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At least 1,063 records · Page 59Linked to original sources
PubMed1984

Sonographic evaluation of pediatric rhabdomyosarcomas.

The ultrasound examinations of ten pediatric patients with retroperitoneal or pelvic embryonal rhabdomyosarcomas were reviewed. A pattern of coarse low-, medium-, or high-level echoes was observed in these tumors. Sonolucent areas, probably secondary to necrosis or hemorrhage, were seen in the tumors of five patients. Serial examinations were performed during the course of chemotherapy and/or radiotherapy in seven patients. These studies were examined retrospectively to determine whether a specific change in the echo characteristics of the tumor occurred during therapy. No such specific change was observed in these patients. Ultrasound was useful in following tumor size and thus, response to therapy, which is important with the advent of newer, more conservative forms of therapy for rhabdomyosarcoma.

Adolescent↗
PubMed1999

Rapamycin causes poorly reversible inhibition of mTOR and induces p53-independent apoptosis in human rhabdomyosarcoma cells.

The mammalian target of rapamycin (mTOR) has been shown to link growth factor signaling and posttranscriptional control of translation of proteins that are frequently involved in cell cycle progression. However, the role of this pathway in cell survival has not been demonstrated. Here, we report that rapamycin, a specific inhibitor of mTOR kinase, induces G1 cell cycle arrest and apoptosis in two rhabdomyosarcoma cell lines (Rh1 and Rh30) under conditions of autocrine cell growth. To examine the kinetics of rapamycin action, we next determined the rapamycin sensitivity of rhabdomyosarcoma cells exposed briefly (1 h) or continuously (6 days). Results demonstrate that Rh1 and Rh30 cells were equally sensitive to rapamycin-induced growth arrest and apoptosis under either condition. Apoptosis was detected between 24 and 144 h of exposure to rapamycin. Both cell lines have mutant p53; hence, rapamycin-induced apoptosis appears to be a p53-independent process. To determine whether induction of apoptosis by rapamycin was specifically due to inhibition of mTOR signaling, we engineered Rh1 and Rh30 clones to stably express a mutant form of mTOR that was resistant to rapamycin (Ser2035-->Ile; designated mTOR-rr). Rh1 and Rh30 mTOR-rr clones were highly resistant (>3000-fold) to both growth inhibition and apoptosis induced by rapamycin. These results are the first to indicate that rapamycin-induced apoptosis is mediated by inhibition of mTOR. Exogenous insulin-like growth factor (IGF)-I protected both Rh1 and Rh30 from apoptosis, without reactivating ribosomal p70 S6 kinase (p70S6K) downstream of mTOR. However, in rapamycin-treated cultures, the response to IGF-I differed between the cell lines: Rh1 cells proliferated normally, whereas Rh30 cells remained arrested in G1 phase but viable. Rapamycin is known to inhibit synthesis of specific proteins but did not inhibit synthesis or alter the levels of mTOR. To examine the rate at which the mTOR pathway recovered, the ability of IGF-I to stimulate p70S6K activity was followed in cells treated for 1 h with rapamycin and then allowed to recover in medium containing > or =100-fold excess of FK506 (to prevent rapamycin from rebinding to its cytosolic receptor FKBP-12). Our results indicate that, in Rh1 cells, rapamycin dissociates relatively slowly from FKBP-12, with a t1/2 of approximately 17.5 h. in the presence of FK506, whereas there was no recovery of p70S6K activity in the absence of this competitor. This was of interest because rapamycin was relatively unstable under conditions of cell culture having a biological t1/2 of approximately 9.9 h. These results help to explain why cells are sensitive following short exposures to rapamycin and may be useful in guiding the use of rapamycin analogues that are entering clinical trials as novel antitumor agents.

Apoptosis↗
PubMed1999

[Embryonal rhabdomyosarcoma in a lamb of the Texel breed].

The subject presented here is a hitherto undescribed case of embryonic rhabdomyosarcoma found in one particular lamb. The neoplasm was localized in the left hemisphere of the head and exited in the regio supraorbitalis. After anatomical and histopathological examination of the tumor, together with immunochemistry tests, an embryonic rhabdomyosarcoma could be positively diagnosed. This is the main type of tumor that occurs in the soft tissue of children, but its appearance in other species has until now not been extensively documented.

Animals↗
PubMedDate not supplied

[Atypical biological behavior of alveolar rhabdomyosarcoma in five patients].

