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At least 919 records · Page 51Linked to original sources
PubMed2001

Localized paediatric orbital rhabdomyosarcoma: influence of imaging on treatment.

AIM: Orbital rhabdomyosarcoma is the most common primary malignant orbital tumour in children and has a good prognosis. The purpose of this paper was to review the imaging and consequent treatment of patients with localized orbital rhabdomyosarcoma from around the U.K. MATERIALS AND METHODS: Patients were identified through the U.K. Children's Cancer Study Group (UKCCSG) database. Investigations and therapy were dictated by the Malignant Mesenchymal Tumour '89 (MMT89) protocol. Imaging and radiological reports of 16 patients from 12 centres were reviewed. The number of patients receiving radiotherapy, timing of radiotherapy, and adherence to treatment protocols were assessed. RESULTS: Local radiologists' reports and imaging techniques varied between sequential examinations and centres. The imaging was adequate for management. No reports quoted measurements of the tumours. Treatment protocols were not always followed rigidly with regard to a residual mass at day 80 post-diagnosis. However, the protocol was not explicit for all outcomes. Fifteen out of 16 patients eventually received radiotherapy. CONCLUSION: There is no standardization of imaging between centres. The presence or absence of a post-therapeutic residue should be stated in the radiology report. Further investigation is needed to differentiate between fibrosis and recurrent tumour. Radiotherapy for residual mass at day 80 is probably more important than standardizing radiological technique.

Adolescent↗
PubMed2002

Pleomorphic rhabdomyosarcoma in an adult forearm: a case report.

Pleomorphic rhabdomyosarcoma (RMS) is a rare malignancy, and treatment protocols for this tumor are not established. Although Intergroup Rhabdomyosarcoma Study data have led to improvement in overall and disease-free survival for children with juvenile RMS, the applicability of those findings to adult pleomorphic RMS is uncertain. This report presents the case of a 29-year-old man with pleomorphic RMS of the forearm (a location not previously reported in current literature); reviews the immunohistochemical, ultrastructural, and histologic diagnostic criteria; and discusses treatment.

Adult↗
PubMed2000

Paratesticular rhabdomyosarcoma in a neonate.

A 13-day-old boy presented with left scrotal tumor and coronary hypospadias. Left radical orchiectomy was performed. Histological diagnosis was embryonal-type paratesticular rhabdomyosarcoma. To the authors' knowledge, this is the first reported case of paratesticular rhabdomyosarcoma in a neonate in English-language literature. Also, the association of a testicular tumor with hypospadias has not been noted.

Humans↗
PubMed2002

Vaginal reconstruction in a patient with rhabdomyosarcoma previously treated by total vaginectomy.

In vaginal rhabdomyosarcoma, because of effectiveness of multiple-agent chemotherapy and brachytherapy, function-preserving treatment without radical surgery has been recently recommended. However, for patients with persistent or recurrent tumors, vaginectomy with or without hysterectomy is appropriate. The authors report experience of successful vaginal reconstruction in a rhabdomyosarcoma patient who had total vaginectomy with preservation of the uterus. Because continuity from the vaginal introitus to the uterus was established, fertility can be expected for this patient.

Child↗
PubMed1999

Congenital rhabdomyosarcoma.

A congenital rhabdomyosarcoma presented as a partially necrotic mass on the left forearm on delivery at term. Ulceration and persistent bleeding were managed by primary curettage followed by local resection including partial excision of the muscles of the extensor compartment of the forearm. The defect was resurfaced with a split skin graft. The surgery was followed by chemotherapy according to the IVA regime. There was no recurrence at 2 years of age and the limb was fully functional. The presentation and management of rhabdomyosarcoma are discussed.

Female↗
PubMed2006

Alveolar rhabdomyosarcoma of the clivus with intrasellar expansion: Case report.

Rhabdomyosarcomas are common tumors of the head and neck region in children. However, a primarily intracranial localization of this tumor entity is rare. We report on a 3-year-old boy presenting with double vision due to left VI (th) nerve palsy. No other neurological deficits were recognized by clinical inspection. MRI scans visualized an enhancing mass lesion in the upper clivus compressing the cavernous sinus and the pituitary gland. Transsphenoidal biopsy was performed and histopathological examination as well as molecular diagnostics confirmed the diagnosis of an alveolar rhabdomyosarcoma (ARMS). Staging identified a metastatic lesion in the fourth thoracic vertebra resulting in the diagnosis of stage IV disease. Treatment modality included stereotactic radio- and chemotherapy.

Abducens Nerve Diseases↗
PubMed1986

Rhabdomyosarcoma of the diaphragm in a 4-year-old girl.

