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

Cancer-related problems are seen frequently by the emergency physician. More difficult presentations are seen with premonitory symptoms, paraneoplastic syndromes, and nonspecific lesions. Dermatologic paraneoplastic syndromes are numerous, nonspecific, and consist of hamartomatous growths, texture changes, new hair growth, or changes in skin color. Alteration of skin color may be of practically any color, localized or diffuse, and of sudden or indolent onset. Hormone production by tumors may lead to acne, hirsutism, gynecomastia, or a cushingoid appearance. Pruritus may herald the onset of leukemia or lymphoma and be intolerable, as with erythroderma. All suspicious presentations require thorough investigation for underlying disease. Metastasis to skin is not common and implies a poor prognosis if seen. Most metastases are seen on the head and neck, anterior chest wall, and abdomen. Basal cell and squamous cell carcinomas commonly occur in sun-exposed areas. Basal cell is locally destructive, whereas squamous cell occasionally metastasizes to local lymph nodes. Malignant melanoma is the leading fatal illness originating in skin, with a dramatic rise in incidence. It is classically described as asymmetric with irregular borders, is elevated, and shows color variegation; however, melanoma may present atypically, particularly in non-whites. Kaposi's sarcoma lesions are well-demarcated, symmetric, smooth nodules that appear purplish-brown, particularly if below the knee (owing to venous stasis). The closely interrelated structures of the eye and orbit are easily disturbed, leading to the presenting symptoms of visual disturbances, exophthalmos, pain, and ocular motility disorders. Primary tumors are not unusual and may include retinoblastoma, rhabdomyosarcoma, and melanoma. Equally common are metastatic lesions, most commonly lung and breast carcinoma. An estimation of the malignancy of bony lesions can be made by assessing the zone of transition, periosteal reaction, and bone destruction. A malignant lesion will more likely have a broad zone of transition, irregular periosteal reaction, and moth-eaten or permeative destruction of trabeculae. Metastatic bone lesions primarily occur in sites of persistent red marrow: skull, ribs, vertebrae, pelvis, and proximal humerus and femur. Bony lesions can be blastic or lytic in nature. Solitary pulmonary nodules that have not grown for 2 years can be assumed to be benign. Calcification seen on plain films are a strong (but not absolute) indication of benignancy. Lesions that are greater than 3 to 4 cm in diameter, have irregular contours, are cavitated with thick walls, have multiple peripheral nodules, and have lack of calcification are more likely malignant.
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PMID:Visual diagnosis of hematologic and oncologic diseases. 849 Nov 9

A retrospective review of 376 pediatric orbital tumor cases seen at the Ankara University Eye Clinic between 1963 and 1993 was undertaken. The diagnosis was made histologically in every case. Secondary tumors accounted for 127 cases (33.8%), cystic lesions for 82 cases (21.8%), rhabdomyosarcomas for 23 cases (6.1%), vascular lesions for 21 cases (5.6%), inflammatory lesions for 21 cases (5.6%), lymphoma and leukemias for 18 cases (4.8%), other mesenchymal tumors for 11 cases (2.9%), metastatic tumors for 5 cases (1.3%), traumatic foreign bodies for 2 cases (0.5%), and lacrimal fossa lesions for 1 case (0.3%). The most common benign orbital tumors were the cystic lesions. The most common primary malignant tumor was rhabdomyosarcoma. Overall, the most frequent orbital lesion was the secondary orbital invasion of retinoblastoma.
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PMID:Pediatric orbital tumors in Turkey. 854 Dec 61

Two children with Klinefelter syndrome (KS), one associated with bilateral hereditary retinoblastoma (RB) and the other with rhabdomyosarcoma (RMS) are reported. Both were boys and chromosomally mosaic for KS. The hereditary retinoblastoma case yielded 46,XY,del(13)(q12q14.2)/47, XXY(c),del(13)(q12q14.2) in PHA-stimulated lymphocytes. The rhabdomyosarcoma case yielded 46,XY/ 47,XXY(c) in peripheral blood cells whereas tumor revealed trisomy 8, trisomy 7, and t(7;13)(q33;q32) in addition to 46,XY/47,XXyc mosaicism.
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PMID:Clinical and cytogenetic studies of two cases of Klinefelter syndrome with hereditary retinoblastoma and rhabdomyosarcoma. 868 18