Five cases of alveolar rhabdomyosarcoma with atypical clinical features are reported. Three patients showed lymphadenopathy as the first clinical manifestation, mimicking a lymphoma or a non identified primary tumor with lymph node metastases. One patient presented systemic neoplastic disease and two had the primary tumor in atypical locations, such as the mediastinum and retroperitoneum. All patients died and in four of them an autopsy was performed. The histological diagnosis was confirmed by immunohistochemical studies on four cases. The alveolar rhabdomyosarcoma has a poor prognosis and can have a variable clinical presentation and morphology, simulating lymphomas, leukemias and systemic metastatic disease with an unknown primary neoplasm, such as in the cases here in reported.

Adolescent↗
PubMed1999

[Paratesticular rhabdomyosarcoma].

We report a case of paratesticular rhabdomyosarcoma in a six-year-old boy. The clinical presentation was subacute. The patient underwent a radical right inguinal orchidectomy. It was classified in IRS-III stage IA (based in the Third Intergroup Rhabdomyosarcoma Study). Subsequently, the child received 3 chemotherapy courses (9 weeks) with vincristine and actinomycin D. The patient is found to be asymptomatic 1 year after the treatment.

Antibiotics, Antineoplastic↗
PubMed1999

[Rhabdomyosarcoma of the middle ear in childhood].

The authors have described a case of a 2.5-year-old boy who suffered from rhabdomyosarcoma of the middle ear. The clinical course, differential histopathology diagnosis and dynamic growth of the tumor have also been presented. In spite of combined therapy--surgery and chemotherapy--prognosis in rhabdomyosarcoma of the middle ear in childhood is poor.

Child, Preschool↗
PubMed2000

[Primary pulmonary rhabdomyosarcoma: a case report].

We report a case of primary pulmonary rhabdomyosarcoma in a 52-year-old woman. The diagnosis was established after radical right pneumonectomy, mainly based on immunohistochemistry and electron microscopy findings. This type of tumor is rare in adults. Its primary origin is suggested when a pulmonary meta-stasis of rhabdomyosarcoma from another site or a component of a mixed tumor are ruled out. The best treatment is surgery. The prognosis is poor.

Adult↗
PubMed2000

[Primary intraosseous rhabdomyosarcoma].

We report a case of primary intraosseous pleiomorphic rhabdomyosarcoma located in the pelvis of a 21-year-old woman followed for 4 years. The lytic tumor involved the acetabulum and the isthma with moderate extension to soft tissue. First line chemotherapy was unable to arrest tumor progression. Hemipelvectomy with saddle prosthesis reconstruction was performed, but septic complications dictated a secondary inter-ilio-abdominal amputation. Recurrence-free remission was achieved for 4 years, suggesting this was indeed a primary tumor. Primary intraosseous rhabdomyosarcomas are exceptional. Bone localizations generally suggest metastasis from a primary tumor often situated in an intraperitoneal localization. When search for a primary tumor is negative, intraosseous lesions can be considered as primary tumors warranting curative treatment. Radical surgical resection is recommended within the framework of a multidiscipinary management protocol associating radiotherapy and chemotherapy to improve prognosis.

Adult↗
PubMed2000

[Rhabdomyosarcoma at site of pacemaker implantation].

BACKGROUND: Malignant proliferation at the site of implantation of a pacemaker generator is uncommon. We report the case of a patient who developed rhabdomyosarcoma. CASE REPORT: A 85-year-old man presented with a voluminous and rapidly evolving tumor localised beneath the right clavicle. This inflammatory and necrotic lesion developed on the area where a titanium pacemaker had been implanted five years earlier. Rhabdomyosarcoma was diagnosis on the basis of immunohistochemistry findings. In spite of a wide surgical excision of this primitive tumour, visceral dissemination appeared, rapidly leading to the patient's death. DISCUSSION: This observation rises the question of the role of the pacemaker implantation in tumor development. The excellent in vivo tolerance and the widespread utilization of titanium as biomaterial is an argument against its carcinogenic action. Inversely, a metal-related chronic inflammatory reaction could favor the neoplastic process in predisposed subjects as has been observed with prosthetic materials used in orthopaedics.

Aged↗
PubMed2000

[Diagnosis and therapy of rhabdomyosarcoma in children].

An analysis of 24-month relapse-free survival in 62 patients with rhabdomyosarcoma (S.I.O.P.-89) is presented. All children with rhabdomyosarcoma (RMS) stage I have survived; stage II--90, and stage III--62%, i.e. twice as many as compared with those treated before 1993. RMS sites included retroperitoneal space, spatium perinei, urinary bladder, testicle, abdominal and thoracic cavity. Diagnosis was based on clinical, instrumental, laboratory, X-ray, radionuclide, ultrasound and morphological data. Recommendations for examination of RMS suspects were worked out for different levels of expertise.

