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 group of nineteen primary renal tumors in childhood was analyzed by flow-cytometry. All clinically important entities were included. Paraffin embedded material was used. Two anaplastic nephroblastomas and a case of renal rhabdomyosarcoma had an aneuploid DNA content. One Wilm's tumor showed a moderate DNA-hyperdiploidy. One of two rhabdoid tumors showed a marked right-sided shift of G1/G0 phase from that of a control and was considered as probably hyperdiploid. All other tumors including high grade neoplasms such as rhabdoid tumor and bone metastasizing tumors were found in the DNA-diploid range. Proliferation index was high in most tumors and in six it reached or exceeded 40. Mitotic index counted in the histological sections correlated with the percentage of G2 + M phase in most cases. Although small, the group of tumors under study shows that prognostic criteria cannot be based on the DNA content or on the proliferation index only. The only relevant result may be an overt DNA-aneuploidy in anaplastic nephroblastomas. Difficulties in interpreting the results of flow cytometry are discussed.
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PMID:[Flow cytometry in primary renal tumors in childhood]. 134 Mar 98

Fifty-six renal neoplasms reviewed by the National Wilms' Tumor Study Pathology Center presented with histologic features that resulted in confusion with rhabdoid tumor of kidney, a usually lethal childhood renal tumor; all were eventually diagnosed as other entities. Conspicuous filamentous cytoplasmic inclusions or large nucleoli, typical findings in rhabdoid renal tumors, were the usual source of diagnostic difficulty. Most, but not all, tumors occurred in pediatric patients. Sixteen were examples of Favorable Histology Wilms' tumor, which invited confusion with rhabdoid tumors either on the basis of filamentous cytoplasmic inclusions (15 cases) or macronucleoli (one case). In most cases, foci of typical Wilms' tumor blastemal aggregation or evidence of definitive nephrogenic differentiation facilitated the correct diagnosis. All 10 patients for whom information about outcome was available were alive at last follow-up. The other 40 renal lesions mimicking rhabdoid tumor of kidney consisted of a clinically and histogenetically diverse group of neoplasms, including anaplastic Wilms' tumor, congenital mesoblastic nephroma, renal cell carcinoma, transitional cell carcinoma, collecting-duct carcinoma, oncocytoma, rhabdomyosarcoma, malignant neuroepithelial tumors, and lymphoma. Most of these lesions could be separated from renal rhabdoid tumors and correctly classified on the basis of careful attention to light microscopic details, but in several cases electron microscopy or immunocytochemical studies were helpful or essential.
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PMID:Renal neoplasms mimicking rhabdoid tumor of kidney. A report from the National Wilms' Tumor Study Pathology Center. 165 2

The authors assessed a panel of immunohistochemical stains against 109 pediatric solid tumors, primarily rhabdomyosarcomas, under the auspices of the Intergroup Rhabdomyosarcoma Study. Fresh tumor tissue received from participating organizations was divided into portions that were either frozen or fixed in formalin, alcohol, or B5. Immunostaining was performed by the avidin-biotin complex method using monoclonal antibodies to desmin, neurofilaments, vimentin, cytokeratin, and leukocyte common antigen on cryostat sections. Tissue was also embedded in paraffin and stained with antimuscle-specific actin (MSA) and polyclonal antibodies to desmin, creatine kinase M subunit (CKM), myoglobin, and neuron-specific enolase (NSE). Antidesmin staining of cryostat sections was the most sensitive indicator of rhabdomyosarcoma (58 of 62 specimens positive). Results with this reagent in alcohol-fixed and formalin-fixed tissue were similar (46 of 56 positive versus 43 of 56 positive, respectively) and comparable with results with anti-MSA in formalin-fixed tissue (43 of 55 positive). However, the proportion of cells stained by antidesmin was higher in alcohol-fixed tissue than in formalin-fixed tissue. Staining with antimyoglobin and anti-CKM was much less satisfactory, with positivity rates of 17 of 37 and 11 of 57, respectively, in formalin-fixed rhabdomyosarcomas. Immunostaining of muscle markers revealed evidence of myogenesis in six undifferentiated sarcomas and in two sarcomas with inadequate histologic study on hematoxylin-eosin-stained sections. However, positivity was also noticed in samples of fibromatosis, Wilms' tumor, ectomesenchyoma, peripheral primitive neuroectodermal tumor, renal rhabdoid tumor, myositis ossificans, malignant fibrous histiocytoma, and embryonal sarcoma of the liver. The authors conclude that combined use of antidesmin and anti-MSA enhances the diagnosis of childhood sarcomas, especially when employed with other techniques such as electron microscopic study.
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PMID:Immunohistochemical study of childhood rhabdomyosarcomas and related neoplasms. Results of an Intergroup Rhabdomyosarcoma study project. 171 May 39

