Cases reported "Rhabdomyosarcoma"

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21/202. Primary pulmonary rhabdomyosarcoma: report of a case in an adult and review of the literature.

    A case of primary pulmonary rhabdomyosarcoma occurring in a 62-year-old man is reported, and a review of the literature is presented. The tumor affected the left upper lobe and involved the mediastinal lymph nodes. Immunohistochemical and ultrastructural studies supported the myogenic phenotype of the neoplasm. A left pneumonectomy was performed with complete surgical removal of the tumor. Postoperative radiotherapy was carried out. The patient is currently alive and free of disease 9 months after operation. Despite the rarity of primary pulmonary rhabdomyosarcoma, this tumor should be differentiated from other poorly differentiated pulmonary neoplasms and from metastatic sarcomas.
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keywords = neoplasm
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22/202. 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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keywords = neoplasm
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23/202. A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging.

    Kaposiform hemangioendothelioma (KH) is an endothelial-derived spindle cell neoplasm often associated with kasabach-merritt syndrome. Most cases arise in infancy and childhood and are soft-tissue tumors. The tumor displays an appearance between capillary hemangioma and Kaposi's sarcoma. We report a case of KH in a 1-year-old girl involving a mass that showed abnormal enhancement of soft tissue superficial to the right temporal bone with partial destruction of the temporal bone, the temporomandibular joint, mandibular condyle, and occipital bone. The physical finding of a discolored mass led clinicians to consider a hemangiomatous lesion, whereas the radiological picture suggested a more aggressive diagnosis of rhabdomyosarcoma and aggressive fibromatosis.
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keywords = neoplasm
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24/202. New techniques in hadrontherapy: intensity modulated proton beams.

    Inverse planning and intensity modulated (IM) X-ray beam treatment techniques can achieve significant improvements in dose distributions comparable to those obtained with forward planned proton beams. However, intensity modulation can also be applied to proton beams and further optimization in dose distribution can reasonably be expected. A comparative planning exercise between IM x-rays and IM proton beams was carried out on two different tumor cases: a pediatric rhabdomyosarcoma and a prostate cancer. Both IM x-rays and IM protons achieved equally homogenous coverage of the target volume in the two tumor sites. Predicted NTCPs were equally low for both treatment techniques. Nevertheless, a reduced low-to-medium dose to the organs at risk and a lesser integral non-target mean dose for IM protons in the two cases favored the use of IM proton beams.
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ranking = 0.048673043920277
keywords = cancer
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25/202. rhabdomyosarcoma of the uterus: report of two cases, including one of the spindle cell variant.

    Most uterine sarcomas fall into the category of leiomyosarcoma, endometrial stromal sarcoma, or undifferentiated sarcoma. Pure rhabdomyosarcomas are extremely rare, although a rhabdomyosarcomatous element may be present as a component of an adenosarcoma or carcinosarcoma (malignant mixed mullerian tumor). This report describes two uterine rhabdomyosarcomas in 28- and 67-year-old women. These were of spindle cell and pleomorphic types, respectively. At presentation the pleomorphic rhabdomyosaroma was stage IV, exhibiting massive pelvic and abdominal dissemination that mimicked an ovarian neoplasm. The spindle cell rhabdomyosarcoma was stage I, being confined to the uterus. Grossly, both uterine tumors had a polypoid appearance. Immunohistochemically, tumor cells were positive with the skeletal muscle markers sarcomeric actin, myoglobin, and myoD1. The patient with stage IV disease died within a short time of diagnosis and the other patient is alive and well at 2 years' follow-up. This report adds to the published literature on uterine rhabdomyosarcomas. This is the first reported uterine case of the spindle cell variant of embryonal rhabdomyosarcoma. Based on these cases and the published literature, rhabdomyosarcoma, especially the pleomorphic variant, appears to be a very aggressive neoplasm with an extremely poor prognosis. Immunohistochemical demonstration of skeletal muscle differentiation is necessary for a definitive diagnosis.
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keywords = neoplasm
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26/202. breast cancer genetics and cancer control. Tumor association.

    Verified breast cancer was present in a father, his mother, and his daughter. His sone had a brain tumor (by history) and his grandson, (ehs sone of the affected daughter), had a histologically verified rhabdomyosarcoma. This familial aggregation of cancers (except for leukemia, which is absent) is consistent with a newly described familial breast cancer syndrome. A single pleiotropic, dominantly transmitted gene, possibly interacting with carcinogenic factors, such as an oncogenic virus, may be the cause. A cancer-control potential exists for tumor associations such as those exhibited in this kindred, as well as for other cancer genetic syndromes where careful consideration is given to all histologic varieties of cancer.
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ranking = 0.68142261488389
keywords = cancer
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27/202. Perianal rhabdomyosarcoma presenting as a perirectal abscess: A report of 11 cases.

