Search results for "Rhabdomyosarcoma"
showing 10 items of 58 documents
Rhabdomyosarcoma of the Oral Tissues: two new cases and literature review
2006
Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm consisting of cells derived from the primitive mesenchyme that exhibit a profound tendency to myogenesis. About 35% of RMS arises in the head and neck, being classified as parameningeal and non-orbital non-parameningeal forms. Parameningeal tumors carry the worst prognosis. The use of contemporary, multi-agent chemotherapy, radiotherapy, and surgery has made treatment of the disseminated disease possible, and has significantly improved overall survival from 25% in 1970 to 70% in 1991. Here, we present the management of two cases of orofacial RMS in adolescents: an 18-year-old, white female that had a 9-month history of a nodule in t…
Fetal rhabdomyomatous nephroblastoma. Pathologic histology and special clinical and biologic features.
1980
Fetal rhabdomyomatous nephroblastoma is a very rare variant of Wilms' tumor. The special clinical and histologic features of this variant are presented on the basis of a case seen in a boy who was 13 months old at nephrectomy 8 months ago and has remained healthy since then. The tumor chiefly consists of fetal striated muscle; it occurs predominantly in infancy and early childhood and is often bilateral. Angiography shows a relative paucity of vessels. The volume of the tumor (determined by ultrasonography) decreases only slightly after preoperative radio- and chemotherapy because of a predominance of mesenchymal structures. The finding of nodular renal blastema in renal parenchyma with an …
Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery
2003
Rhabdomyosarcoma of pelvic organs is not common enough for many people to develop large series. However, the authors from Mainz retrospectively analysed 107 children with this condition, and suggest that primary chemotherapy followed by radical surgery yields excellent cure rates. OBJECTIVE To retrospectively analyse the outcome of children with rhabdomyosarcoma (RMS) of the bladder, prostate or vagina who were treated with chemotherapy, with or without radical surgery or additional radiotherapy, at our institution since 1968. PATIENTS AND METHODS From a total of 107 children with RMS seen between 1968 and December 2001, 22 (mean age 5.9, range 0.5–18) had RMS of bladder/prostate or vagina.…
Childhood soft tissue sarcomas incidence and survival in European children (1978-1997): Report from the Automated Childhood Cancer Information System…
2006
This population-based study is based on 5802 cases of soft tissue sarcomas (STS) in children aged 0-14 years extracted from the database of the Automated Childhood Cancer Information System (ACCIS) and registered in population-based cancer registries in Europe for the period 1978-1997. STS represent almost 8% of neoplasms in children, almost half of whom are less than 5 years at diagnosis. Rhabdomyosarcoma is the most frequent childhood STS (50%). During 1988-1997 the age-standardised incidence of STS in Europe was 9.1 per million children, lowest in the West and East and highest in the North. The incidence of STS increased almost 2% per year over the period 1978-1997, attributable mostly t…
PROX1 transcription factor controls rhabdomyosarcoma growth, stemness, myogenic properties and therapeutic targets
2022
Funding Information: ACKNOWLEDGMENTS. We would like to thank Dr. Tuomas Tammela and Dr. Monika Ehnmann for providing RMS cell lines and Dr. Jenny Högström for discussions and comments during the project. Kirsi Mattinen, Jefim Brodkin, Maxime Laird, Manon Gruchet, Ilse Paetau, Tanja Laakkonen, and Tapio Tainola are acknowledged for their excellent technical help. We also thank the Laboratory Animal Center at the University of Helsinki for expert animal care, the Biomedicum Imaging Unit for microscope support, the Biomedicum Functional Genomics Unit for the RNAseq experiments and the FIMM Technology Centre High Throughput Biomedicine for the drug sensitivity and resistance testing. Our first …
Terminally differentiated postmitotic tumor cells in a rat rhabdomyosarcoma cell line.
1988
A permanent rat rhabdomyosarcoma cell line (BA-HAN-1C) has been established, the phenotype of which is characterized by the coexistence of undifferentiated mononuclear cells and differentiated multinuclear myotube-like giant cells. The failure of attempts to separate these two cell types by repeated recloning procedures indicates their close histogenetic relationship and suggests that differentiation in this tumor proceeds in a similar manner to that in normal striated muscle where postmitotic myotubes arise from mononuclear myoblasts by fusion. The morphologically undifferentiated mononuclear tumor cells were shown to be actively proliferating and to incorporate thymidine methyl-3H(3H-TdR)…
Initial surgery for localized rhabdomyosarcoma: a report from the italian Soft Tissue Sarcoma Cooperative Group Studies.
2005
Objective. Analysis of the initial surgical approach in patients with localized rhabdomyosarcoma, enrolled in Italian Studies (1979-2003). Methods. Surgical records of 587 patients were evaluated. As primary surgery, the excision was recommended if microscopically complete, otherwise biopsy was preferable. Results. The initial biopsy, performed in almost 50% of patients in RMS-79 protocol, has been adopted more often in the protocol RMS-88 and 96. However, the “complete resection” rate has remained quite stable through the years, as well as the percentage of microscopic residue. The macroscopic residue disease has been more important in RMS-88 study than in RMS-96. Most GUnBP tumors and abo…
Damage to the urinary tract secondary to irradiation.
1995
During the past two decades, highly effective multimodality therapy with surgery, chemotherapy and irradiation has been developed through consecutive national and international study protocols for childhood genitourinary cancers, the model being Wilms' tumor. These studies represent a landmark achievement in the history of pediatric oncology and mark the success of multi-institutional efforts. Now that excellent survival rates have been established, current interest is now directed primarily at examination of survivors for long-term treatment complications and minimizing the side effects while preserving treatment efficacy. Treatment sequelae may not become evident until many years after th…
Morphological, biochemical, and molecular biological characterization of a rat rhabdomyosarcoma cell line during differentiation induction in vitro.
1990
BA-HAN-1C is a clonal rat rhabdomyosarcoma cell line consisting of proliferating mononuclear tumor cells, some of which spontaneously fuse to form terminally differentiated postmitotic myotubelike giant cells. Exposure to retinoic acid resulted in an inhibition of proliferation and a marked increase in cellular differentiation. The number of myotubelike giant cells significantly increased, and about 30% of the mononuclear tumor cells exhibited morphological features of rhabdomyogenic differentiation which were not observed in the mononuclear cells of untreated cultures. Morphological differentiation was paralleled by an increase in total creatine kinase activity as a biochemical marker of d…
Cytogenetic and molecular findings related to rhabdomyosarcoma. An analysis of seven cases.
2003
Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in childhood. Histologically, it is subdivided histologically into two main subtypes: alveolar (ARMS) and embryonal (ERMS). ARMS is characterized by t(2;13)(q35;q14) or its variant t(1;13)(p36;q14), which fuse PAX3 and PAX7, respectively, with FKHR to produce chimeric genes. ERMS is frequently associated with loss of heterozygosity of 11p15.5. We investigated seven RMS (three ARMS and four ERMS) by means of cytogenetic, fluorescence in situ hybridization, and molecular analyses, including the study of the main genes implicated in the G1- to S-phase cell cycle transition, and correlated these studies with pathologic findings and c…