Fine needle aspiration (FNA) was performed under ultrasound guidance on 17 abdominal masses in 16 pediatric patients at Baragwanath Hospital. The aspirated cellular material was assessed by conventional cytomorphology and by electron microscopy (EM). A diagnosis of malignancy was rendered for all 15 tumors that were adequately sampled (88.2%); the remaining 2 masses yielded insufficient material for either light microscopy or EM. Cytologic cell typing (including the use of EM) was successful in 12 of the 15 tumors (80%) as compared with the histologic diagnosis. EM was in agreement with the initial cytologic diagnosis in eight tumors, but corrected the initial impression in four tumors. The tumors with adequate aspirates included nine nephroblastomas and single examples of neuroblastoma, hepatoblastoma, non-Hodgkin's lymphoma, rhabdomyosarcoma, renal carcinoma and malignant rhabdoid tumor. The last three were not accurately typed by cytology plus EM. These preliminary results suggest that FNA cytology with adjunctive EM could become a useful technique in the preoperative assessment of pediatric abdominal tumors.
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PMID:Fine needle aspiration of pediatric abdominal masses. Cytologic and electron microscopic diagnosis. 185 56

Immunoscans with the Fab-fragment of the monoclonal anti-myosin antibody R11D10 labeled with 111In showed significant uptake in rhabdomyosarcoma, rhabdoid tumor, and primitive neuroectodermal tumor. The diagnosis of an antimyosin positive or negative tumor with the aid of the immunoscan was superior to clinical findings in combination with ultrasound and CT, and to histological diagnosis in tumor biopsies. In addition chemotherapeutically induced renal impairment can be diagnosed by diminished renal uptake of antimyosin.
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PMID:Application of an anti-myosin antibody for scintigraphic differential-diagnosis of infantile tumors. 201 Mar 12

Transcripts for the muscle regulatory gene MyoD1 are expressed during normal skeletal muscle myogenesis and in rhabdomyosarcomas but not in other tissues or in soft-tissue sarcomas. Here we report the distribution of MyoD1 protein, determined by reactivity with anti-MyoD1 polyclonal sera in normal tissues, rhabdomyosarcoma cell lines, and in a variety of pediatric solid tumors. The distribution of MyoD1 protein was highly restricted in normal tissues and was detected only in fetal skeletal muscle and more faintly in adult skeletal muscle. All six human rhabdomyosarcoma cell lines analyzed expressed MyoD1 mRNA transcripts as well as immunoreactive protein. The immunohistochemical expression of MyoD1 protein was then examined in 49 surgical specimens from a variety of pediatric solid tumors. Each of 16 rhabdomyosarcoma specimens was positive for MyoD1, including four that did not express the intermediate filament protein desmin. Two of five specimens originally designated sarcoma type indeterminate (STI) and two of three specimens originally designated extraosseous Ewing's sarcoma (EOE) were positive for MyoD1, suggesting commitment to myogenic differentiation. Three of eight Wilms' tumors, which also expressed desmin and had clearly evident myogenic elements, also were positive for MyoD1. Tumors that failed to express MyoD1 protein included neuroblastoma, primitive neuroectodermal tumor, non-Hodgkins lymphoma, embryonal sarcoma of the liver, malignant fibrous histiocytoma, malignant rhabdoid tumor, and Ewing's sarcoma of the bone. These results indicate that expression of MyoD1 protein is highly restricted in normal human tissues and that expression of this gene product in malignant tissue may be diagnostic for rhabdomyosarcoma. Furthermore MyoD1 staining may be a valuable adjunct in the classification of pediatric soft-tissue sarcomas.
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PMID:Myogenic regulatory protein (MyoD1) expression in childhood solid tumors: diagnostic utility in rhabdomyosarcoma. 226 Jun 21