While fairly complete and reliable incident data on childhood cancers are available from the registries in India, mortality and survival information is not. Information concerning the latter was obtained by the Bangalore cancer registry through active follow-up involving visits to homes of patients. Between 1982 and 1989, 617 cases of cancers in childhood were registered, giving an age-standardized incidence rate of 84.8 and 48.4 per million in male and female children, respectively. Active follow-up provided mortality/survival information in 532 or 86.2 percent of these cases. Overall, observed five-year survival was 36.8 percent (both genders combined) with a relative survival of 37.5 percent when childhood mortality in the general population was taken into account. The five-year relative survival was best for thyroid carcinoma (100 percent) followed by Hodgkin's disease (73 percent) and retinoblastoma (72.9 percent). Survival was comparatively low, being 9.9 percent in acute nonlymphatic leukemia and less than 20 percent in rhabdomyosarcoma and the category grouped as 'other malignant neoplasms.' Survival in Hodgkin's disease was influenced by clinical stage at presentation, but was not statistically significant possibly due to small numbers.
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PMID:Descriptive epidemiology of childhood cancers in Bangalore, India. 881 27

Cancer survival among children and adolescents has improved markedly due to evolution of multimodal treatment that incorporates combination chemotherapy, radiation therapy and/or surgery. However, 20-30% of children with malignancies will succumb to their disease or complications associated with their disease or treatment. A major limiting factor to improvement in survival among these patients is the occurrence of intrinsic and/or acquired resistance to our treatment interventions, chemotherapy and radiotherapy. Among these mechanisms, multidrug resistance, the focus of this review, is a well-documented phenomenon whose biochemistry, pharmacology and molecular biology has been extensively studied. A role for multidrug resistance in chemoresistance and therapeutic failure in childhood malignancies is suggested by the observation of clinical resistance to treatment regimes containing agents that are known substrates of multidrug resistance mechanisms. With the current results from studies in rhabdomyosarcoma, neuroblastoma, osteosarcoma, Ewing's sarcoma, leukemia and retinoblastoma, the role of multidrug resistance is still unclear. Earlier studies attempted to define a role for P-glycoprotein-mediated multidrug resistance; however, a limited number of reports suggest that the multidrug-associated resistance protein may play an active role in neuroblastoma. Further studies will be necessary using standardized and uniform approaches for the analyses of these mechanisms. Clinical trials directed toward reversal of multidrug resistance are premature since the exact role of P-glycoprotein is controversial in pediatric malignancies, the role of other mechanisms of multidrug resistance must be assessed and selective inhibitors of multidrug resistance have yet to be developed.
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PMID:Multidrug resistance in pediatric oncology. 888 Mar 94

Terminal differentiation of myocytes involves withdrawal from the cell cycle, induction of myogenin expression, and finally formation of myotubes. To study the factors that regulate the initial phase of muscle differentiation, we analyzed the binding activities of transcription factors AP-1, Sp-1, and NF-kappa B in L6, C2C12, and rhabdomyosarcoma BA-Han-1C cells. Temporal changes in transcription factor binding activities were compared to the activation of myogenin promoter-driven CAT reporter gene and the expression level of myogenin, a master gene of myogenic differentiation. We observed a prominent decrease in the nuclear binding activities of AP-1, Sp-1, and NF-kappa B already 12 to 24 h after the transfer of cells to differentiation medium. The response was very similar in L6 and C2C12 myocytes and in BA-Han-1C rhabdomyosarcoma cells. The down-regulation clearly preceded the activation of myogenin promoter and the induction of myogenin and retinoblastoma expression, as well as the initiation of myocyte fusion. Cholera toxin and okadaic acid, established inhibitors of myogenin expression and muscle differentiation, strongly up-regulated the binding activities of AP-1, Sp-1, and NF-kappa B in differentiation medium. Myogenin expression and myocyte fusion were also inhibited. Levels of nuclear c-Fos and c-Jun proteins, components of the AP-1 complex, showed a prominent decrease already after 12 h in differentiation medium. These results show that the down-regulation of the proliferation-promoting transcription factors is a prerequisite to the initiation of myocyte differentiation.
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PMID:Down-regulation of transcription factors AP-1, Sp-1, and NF-kappa B precedes myocyte differentiation. 895 80

The risk of second malignancy after retinoblastoma is reported to be as high as 20% at 10 years after initial diagnosis. This incidence may be an overestimate because of difficulties in distinguishing a second malignancy from recurrent tumor. We encountered a patient with bilateral retinoblastoma who developed a temporal mass 3.5 years after initial treatment for what had first been diagnosed as rhabdomyosarcoma; further study suggested that it was recurrent retinoblastoma manifesting as primitive neuroectodermal tumor (PNET) with multilineage differentiation. Chromosome 13 abnormalities were compatible with either rhabdomyosarcoma or recurrent retinoblastoma. To determine how often second malignancies in retinoblastoma patients may be confused with recurrent primary tumor, we reviewed our experience at Children's Hospital of Pittsburgh. Of 43 retinoblastoma patients seen between 1951 and 1992, presumed second malignancies were documented in four, including the current case. Of the three other second tumors, one had both neural and skeletal muscle differentiation; another was diagnosed as rhabdomyosarcoma unclassifiable as embryonal or alveolar; the last was an osteosarcoma. Only the osteosarcoma was clearly a second neoplasm; two and perhaps three of the other cases may be recurrent retinoblastoma. The distinction between second malignancy and recurrent retinoblastoma may be difficult but is worth determining, because treatment may differ, depending on the correct designation.
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PMID:Malignancy after retinoblastoma: secondary cancer or recurrence? 902 3

