Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A phase I study with recombinant human tumor necrosis factor alpha (rhuTNF-alpha; Knoll AG, Ludwigshafen, FRG) in patients with advanced malignant disease was undertaken to evaluate drug toxicity (organ specificity, time course, predictability, reversibility, maximal tolerated dose), effectiveness, antigenicity and pharmacokinetics. TNF was administered as a test dose followed by daily i.v. infusions for 5 days, every 3 weeks (single i.v. infusion lasting 10 min, TNF dissolved in 50 ml 5% human albumin). Dosage was increased in groups of 3 or 4 patients from 0.04 mg/m2 to 0.28 mg/m2. A total of 19 patients with different cancers, including seven large-bowel carcinomas, three chronic myelogenous leukemias, three hypernephromas, two small-cell lung cancers, one malignant melanoma, one malignant lymphoma, one rhabdomyosarcoma and one fibrosarcoma were treated. Major side-effects were chills and fever (maximum 40.4 degrees C, median 38.7 degrees C, 19/19), headache (12/19), nausea and vomiting (12/19) and pronounced (greater than 20%) hypotension (4/19). Acute side-effects could be diminished by paracetamol or indomethacin pretreatment, and with one possible exception no tachyphylaxis to TNF was noted. Mild renal toxicity was seen during TNF treatment. Pharmacokinetic studies showed a serum half-life (t1/2) ranging from 11 min to 17 min for doses from 0.04 mg/m2 to 0.16 mg/m2 and prolonged clearance with t1/2 ranging from 54 min to 70 min in the 0.20-0.28 mg/m2 dose range. No objective antitumor effects were observed in this phase I study.
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PMID:Phase I study of recombinant human tumor necrosis factor alpha in advanced malignant disease. 272 Jul 7

Twenty cases of metastatic neoplasms in the breast were identified in a series of 1,034 fine-needle aspirations (FNAs) of the breast, of which 389 were malignant. Patients with breast carcinomas in whom metastasis to the contralateral breast developed were excluded from this study. This series consisted of 17 women and 3 men, ranging in age from 28 to 63 years (mean, 49 years). The tumors included oat cell carcinoma (three), melanoma (three), ovarian serous carcinoma (one), bronchogenic adenocarcinoma and squamous carcinoma (four and two, respectively), lymphoma (two), carcinoid (two), transitional cell carcinoma (one), plasma cell myeloma (one), and rhabdomyosarcoma (one). In two patients, the breast mass was the first manifestation of an extramammary cancer (two adenocarcinoma of the lung). Eleven patients died of disseminated cancer shortly after the breast metastasis was diagnosed. In most cases, the aspirates displayed the cytologic features characteristic of the primary tumors, thereby establishing the metastatic nature of the neoplasm. In four cases (two carcinoids, one myeloma, and one rhabdomyosarcoma), the cytologic features were difficult to differentiate from a primary breast carcinoma; however, the final diagnosis was established by electron microscopic examination and immunocytochemical studies on the aspirates. One case (adenocarcinoma of the lung) was misdiagnosed as primary breast carcinoma on both FNA and mastectomy specimen. Because metastatic neoplasms in the breast may mimic primary breast tumors, the authors recommend the following: (1) Evaluation of FNA of breast should be done with complete knowledge of the patient's clinical history. (2) The possibility of metastasis should be suspected in lesions with unusual cytologic patterns. (3) Ancillary studies on FNA can be helpful in interpreting selected cases.
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PMID:Fine-needle aspiration cytology of metastatic neoplasms in the breast. 275 Jul 5

A 26-year-old male with a primary rhabdomyosarcoma of maxillary sinus is reported. The tumor was initially mistaken for an anaplastic Ki-1 positive anaplastic lymphoma in the histological preparation, owing to its high indifferentiation degree. After a smear study it was categorized as IEA, and chemotherapy of CHOP type was started; after two courses, local telecobalt therapy was given. After this was completed, the disease showed a progression, involving the bone marrow and resulting in clinical and cytological features consistent with acute leukemia. At that time we saw the patient for the first time. He is presently in complete remission after having started polychemotherapy of CVADIC type. After a commentary on the major study steps that led to the diagnosis, the crucial role of electron microscopy and, particularly, immunocytology for the correct identification of anaplastic tumors is emphasized.
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PMID:[Pseudohemopathy caused by rhabdomyosarcoma]. 275 52

