11/153. Experience with anti-angiogenic therapy of giant cell granuloma of the facial bones.Interferon alfa-2a inhibits angiogenesis and was discovered through a series of laboratory experiments that began in 1980. It was first used in 1989 in the management of a child with pulmonary haemangiomatosis. Interferon alfa A was then subsequently use to treat life threatening haemangiomas and other vascular tumours in various organs. Kaban reported on anti-angiogenic therapy of a recurrent giant cell tumour of the mandible in a 5 year old girl with interferon alfa-2a reasoning that as it was a rapidly proliferating vascular lesion it could be treated as an haemangioma. This paper reviews the history and role of interferon alfa-2a as an angiogenesis inhibitor in the treatment of complex haemangiomas and presents its use in the successful management of a rapidly growing central giant cell granuloma in a 4 year old boy in australia.- - - - - - - - - - ranking = 1keywords = tumour (Clic here for more details about this article) |
12/153. Primary sarcoma of the lung.Primary sarcoma of lung is defined to exclude sarcomata of lymphatic origin. A series of nine patients is presented, all of whom were seen in Edinburgh. In each case there is histological proof of the diagnosis or sarcoma. From the same population and during the same period of time, 6000 cases of bronchial carcinoma presented. Eight of the nine patients underwent resection of the tumour, and the pathological findings and clinical results are described. From a review of the literature and from the Edinburgh series, a general series of 59 cases of primary sarcoma of the lung has been selected. The criteria for selection were directed at the assessment of the results of surgery in the treatment of this lesion. The five-year survival rate after 'curative' surgery is 52%, and the prognosis after surgery does not appear to depend on the radical nature of the operation.- - - - - - - - - - ranking = 0.5keywords = tumour (Clic here for more details about this article) |
13/153. rhabdomyosarcoma of the adult nasopharynx.rhabdomyosarcoma of the adult head and neck is rare, particularly beyond 40 years of age. In this region, the nasopharynx is an unusual site. Most nasopharyngeal rhabdomyosarcomas are of an alveolar variety. We report a case of embryonal or spindle cell rhabdomyosarcoma in the nasopharynx of a 47-year-old man. The histology of this tumour revealed clear cells that have not been described in embryonal rhabdomyosarcomas occurring in the head and neck. The pathology is discussed and current literature reviewed.- - - - - - - - - - ranking = 0.5keywords = tumour (Clic here for more details about this article) |
14/153. Rhabdoid epithelioid leiomyosarcoma of the uterine corpus: a case report and literature review.A case of epithelioid leiomyosarcoma of the uterus with rhabdoid phenotype and early rhabdomyoblastic differentiation is described. A 72-year-old woman with a 5-week history of increased abdominal girth was found to have a large pelvic mass. The uterus revealed a large intramyometrial and left adnexal necrotic tumor that had spread to the small bowel mesentery and to the anterior abdominal peritoneum. The tumor was an epithelioid leiomyosarcoma with rhabdoid phenotype and focal early rhabdomyoblastic differentiation, as confirmed by immunohistochemical and ultrastructural techniques. Also called composite extrarenal rhabdoid tumor (CERT), this lesion should be differentiated from malignant mixed mullerian tumor, rhabdomyosarcoma, endometrial stromal sarcoma, and pure rhabdoid tumors of the uterus. The recognition of a rhabdoid phenotype is of clinical importance since these tumors are prone to be aggressive.- - - - - - - - - - ranking = 28.819481786681keywords = rhabdoid (Clic here for more details about this article) |
15/153. Concomitant intramyocardial and epicardial vasculitis in an autopsied heart allograft for cardiac rhabdomyosarcoma.Primary cardiac tumours are rare, with only one quarter of the patients being malignant. The vast majority of malignant neoplasms of the heart are sarcomas. We describe a patient of primary cardiac rhabdomyosarcoma presented as coronary artery disease and recurrent myocardial infarction. Histopathologic finding of the excised native heart revealed a high grade pleomorphic rhabdomyosarcoma in the myoepicardial portion of the anterior wall with rupture. The accompanying unusual feature was myocardial infarction because of tumour emboli of the left anterior descending and left circumflex coronary arteries. After transplantation, the patient developed mild to moderate acute cellular rejection of the transplanted heart on post-transplantation day 1, 8, and 44, respectively. Unfortunately, he expired on the post-transplantation day 47 because of acute rejection, presenting as concomitant intramyocardial and epicardial lymphocytic vasculitis and multifocal myocardial ischaemia. We found that this uncommon medial lymphocytic vasculitis lesion was mediated by T cells and also by antibody directly against smooth muscle cells of small arteries. The consequence of such immune response would be compromised myocardial oxygenation resulting in allograft failure.- - - - - - - - - - ranking = 1keywords = tumour (Clic here for more details about this article) |