Rhabdomyosarcoma of the diaphragm is a very rare entity, especially in childhood. A case of a 4-year-old girl is reported. The mass extended completely into the abdominal cavity, causing problems in differential diagnosis with other abdominal masses. Dimensions, structure and limits of the mass had been outlined by several examinations (IVP, sonography, CT, angiography), but its actual nature could not be shown, so that a surgical exploration was needed. A pedunculated, well-capsulated mass, arising from the left diaphragmatic portion, was found. A complete excision could be performed and histologic findings were compatible with rhabdomyosarcoma of the diaphragm.

Abdominal Neoplasms↗
PubMed1992

[Neoplastic space-occupying lesions of the orbit. I. Review; hemangioma, lymphangioma and embryonal rhabdomyosarcoma].

From 1987 to 1990 182 patients were treated for orbital lesions in the Department of Ophthalmology of the University of Zürich. 37% were tumors (without tumors of the vascular system), 20% inflammatory lesions, 12% lesions of the vascular system and tumors of it, the rest was not diagnosed. On this background the first of two chapters on orbital tumors deals with diagnosis, differential diagnosis, therapy and follow up of the capillary hemangioma, cavernous hemangioma, lymphangioma and embryonal rhabdomyosarcoma. Although ultrasound echography, CT, MRI and angiography have greatly changed the diagnostic work up of orbital lesions, diagnosis is by far not made straight forward but often only after errors. To enhance diagnosis in the future this study is meant to analyse our misinterpretations. In the case of capillary hemangioma only complications justify an active treatment, normally the lesion resolves spontaneously. The infiltration of the upper eyelid with ptosis needs to be treated because of the risk of amblyopia. Our cases demonstrate, that the attempt of treatment with steroids is justified. Embolisation in the region of the a. ophthalmica is quite dangerous. The cavernous hemangioma only needs treatment in the case of compression of the optical nerve and total excision is not mandatory. The lymphangioma, although classified as benign tumor, can only be excised subtotally and with the danger of traumatising important structures of the orbit. The infiltrative growth and tendency of recurrence in fact is dangerous. It is important to diagnose the tumor without biopsy and to wait with surgical treatment as long as possible. The embryonal rhabdomyosarcoma itself is rare but it is the most frequent primary malignant tumor in childhood. Even accounting for being familiar with mimikri-false history of trauma, inflammation, hemangioma-like angiography-the diagnosis is often made rather late. Our cases demonstrate this and also the change of treatment in the last 30 years. Unfortunately the prognosis of the good results of chemotherapy in combination with radiation if necessary is clouded by the occurrence of secondary malignant tumors.

Adolescent↗
PubMed2006

A Drosophila model of the rhabdomyosarcoma initiator PAX7-FKHR.

Alveolar rhabdomyosarcoma (ARMS) is an aggressive myogenic-type tumor and a gain-of-function disease, caused by misexpression of the PAX3-FKHR or PAX7-FKHR fusion oncoprotein from structurally rearranged chromosomes. PAX3-FKHR misexpressed in terminally differentiating mouse myofibers can cause rhabdomyosarcoma at a low frequency, suggesting that skeletal muscle is an ARMS tissue of origin. Because patterned muscle is widely viewed as irreversibly syncytial, questions persist, however, regarding this potential pathogenetic mechanism for ARMS tumor initiation. To further explore this issue, we generated transgenic Drosophila lines that conditionally express human PAX-FKHR. Here we show that PAX7-FKHR causes nucleated cells to form and separate from syncytial myofibers, which then spread to nonmuscular tissue compartments, including the central nervous system, and that wild-type PAX3 demonstrates similar potential. We further show that Ras, which is known to interfere with the differentiation of myogenic cells, genetically interacts with PAX7-FKHR: constitutively activated Ras enhances PAX7-FKHR phenotypes, whereas loss-of-function ras alleles dominantly suppress PAX7-FKHR activity, including rescue of lethality. These results show that PAX-FKHR can drive the generation of discrete nucleated cells from differentiated myofibers in vivo, argue for syncytial muscle as an ARMS tissue of origin, and demonstrate that Drosophila provides a powerful system to screen for genetic modifiers of PAX-FKHR.

Animals↗
PubMed1997

Fusion genes resulting from alternative chromosomal translocations are overexpressed by gene-specific mechanisms in alveolar rhabdomyosarcoma.