This large series of cases demonstrates that the breast of the child or adolescent is the potential site for a number of interesting lesions exclusive of fibroadenoma and gynecomastia. Adenoma of the nipple, juvenile papillomatosis, and juvenile or cellular fibroadenoma should be correctly diagnosed to insure conservative surgical management and appropriate clinical follow-up. The results of the Juvenile Papillomatosis Registry suggest that this lesion is a morphological marker of potentially more serious breast disease in the future. Recognition of a cellular fibroepithelial neoplasm, especially in young black females, as a cellular fibroadenoma will hopefully eliminate the need for the diagnosis of "benign" cytosarcoma phyllodes. On occasion, the equivocal case may be labeled a "phyllodes tumor fo indeterminate biologic behavior." Those neoplasms with stromal atypia and mitotic activity, overgrowth of stroma with epithelial dissociation, and sarcomatous elements are appropriately designated as cytosarcoma phyllodes. In contrast to vascular tumors of the breast in adults, the majority of vascular lesions in the child's breast are bening and represent either a capillary or a histiocytoid hemangioma. Our experience and reports in the literature support the conclusion that most malignancies of the breast in children are metastatic. Rhabdomyosarcoma was the most common example in our study, but secondary involvement by acute leukemia or malignant lymphoma also occurs.
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PMID:Lesions of the breast in children exclusive of typical fibroadenoma and gynecomastia. A clinicopathologic study of 113 cases. 277 54

Developments in the treatment of childhood cancer have been evaluated in patients who had been treated in the National Children's Hospital from 1965 to 1987. The total number of patients was 867, of which leukemia accounted for 376, malignant lymphoma 61, neuroblastoma 174, Wilms' tumor 55, yolk sac tumor 29, rhabdomyosarcoma 36 and hepatoblastoma 30. Patients were divided into three time intervals: the 1960s, 1970s and 1980s. A marked improvement in five-year survival was recognized in Wilms' tumor and yolk sac tumor, amounting to 80%, followed by rhabdomyosarcoma, acute lymphoblastic leukemia and malignant lymphoma. There was no improvement in patients with acute non-lymphoblastic leukemia, neuroblastoma and hepatoblastoma. Prognostic factors for neuroblastoma were further analyzed, and the age of onset and stage of disease were found to have remained constant for 23 years. Factors relating to the improvement of survival were discussed.
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PMID:Improvement in the treatment of childhood cancer: analysis of survival data from the National Children's Hospital (1965-1987). 284 93

Skeletal involvement in childhood nonosseous tumors can be due to primary involvement, arrosion or metastasis, secondary due to therapy induced alterations or osteomyelitis following diminished immunity. The occurence of bone changes differs widely from those in adults. Neuroblastoma, rhabdomyosarcoma and malignant lymphoma are discussed in detail. Rare tumors are listed for synopsis. As diagnostic screening method skeletal scintigraphy is recommended, whereas in localized disease X-rays should be performed. Beside roentgenmorphology-particularly in primary disease-localisation, frequency and age dependency may give essential diagnostic hints. Prognosis depends on primary tumor.
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PMID:[Skeletal manifestations of malignant, nonosseous tumors in childhood]. 298 8

