study of 40 cases of osteosarcoma without evidence of multifocal disease, pulmonary metastasis, or history of exposure to predisposing factors has given histologic evidence of microscopic foci of osteosarcoma separate from the primary focus of osteogenic sarcoma. These "skip" lesions are to all pathologic examination completely separate from the primary focus of osteogenic sarcoma. They are more often found proximal to the primary, both intraosseously and transarticularly. Histologically, these "skips" represent areas of osteosarcoma which in many cases are a less-differenitated form of the tumor. The natural history of such tumors with "skips" following ablative surgery is an increased incidence of local recurrence and subsequent pulmonary metastases.- - - - - - - - - - ranking = 4.8765426767472keywords = osteogenic, osteosarcoma, sarcoma (Clic here for more details about this article) |
9/451. 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.- - - - - - - - - - ranking = 4.587848008952keywords = osteogenic, osteosarcoma, sarcoma (Clic here for more details about this article) |
10/451. Spontaneous pneumothorax and pulmonary malignant disease--a syndrome sometimes associated with cavitating tumours. Report of nine new cases, four with metastases and five with primary bronchial tumours.Tumours of the lung, primary or secondary, may occasionally be complicated by a pneumothorax, and on rare occasions this may be the presenting feature. Metastatic tumours associated with pneumothorax arise usually from bone or soft tissue sarcomas and hence are more common in the young.- - - - - - - - - - ranking = 0.14285714285714keywords = sarcoma (Clic here for more details about this article) |
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