Chromosomal translocations identified in hematopoietic and solid tumors result in deregulated expression of protooncogenes or creation of chimeric proteins with tumorigenic potential. In the pediatric solid tumor alveolar rhabdomyosarcoma, a consistent t(2;13)(q35;q14) or variant t(1;13)(p36;q14) translocation generates PAX3-FKHR or PAX7-FKHR fusion proteins, respectively. In this report, we demonstrate that in addition to functional alterations these translocations are associated with fusion product overexpression. Furthermore, PAX3-FKHR and PAX7-FKHR overexpression occurs by distinct mechanisms. Transcription of PAX3-FKHR is increased relative to wild-type PAX3 by a copy number-independent process. In contrast, PAX7-FKHR overexpression results from fusion gene amplification. Thus, gene-specific mechanisms were selected to overexpress PAX3-FKHR and PAX7-FKHR in alveolar rhabdomyosarcoma, presumably due to differences in regulation between the wild-type loci. We postulate that these overexpression mechanisms ensure a critical level of gene product for the oncogenic effects of these fusions.

Chromosomes, Human, Pair 1↗
PubMed2000

The insulin-like growth factor-phosphatidylinositol 3-kinase-Akt signaling pathway regulates myogenin expression in normal myogenic cells but not in rhabdomyosarcoma-derived RD cells.

Insulin-like growth factors (IGFs) can stimulate skeletal muscle differentiation. One of the molecular mechanisms underlying IGF-stimulated myogenesis is transcriptional induction of myogenin. The current work is aimed to elucidate the signaling pathways mediating the IGF effect on myogenin promoter in mouse C2C12 myogenic cells. We show that phosphatidylinositol 3-kinase (PI3K)/Akt and p70(S6K) are crucial signaling molecules mediating the stimulatory effect of IGFs on myogenin expression. We have identified three cis-elements, namely the E box, MEF2, and MEF3 sites, within the 133-base pair mouse proximal myogenin promoter that are under the control of the IGF/PI3K/Akt pathway. Simultaneous mutation of all three elements completely abolishes activation of the myogenin promoter by PI3K/Akt. We demonstrate that PI3K/Akt can increase both the MyoD and the MEF2-dependent reporter activity by enhancing the transcriptional activity of MyoD and MEF2. Interestingly, IGF1 does not enhance myogenin expression in Rhabdomyosarcoma-derived RD cells. Consistently, the constitutively active PI3K/Akt fail to activate the myogenic reporters, suggesting the IGF/PI3K/Akt pathway is defective in RD cells and the defect(s) is downstream to PI3K/Akt. This is the first time that a defect in the IGF/PI3K/Akt pathway has been revealed in RD cells which provides another clue to future therapeutic treatment of Rhabdomyosarcoma.

Amino Acid Sequence↗
PubMedDate not supplied

Primary pulmonary rhabdomyosarcoma: report of a case in an adult and review of the literature.

A case of primary pulmonary rhabdomyosarcoma occurring in a 62-year-old man is reported, and a review of the literature is presented. The tumor affected the left upper lobe and involved the mediastinal lymph nodes. Immunohistochemical and ultrastructural studies supported the myogenic phenotype of the neoplasm. A left pneumonectomy was performed with complete surgical removal of the tumor. Postoperative radiotherapy was carried out. The patient is currently alive and free of disease 9 months after operation. Despite the rarity of primary pulmonary rhabdomyosarcoma, this tumor should be differentiated from other poorly differentiated pulmonary neoplasms and from metastatic sarcomas.

Humans↗
PubMed2002

Spindle cell lesions of the head and neck mimicking rhabdomyosarcoma in children.

Malignancies of the head and neck are uncommon among children. The most common solid tumors to occur in this region are rhabdomyosarcoma, Hodgkin disease, and non-Hodgkin lymphoma. Two children are described who presented with signs and symptoms consistent with rhabdomyosarcoma, but who were found to have benign spindle cell lesions. These rare lesions should be part of the differential of children with a head and neck mass.

Child↗
PubMed2005

Poor outcome of two children with relapsed state stage IV alveolar rhabdomyosarcoma after allogeneic stem cell transplantation.

The authors report on 2 boys, 11(1/2) and 13 years old, who received allogeneic stem cell transplantation (alloSCT) from their HLA-identical sibling after relapse of stage IV alveolar rhabdomyosarcoma. Both patients were transplanted in a non-remission status. After alloSCT both patients experienced disease progression at the primary tumor location sites and died due to the underlying disease 146 and 379 days after transplantation. The authors conclude that an alloSCT derived graft versus tumor effect might not be effective enough to overcome alveolar rhabdomyosarcoma when transplantation is carried out in a nonremission status.

Adolescent↗
PubMed2005

Primary rhabdomyosarcoma of the lung arising over cystic pulmonary adenomatoid malformation.

The authors report the case of a 2-year-old girl with pulmonary cystic adenomatoid malformation type II who presented with a mass on the lower lobe of the left lung. Resection and histological examination revealed pleomorphic rhabdomyosarcoma. Chemotherapy and radiotherapy followed the operation and the girl is alive and in perfect condition 10 years after the operation. The literature on primary rhabdomyosarcoma of the lung in children is reviewed.