Modern multidisciplinary treatment of childhood cancer has made extent of disease evaluation important for proper treatment planning. Accurate staging is essential to cooperative group studies and for comparing treatment modalities at different centers. Operative staging plays an important role where clinical or imaging methods are limited, as in abdominal Hodgkin's disease or regional nodal metastasis. Operative staging is carried out either as a special diagnostic procedure, as in lymphoma, or as part of a planned surgical resection of a solid tumor. For lymphomas: Operative staging of abdominal Hodgkin's disease is required where protocols include involved field irradiation and sparing of normal growing tissue in the child. In non-Hodgkin's lymphoma, bulky abdominal tumor may be surgically evaluated after intensive chemotherapy either in delayed primary surgery or in second look procedures. Residual tumor may be excised or tagged with clips for localized irradiation to the tumor sparing normal abdominal organs. For solid tumors: During surgical resection of neuroblastoma, Wilms' tumor and rhabdomyosarcoma, the correct procedure involves regional staging either by formal node dissections or by multiple biopsies to determine extent of spread. Regional node dissections are often part of a correct cancer operation for cure, but also give staging information unobtainable by other methods. The surgeon must plan every procedure carefully with the aim of curing the patient and also deriving maximum information from the operation to enable correct planning of further treatment.
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PMID:The objectives and importance of operative staging of children with cancer. 301 92

Hypercalcemia, often associated with certain types of adult tumors, has also been described in pediatric neoplasms. In childhood, the more common associations include lymphoma, leukemia, rhabdomyosarcoma and rarely neuroblastoma. However, recently, several infants with hypercalcemia were described having renal tumors without bone metastases. The following is a case report of a 2-month-old infant who presented with severe hypercalcemia and a large right-sided abdominal mass, which at surgery was diagnosed as a cellular mesoblastic nephroma.
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PMID:Hypercalcemia in association with mesoblastic nephroma: report of a case and review of the literature. 302 25

Between 1981 and May 1986 31 children with solid abdominal tumor masses were observed in our clinic. The first diagnostic procedure was a sonographic examination, followed by further radiological investigations if necessary. 30 cases were examined histologically; in one case the sonographic findings were confirmed by an angiography. The most frequent abdominal masses were neuroblastomas and Wilms tumors (7 cases each). A mesoblastic nephroma was diagnosed in 3 cases, a lymphoma, a hepatoblastoma and a rhabdomyosarcoma 2 times each. One time we found a pancreas carcinoma, a teratoma, a hemangiomatosis of the liver, a malignant Schwannoma, a Ewing sarcoma, an adenoma of the adrenal gland, a pheochromocytoma and an osteosarcoma. According to our own experience and recent reports in the literature it seems possible in most cases, to predict the correct diagnosis of solid abdominal masses using the informations of sonographic imaging. Sonography is a highly specific non-invasive diagnostic tool for planning treatment (e.g. early surgery, cytostatic therapy and/or radiation) of solid abdominal masses. Nevertheless the histological examination should be performed in every case to confirm the definitive diagnosis.
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PMID:[Sonographic diagnosis of solid space-occupying abdominal lesions in childhood]. 303 88

22 undifferentiated tumours of the Salivary Gland Register (University of Hamburg) were studied by conventional light microscopical and immunocytochemical methods to elucidate the heterogeneity of this tumour group. The following observations were made in this collective: 18 tumours displayed one or more markers for the epithelial character and were classified as carcinomas. 10 carcinomas were considered as primary ones and 8 were considered as secondary ones (metastatic or invasive "per continuitatem"). Primary carcinomas were subclassified as poorly differentiated variants of a distinctive type of salivary gland tumours, as follows: 6 cases of carcinoma in pleomorphic adenoma, and one case each of mucoepidermoid tumour, adenocarcinoma, salivary duct carcinoma and epidermoid carcinoma. Secondary carcinomas were subclassified as follows: 3 epidermoid carcinomas, 3 nasopharyngeal carcinomas and 2 bronchial carcinomas. One tumour positive for S-100 protein and NSE (Neuron-specific enolase) was classified as a metastatic melanoma. Another tumour positive for vimentin and actin was classified as a rhabdomyosarcoma of the periglandular tissue. Two tumours lacked any markers studied here and were regarded as a malignant paraganglioma and an undifferentiated lymphoma, respectively. The differential diagnosis of the undifferentiated tumours of salivary glands and the special problems of this tumour group are discussed.
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PMID:Undifferentiated tumours of salivary glands. Immunocytochemical investigations and differential diagnosis of 22 cases. 303 6


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