16/153. Botryoid rhabdomyosarcoma of prostate--a case report.Botryoid rhabdomyosarcoma of the prostate is a rare tumour in children, mostly below 6 years of age. A case of 5 year old male child presenting with acute retention of urine due to botryoid rhabdomyosarcoma of the prostate is described.- - - - - - - - - - ranking = 0.5keywords = tumour (Clic here for more details about this article) |
17/153. Alpha-fetoprotein production by rhabdomyosarcoma of the urinary bladder.Alpha-fetoprotein has emerged as a useful diagnostic tool for hepatic tumour and tumours of germ cell origin. However, isolated case reports of association of this tumour marker with tumours of the lung and non-germ cell tumours of the ovary are reported. We present a case of a rhabdomyosarcoma, a generally non-secretory tumour that showed raised levels of AFP in serum and reacted positively for the same in a PAP technique for AFP. Cross striations were visible in many cells on II & E stained sections.- - - - - - - - - - ranking = 3keywords = tumour (Clic here for more details about this article) |
18/153. microsatellite instability in a pleomorphic rhabdomyosarcoma in a patient with hereditary non-polyposis colorectal cancer.AIMS: To describe the rare occurrence of a pleomorphic sarcoma with microsatellite instability in a patient with hereditary non-polyposis colorectal cancer (HNPCC). methods AND RESULTS: A soft tissue tumour was removed from the upper leg of a patient who had previously been shown to harbour a germ-line MSH-2 mutation. The tumour was analysed with immunohistochemistry and molecular methods. The morphology and immunohistochemical findings were in keeping with a pleomorphic rhabdomyosarcoma. microsatellite instability was documented in the tumour with molecular methods and in addition loss of MSH-2 expression in the tumour cells was confirmed by immunohistochemistry. CONCLUSIONS: Although sarcomas do not form part of the HNPCC diagnostic criteria, they may occur in this mismatch repair syndrome and, moreover, may well be caused by the underlying genetic defect.- - - - - - - - - - ranking = 2keywords = tumour (Clic here for more details about this article) |
19/153. Rhabdomyosarcomas developing in association with mediastinal germ cell tumours.Two mediastinal rhabdomyosarcomas that arose in association with germ cell tumours are reported. One presented as a small component of a mixed germ cell tumour with yolk sac and immature teratomatous elements. The other appeared as a large mass 4 months after diagnosis of a yolk sac carcinoma that had been treated with chemotherapy. The first patient was alive and free of disease 7 years later, whereas the second died of tumour 3 months post-operatively. The proportion of rhabdomyosarcoma within the germ cell tumours appears to have influenced the prognosis of these patients. This observation emphasizes the necessity of performing a thorough search for sarcomatous elements and quantifying their relative proportion in germ cell tumours of the mediastinum.- - - - - - - - - - ranking = 4.5keywords = tumour (Clic here for more details about this article) |
20/153. Malignant rhabdoid tumor of soft tissues: a cytopathological and immunohistochemical study.We describe the cytopathological and immunohistochemical features of a primary malignant rhabdoid tumor (MRT) located in the left forehead region of an 8-mo-old female. The cardinal characteristics are hyaline cytoplasmic inclusions, rounded or bean-shaped nucleus, vesicular chromatin, central prominent nucleolus, and poorly defined cytoplasmic profile. Both the cytoplasm and the hyaline inclusion are vimentin and keratin positive, but negative with desmin, S-100 protein, neuron-specific enolase, (NSE) myoglobin, leukocyte common antigen (LCA), and alpha-1-antichymotrypsin. This immunohistochemical pattern, although not exclusive to MRT, is fairly typical of MRT, and when it is assessed in conjunction with cytopathological findings and clinical data permits a confident diagnosis of MRT. We emphasize the utility of immunohistochemical techniques in making the differential diagnosis of MRT against other childhood round-cell tumors.- - - - - - - - - - ranking = 28.819481786681keywords = rhabdoid (Clic here for more details about this article) |
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