In the framework of the ITACARE project, a cooperative investigation conducted on the data from the Italian population-based cancer registries, survival of patients with childhood malignant neoplasms was studied. The study included 1,768 cases diagnosed at age 0-14 plus 29 osteosarcoma cases diagnosed at age 15-19. Cases were collected over the period 1978-1989, or more limited periods for some participating registries. A total of 1,138 cases were from the Childhood Cancer Registry of Piedmont and 659 from the registries operating in the provinces of Varese, Parma, Modena, Forli and Ravenna, Florence, Latina, Ragusa and in the cities of Genova and Torino (the last contributed only for bone neoplasm diagnosed at age 15-19). Overall 5-year survival was 54% for malignancies diagnosed in 1978-1981, 60% for the period 1982-1985; and 69% for the period 1986-1989. The range among registries of 5-year survival for cases diagnosed in 1986-1989 was 55-78%. Most diagnostic categories presented an improved prognosis for the cases diagnosed more recently. For cases diagnosed in 1986-1989, 5-year survival was: 74% for acute lymphatic leukaemia, 40% for acute non-lymphatic leukaemia, 65% for central nervous system neoplasms (76% for astrocytoma, 75% for ependymoma and 85% for medulloblastoma), 66% for osteosarcoma, 55% for Ewing's sarcoma, 87% for Hodgkin's disease, 64% for non-Hodgkin's lymphoma, 74% for rhabdomyosarcoma, 64% for neuroblastoma, 78% for nephroblastoma and 100% for retinoblastoma. Italian survival was similar to that observed in other population-based surveys in the UK and USA.
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PMID:Survival of childhood cancer patients in Italy, 1978-1989. ITACARE Working Group. 915 68

Multidrug resistance protein (MRP), like P170, confers multidrug resistance, but its clinical relevance is uncertain, whereas P170 is an accepted cause of chemotherapy failure for which ongoing reversal trials are being conducted. Because such trials have been only modestly successful, we must investigate alternative drug resistance mechanisms such as MRP, which is poorly blocked by P170 inhibitors. The significance of MRP has remained undefined because MRP mRNA is difficult to assay in archival material, does not necessarily reflect MRP levels, and is widely expressed in normal or hematopoietic cells within tumors and bone marrow. Because conventional immunoblot or immunocytochemistry may not be sensitive enough to detect low or heterogeneous MRP expression in clinical samples, we elected to score MRP in single tumor cells by modifying our P170 assays that have proven valuable for correlating P170 expression with the outcome of pediatric cancer chemotherapy. We enhanced the signal-to-noise ratio with several peroxidase-tagged secondary antibody layers and staining refinements, standardizing the assay with MRP-negative and MRP-positive but P170-negative transfected or drug-selected controls in which MRP was quantified by immunoblot. We confirmed sensitivity by staining a very low MRP-expressing revertant line and "mixed" samples containing small numbers of positive cells; we confirmed specificity by applying two antibodies directed against separate MRP epitopes. We examined neuroblastoma, osteosarcoma, rhabdomyosarcoma, and retinoblastoma samples, identifying MRP-positive malignant cells, which were distinguishable from MRP-positive normal cells. This assay may be valuable for early diagnosis of low but potentially important MRP expression, which would allow timely application of alternative therapy, perhaps with MRP-specific blockers.
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PMID:Standardization of a single-cell assay for sensitive detection of multidrug resistance protein expression in normal and malignant cells in archival clinical samples. 934 90

The authors present data on problems associated with the diagnosis of orbital rhabdomyosarcoma in children. They analyze a ten-year period during which they verified in a department of child ophthalmology the disease in four children. The presence of the tumour is one per 60,000 out-patient examinations and during the follow-up period it had a seven times rarer incidence than retinoblastoma. The authors emphasize the varied clinical picture of the tumour and its differential diagnostic pitfalls and the possibility of mistaking temporarily the tumour for inflammatory or post-traumatic orbital changes. The authors remind of the necessity to consider the possible presence of rhabdomyosarcoma in all rapidly growing tumourous orbital processes, in particular in young children.
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PMID:[Clinical diagnosis of orbital rhabdomyosarcoma in a child]. 937 12


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