Child, Preschool↗
PubMed2005

Dose rate dependence of the relative biological effectiveness of 103Pd for continuous low dose rate irradiation of BA1112 rhabdomyosarcoma cells in vitro relative to acute exposures.

PURPOSE: To measure the relative biological effectiveness (RBE) of continuous low dose rate irradiation (CLDRI) using 103Pd sources relative to acute high dose rate irradiations (AHDRI) from a 250 kVp x-ray beam and an x-ray beam having an equivalent mono-energetic photon energy equal to the average energy of the 103Pd source for BA1112 rhabdomyosarcoma cells. MATERIALS AND METHODS: A customized 103Pd irradiator was built to provide CLDRI using 103Pd at different dose rates relevant to clinical interstitial brachytherapy to BA1112 rhabdomyosarcoma cells growing in exponential phase in culture. A special x-ray beam that simulates the photon energies emitted by the 103Pd source was also developed to provide acute high dose rate irradiation at those energies. Cell survival curves from different irradiation conditions were measured. The RBE with respect to AHDRI using standard 250 kVp x-rays was determined from the doses required to achieve a cell surviving faction of 0.01. RESULTS: For acute irradiation, the RBE of the x-rays simulating (103)Pd was 1.24 relative to 250 kVp x-rays. A profound dose rate effect was observed at low dose rates in the range of 6.8 - 14.4 cGy/h that are typical of permanent interstitial brachytherapy. At cell-surviving fraction of 0.01, the RBE of CLDRI at 6.8 and 14.4 cGy/h using 103Pd sources was reduced by a factor of 3 and 2, respectively, relative to the acute exposure. This observation is in good agreement with recent in vivo tumor cure studies performed on BA1112 tumor. CONCLUSION: The relative biological effectiveness of the photons emitted by 103Pd depends on both the linear energy transfer (LET) of the low energy photons and the dose rate of the irradiation. The higher LET of 103Pd photons is biologically more effective in killing BA1112 tumor cells compared to conventional 250 kVp x-rays when both are delivered at the same dose rate. But the gain in RBE that results from the higher LET can be quickly negated by the reduced dose rate of the irradiation.

Animals↗
PubMed2001

Effect of TNP-470 (AGM-1470) on the growth of rat rhabdomyosarcoma tumors of different sizes.

Potential anticancer therapy with the fumagillin analog TNP-470 was investigated in the present project using subcutaneously growing rhabdomyosarcomas in rats. Specifically, influences of different tumor sizes at the start of treatment as well as dose/schedules were evaluated with this angiogenesis inhibitor. The results show a significant (p = < or = 0.01) reduction of the growth rate, even for relatively large-sized (> 7 cm3) tumors, when 50 mg/kg TNP-470 was used every other day for up to 3 or 5 injections. With 30 mg/kg TNP-470 injections, effects were seen only with tumors measuring < 7 cm3. The histologic examinations demonstrate an increase in necrosis, both in the center and in the peripheral part of TNP-470-treated tumors. Overall, both tumor volume and drug dose determine treatment outcome with the rat rhabdomyosarcoma. The results suggest that angiogenesis inhibitors could represent a valid component in the treatment of progressive tumor growth, also of large tumors as often encountered in clinics. The antivasculature therapy might also improve hypoxia/necrosis-related therapeutic approaches.

Angiogenesis Inhibitors↗
PubMed1998

p53 regulates human insulin-like growth factor II gene expression through active P4 promoter in rhabdomyosarcoma cells.

The developmentally regulated human insulin-like growth factor II (IGFII) gene is expressed at high levels in many types of tumors and promotes the proliferation of tumor cells with a high incidence of p53 gene defects. We have previously shown that p53 inhibits IGFII P3 promoter activity and decreases endogenous IGFII gene expression derived from the P3 promoter in rhabdomyosarcomas by interfering with TBP binding to the TATA element of the IGFII P3 promoter. In this report, we demonstrate that wild-type p53 expression in rhabdomyosarcoma cell lines containing mutant p53 leads to a decrease in the activity of another active IGFII promoter, P4, and a 5-fold reduction of IGFII mRNA derived from the P4 promoter. This inhibition of P4 activity is associated with direct binding of p53 to the P4 proximal promoter element despite the lack of a p53 consensus binding site. Our results suggest that p53 inhibits IGFII P4 promoter activity by a mechanism different than its effect on the P3 promoter. These data also supply further evidence of cross-talk between the IGF and p53 signaling pathways.

DNA, Neoplasm↗