Child↗
PubMed2000

Embryonal rhabdomyosarcoma of prostate in an adult--a diagnostic dilemma.

Embryonal rhabdomyosarcoma of the prostate is a rare. Highly malignant tumour. The median age of occurrence is five years, but sporadic cases have been reported in adults' To the best of our knowledge, till date, fewer than ten cases have been reported of which only two are above the age of sixty years. We report a case of embryonal rhabdomyosarcoma of prostate in a patient more than sixty years of age. If one is not aware of this entity, one can make a mistake in the diagnosis as well as treatment.

Aged↗
PubMed2001

Solid variant of alveolar rhabdomyosarcoma with unbalanced t(2;13) and hypotetraploidy, without MYCN amplification.

The histological subtype of alveolar rhabdomyosarcoma (AR) is characterised by the cytogenetic translocation t(2;13)(q35;q14) in approximately 70% of cases, a rearrangement rarely present in the embryonal rhabdomyosarcoma (ER) subtype. The MYCN gene is amplified in some cases of AR. We present a young man with an unusual pattern, namely solid variant of AR with hypotetraploidy and the t(2;13) in an unbalanced form. The MYCN gene was not amplified on FISH, but showed increased copy number, consistent with ploidy.

Adult↗
PubMed2001

What constitutes optimal therapy for patients with rhabdomyosarcoma of the female genital tract?

BACKGROUND: Factors affecting outcome for rhabdomyosarcoma (RMS) of the female genital tract in patients treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols I-IV were evaluated to define optimal therapy. METHODS: Records of 151 patients with tumors of the female genital tract who were treated on IRSG protocols I-IV were reviewed for details regarding chemotherapy, surgery, radiotherapy (RT), and outcome. RESULTS: The overall 5-year survival was 82%, (87% for patients with locoregional tumors). Chemotherapy was primarily vincristine, actinomycin-D, and cyclophosphamide (VAC) based. Local therapy was surgery alone in 42% of patients, surgery plus RT in 19% of patients, biopsy plus RT in 12% of patients, and biopsy without RT in 21% of patients. The rate of hysterectomy decreased from 48% in IRS-I/II to 22% in IRS-III/IV with an increase in the use of RT from 23% in IRS-II to 45% in IRS-IV and continued excellent survival. Many patients with vaginal primary tumors received delayed RT or had it omitted on later studies with excellent outcome. For patients with localized embryonal/botryoid tumors, there were no significant differences in 5-year survival among patients with tumors at different sites or among patients treated on IRS-I-IV. In patients with Group I-III tumors, 43% of deaths were from toxicity. Analysis of prognostic factors, with toxic deaths censored, revealed that an age of 1-9 years at the time of diagnosis, noninvasive tumors, and the use of IRS-II or IRS-IV treatments were associated significantly with better outcome. Patients ages 1-9 years fared best (5-year survival of 98%) and patients outside of this age range especially benefited from the intensified therapy used in IRS-III or IRS-IV (5-year survival of 67% on the IRS-I/II vs. 90% in IRS-III/IV). CONCLUSIONS: Localized female genital RMS usually is curable with combination chemotherapy, a conservative surgical approach, and the use of RT for selected patients.

Adolescent↗
PubMed2001

In vitro prevention of the emergence of multidrug resistance in a pediatric rhabdomyosarcoma cell line.

We have established preclinical models for the development of drug resistance to vincristine (a major drug used in the treatment of pediatric rhabdomyosarcoma) using cell lines. The RD cell line has a mutant P53 phenotype and does not have detectable P-glycoprotein (P-gp) or multidrug resistance-related protein (MRP) despite expressing low levels of mdr-1 mRNA, which encodes P-gp and mrp1 mRNA. Resistant variants of RD were derived by exposure to increasing concentrations of vincristine. This was repeated on six occasions, resulting in three cell lines which could tolerate 64 x the IC(50) concentration. Six independent agents were tested for their ability to prevent the development of resistance in this model. Despite at least 10 attempts, resistance did not develop in the presence of the multidrug resistance (MDR) modulators PSC833, VX710, and XR9576. This strongly suggests that these agents may delay or even prevent the development of resistance to vincristine. This was also confirmed in a second rhabdomyosarcoma cell line, Rh30. In contrast, the agents indomethacin (MRP1 modulator), CGP41251 (protein kinase C inhibitor), and dexrazoxane (putative MDR prevention agent) did not affect the development of resistance in the RD model. Characterization of the resistant cell lines indicated the presence of increased mdr-1 and P-gp expression, which resulted in resistance to the agents doxorubicin, etoposide, and vincristine but not cisplatin. The resistance could be modulated using PSC833 or VX710, confirming that functional P-gp is present. No apparent differences were seen between the resistant cell lines derived in the absence and presence of the various agents. These experiments strongly suggest that the development of MDR may be preventable using modulators of MDR and merit clinical studies to test this hypothesis.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed1975

Growth of rhabdomyosarcoma colonies from pleural fluid.