    BACKGROUND PURPOSE: The organs and soft tissues of the pelvis are some of the most common primary sites for rhabdomyosarcoma (RMS) in children and adolescents. In most cases a mass is detectable on clinical examination, and the initial concern is focused on the possibility of a neoplasm. The current report concerns 11 patients, each presented with a painful perineal-perianal mass suggesting an abscess to the extent that each one of these patients was treated initially with antibiotics or incision and drainage for several weeks to months before the pathologic diagnosis of RMS was established. methods: The authors reviewed the clinical histories of all patients with perirectal/perianal RMS from their respective institutions to identify cases in which the initial clinical diagnosis or impression was that of a perirectal abscess. Pathologic material was reviewed in all cases. RESULTS: Eleven patients, 7 of whom were girls, ranged in age from 1 to 16 years at diagnosis (median age, 14 years). fever accompanied the clinical presentation in 2 patients. None of the patients had a past medical history of illness that may have predisposed them to a perirectal abscess, although one patient had a family history of inflammatory bowel disease. Duration of symptoms ranged from 1 month to 1 year (mean, 4.6 months). Each patient presented with a tender perianal/perineal nodule or mass. Inguinal adenopathy was present in 6 patients at diagnosis. White blood cell counts ranged from 6,600/mm(3) to 24,500/mm(3). LDH levels ranged from 414 to 3,432 U/L. The average time from presentation to pathologic diagnosis of RMS was 2.1 months. Nine of the 11 cases showed an alveolar pattern. All were high-stage disease. Of 7 patients with follow-up longer than 1 year, 2 (29%) are alive without disease. CONCLUSION: This report presents the need to consider the possibility of a malignant neoplasm, in this case RMS, in a child or adolescent with a putative perirectal abscess that fails to respond in the expected manner to incision and drainage and antibiotic therapy.
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keywords = neoplasm
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28/202. Efficacy of phenytoin against hyponatremic seizures due to SIADH after administration of anticancer drugs in a neonate.

    A neonate with the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) developed refractory hyponatremic seizures following administration of anticancer drugs. The seizures did not respond to diazepam, phenobarbital, or lidocaine, but resolved immediately with administration of phenytoin. The low water-excretion capacity in neonates should be taken into consideration when fluid loading is attempted, to avoid renal damage upon administration of drugs such as cisplatin that have a potential damaging effect on the kidney. phenytoin could be the therapy of choice for SIADH and resulting seizures in the neonatal period.
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ranking = 0.24336521960139
keywords = cancer
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29/202. Orofacial rhabdomyosarcoma in neonates and young children: a review of literature and management of four cases.

    rhabdomyosarcoma (RMS) is an aggressive malignant skeletal muscle neoplasm arising from embryonal mesenchyme. It accounts for over 50% of all pediatric soft tissue sarcomas. The head and neck region is the most common site for this tumor in children. Neonatal presentation of this tumor is rare. We present the management of one neonatal case and three additional cases of orofacial RMS in children under the age of 7 years. All four patients were seen in the department of oral and maxillofacial surgery at Children's Hospital and Regional Medical Center (CHRMC) in Seattle between 1992-2000. Three of the four cases were alveolar RMS and one was botryoid sub-type of embryonal RMS. Three patients were treated with a combination of surgery, chemotherapy and radiation, while the patient with botryoid RMS was treated with surgery and chemotherapy only. The patient with congenital RMS died at 2.5 years of age due to recurrent metastatic disease. The other three patients are alive without evidence of recurrent with a mean follow up was 5.5 years (range 2.5-8.5 years). We discuss the current management, diagnosis, biological behavior, histopathology, prognosis and survival of head and neck RMS in neonates and young children.
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30/202. Rhabdomyosarcomatous (myoblastic?) phenotype of metastatic malignant melanoma. A case report.

    melanoma may present a great variety of cell morphology and mimic virtually almost all other neoplasms, and metastatic lesions pose major diagnostic difficulties. A typical immunoreactive profile of melanoma is: S-100p- and HMB-45-positivity, but these markers are not always present. Other new antigens for melanoma are NKIC3 and Anti-Melan-A (MART-1). This tumor may also present expression of unspecific markers, as cytokeratin, CEA, EMA, desmin, NFP, GFAP, CD68 and actins. We present here unusual features of two metastatic melanomas, showing the anaplastic pattern, immunopositive for desmin.
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keywords = neoplasm
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