Ifosfamide/mesna was given to 97 patients who had malignant solid tumors diagnosed before they were 21 years of age. Patients received 1.6 g/m2 ifosfamide daily x 5, given i.v. over 15 min, followed by 400 mg/m2 i.v. mesna at 15 min and 4 and 6 h after ifosfamide. Responses were noted in patients with osteosarcoma, Ewing's sarcoma, rhabdomyosarcoma and other soft-tissue sarcomas, rhabdoid tumor, neuroblastoma, Wilms' tumor, primitive neuroectodermal tumor, retinoblastoma, germ-cell tumors, and B-cell lymphoma. Toxicity included mild to moderate nausea and vomiting, transient, reversible myelosuppression, transient elevations of serum blood urea nitrogen (BUN) and creatinine and liver enzymes, infections, and self-limiting neurotoxicity characterized by changes in mental status, motor dysfunction, cranial nerve palsy, cerebellar dysfunction, and seizures. Neurotoxic symptoms were generally seen in patients who had previously received cisplatin. Ifosfamide is an important alkylating agent that should be combined with other agents in phase II and III trials. Alternate dose schedules should also be investigated.
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PMID:Ifosfamide in pediatric malignant solid tumors. 250 57

Malignant rhabdoid tumour of the kidney is a recently reported tumour presenting in young children. Irrespective of stage and despite intensive chemotherapy these tumours have a poor prognosis, with death usually occurring within a matter of months. A recent report has shown the association of second embryonal tumours of the central nervous system occurring in patients with the renal tumour; most of these second tumours have occurred in the posterior fossa. We report here an infant who presented with a mass in the right groin, showing features of a poorly differentiated sarcoma, possibly rhabdomyosarcoma. Further investigations revealed a tumour in the lower pole of the right kidney which was subsequently shown to be a malignant rhabdoid tumour. The child was given chemotherapy but re-presented at 10 months of age with hydrocephalus, irritability and spasms leading to death. At autopsy a large tumour was found filling the right lateral and third ventricles; histology showed a primitive neuroectodermal tumour with focal astrocytic differentiation. Residual rhabdoid tumour was restricted to a few para-aortic lymph nodes and focal lymphatic micrometastases in lungs. The association of two embryonal neoplasms of possible similar histogenesis is discussed.
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PMID:Primitive neuroectodermal tumour of the central nervous system associated with malignant rhabdoid tumour of the kidney: report of a case. 301 61

Immunocytochemistry and electron microscopy have allowed more precise identification and classification of neoplasms formerly considered as representatives of other groups. The malignant rhabdoid tumor is one example. Principally neoplasms of the kidney, rhabdoid tumors present uncommonly in the head and neck, where because of the age of the patients and the neoplasms' resemblance to rhabdomyosarcoma, misdiagnosis is possible and likely. Wherever it arises, the lesion is usually fatal.
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PMID:Malignant rhabdoid tumor. 320 74

The differential diagnosis of malignant soft tissue tumors constitutes a major problem in surgical pathology. A distinction among sarcomas is sometimes difficult, but this is a situation in which histologic examination of representative tissue will give a diagnosis in the majority of cases. Electron microscopy sometimes contributes to the proper diagnosis of sarcomas. For electron microscopy, fresh samples from 110 cases of sarcoma filed at the Department of Pathology, Faculty of Medicine, Kyushu University, were examined. The distinguishing ultrastructural appearance of the following tumors are discussed: Spindle cell sarcomas (leiomyosarcoma 10 cases, malignant Schwannoma 5, fibrosarcoma 5, monophasic synovial sarcoma 7). Round cell sarcomas (rhabdomyosarcoma 10 cases, extraskeletal Ewing's sarcoma 1, malignant neuroepithelioma 1, malignant rhabdoid tumor of soft parts 3, monophasic synovial sarcoma 9). Pleomorphic sarcomas (malignant fibrous histiocytoma 24 cases, pleomorphic liposarcoma 1). Myxoid sarcomas (myxoid malignant fibrous histiocytoma 8 cases, myxoid liposarcoma 8, extraskeletal myxoid chondrosarcoma 4). Sarcomas with a specific pattern. Using electron microscopy, general analysis of cellular morphology and detection of specific structures are helpful for evaluating these sarcomas. Nevertheless, poorly differentiated sarcomas may still pose a diagnostic dilemma, since their specific features are poorly represented.
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PMID:[An ultrastructural analysis of malignant soft tissue tumors]. 359 2


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