Pleural fluid from a child previously treated for rhabdomyosarcoma produced colonies in vitro. Cells from these colonies appeared to have the light and electron microscopic appearance of rhabdomyosarcoma cells. In this case, the malignant nature of the effusion had been suspected because of the patient's previous history; however, this technique may prove useful in the diagnosis of effusion of unknown etiology.

Animals↗
PubMedDate not supplied

Intraperitoneal involvement in rhabdomyosarcoma CT findings in a child.

Intraperitoneal neoplastic involvement in rhabdomyosarcoma is rare and its incidence and imaging characteristics need to be further described. We present the computerized tomography (CT) findings of a case with pelvic rhabdomyosarcoma and intraperitoneal neoplastic involvement. Enhanced peritoneal and retroperitoneal masses were seen around the liver, spleen, in the paracolic gutters, and in the lesser sac without evidence of ascites, mesenteric nodules or omental caking. Our case also showed that absence of ascites does not preclude the presence of peritoneal involvement. Progression in the peritoneal disease was also well demonstrated by CT.

Adolescent↗
PubMed2002

Usefulness of immunohistochemical testing and electron microscopy in the diagnosis of embryonal rhabdomyosarcoma.

BACKGROUND: This article presents the results of histological, immunohistochemical, and ultrastructural examinations of embryonal rhabdomyosarcoma. MATERIAL/METHODS: 20 tumors were tested histologically, 13 immunohistochemically, and 5 under electron microscopy. RESULTS: We found that in cases when the entire tumor or large specimens were obtained for estimation, it was possible by examining many areas of the neoplasm to establish a diagnosis on the basis of testing paraffined sections, routinely HE stained. Our evaluation of the value of certain additional histological methods indicated only slight diagnostic usefulness for tumors of this kind. Among the immunohistochemical methods we evaluated, the desmin assay had the greatest practical applicability, due to the fact that this antigen is also expressed in highly immature cells; in second place was myoglobin. Differences are pointed out in the results obtained by assaying myoglobin using DAB and the DAKO kit. Ultrastructural examinations revealed myofilaments of myosin and actin in all types of cells, including immature cells. Particular attention is drawn to the possibility of using material fixed in formalin for ultrastructural examination. The preservation of intact myofilaments in material prepared in this way constitutes an essential diagnostic aid. CONCLUSIONS: In diagnosing embryonal rhabdomyosarcoma the most useful examination, apart from histological examination, is electron microscopy, followed by immunomorphological examination.

Adolescent↗
PubMed2003

[Influences of PAX3-FKHR transfection on expression of MRF4 and differentiation of cultured human rhabdomyosarcoma RD cells].

BACKGROUND & OBJECTIVE: There were a few reports about the role of PAX3-FKHR fusion gene in the oncogenesis of alveolar rhabdomyosarcoma (ARMS); however, its effect on muscle-producing factor (MPF) is not clear. This study was designed to observe the effects of fusion gene PAX3-FKHR transfection on expression of MRF4 and differentiation of cultured human rhabdomyosarcoma RD cells. METHODS: To construct an eukaryotic expression vector of PAX3-FKHR gene; the plasmids of MRF4 cDNA and PAX3-FKHR cDNA were transfected into cultured RD cells with lipofectin methods. The changes of cells growth, morphologic and the expression of MHC and alpha-actin were observed. RESULTS: There was no significant changes in RD cells after transfection with PAX3-FKHR gene in growth rate, growth manner and the expression of MHC and alpha-actin. In MRF4 expressing RD cells after transfected with PAX3-FKHR, although the expression of MRF4 was not changed, morphologic appearance showed a low differentiation; expression of MHC and alpha-actin significantly decreased. CONCLUSION: There is no obvious influence on biological character and differentiation in RD cells with transfection of PAX3-FKHR gene; PAX3-FKHR does not inhibit the expression of MRF4, but can reverse the effect of MRF4 on promoting the differentiation of RD cell.

Actins↗