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Results for “RHABDOMYOSARCOMA”

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At least 829 records · Page 46Linked to original sources
PubMed1994

Tissue-specific expression of neural cell adhesion molecule (NCAM) may allow differential diagnosis of neuroblastoma from embryonal rhabdomyosarcoma.

The MSD1 region of neural cell adhesion molecule (NCAM) was originally described as being spliced into the 120-kDa isoform of NCAM isolated from muscle. The 105 bp region is inserted between exons 12 and 13 and actually consists of three separate exons, MSD1a, MSD1b and MSD1c of 15, 48, 42 bp, respectively. In addition, a further exons consisting of a single triplet has been designated MSD1d, making the full insert size 108 bp. As the MSD1 region was originally described as being selectively expressed in muscle tissue, we have investigated whether it is also present on tumours of rhabdoid origins and whether its presence can be used as the diagnostic marker to distinguish other small round cell tumours of childhood, such as neuroblastoma. Using a variety of human tumour cell lines, we demonstrated the presence of the MSD1 region on all rhabdomyosarcomas investigated. However, neuroblastoma cell lines only expressed subcompartments of the MSD1 region. The MSD1c exon was not spliced into the NCAM molecules isolated from any of the neuroblastoma cell lines investigated. On the basis of this finding, it appears that neuroblastoma and rhabdomyosarcoma can be distinguished by the expression of MSD1c mini-exon. Further studies are underway to attempt to define a monoclonal antibody that recognises the region, using mice immunised with synthetic peptides, and to confirm the finding using fresh biopsy material.

Base Sequence↗
PubMed1996

Response of previously untreated metastatic rhabdomyosarcoma to combination chemotherapy with carboplatin, epirubicin and vincristine.

This study aimed to assess the response to a novel combination chemotherapy containing carboplatin plus epirubicin in previously untreated children with metastatic rhabdomyosarcoma. 81 children (< or = 18 years) were treated between 1989 and 1994 with a combination of carboplatin, epirubicin and vincristine, given as initial therapy as part of a multicentre European trial (SIOP Intergroup Study of Stage IV Malignant Mesenchymal Tumours in Children). The chemotherapy regimen (CEV) was: carboplatin 500 mg/m2 plus epirubicin 150 mg/m2 administered on day 1, and vincristine 1.5 mg/m2 administered on days 1 and 7. Response was evaluated at day 21. 2 patients achieved complete remission and 41 patients partial remission, with an overall response rate of 53% (95% confidence interval 45-76%). Three patients showed progressive disease (4%). Toxicity was mainly haematological, with 52% experiencing grade IV neutropenia and 34% grade IV thrombocytopenia. Mucositis and infections were not severe. There were no toxic deaths. The combination of carboplatin, epirubicin and vincristine is effective and well tolerated in patients with metastatic rhabdomyosarcoma.

Adolescent↗
PubMed2006

Ovarian Sertoli-Leydig cell tumor of intermediate grade with heterologous elements of rhabdomyosarcoma. A case report and a review of the literature.

Ovarian Sertoli-Leydig cell tumors (SLCTs) are rare sex cord-stromal tumors, and among them, tumors with heterologous mesenchymal elements are exceptional and mainly associated with poorly differentiated tumors and are often fatal. We present the fourth case of an ovarian SLCT of intermediate differentiation with rhabdomyosarcoma and a review of the literature. Surgical treatment was conservative with preservation of the contralateral adnexa and uterus. No adjuvant treatment was given. At 4 years control post surgery, the patient was without evidence of disease. Extensive sampling of SLCTs is important because heterologous elements may be sparse. Immature skeletal muscle cells in SLCTs often reveal only moderate pleomorphism, and as they are closely admixed with the Sertoli cells or immature gonadal stroma, they can be rather difficult to differentiate from the latter ones. Immunohistochemical analysis with a panel of antibodies including antibodies against myogenin and alpha-inhibin is very important to diagnose the rhabdomyosarcoma and to grade the SLCT accurately.

Adult↗
PubMed2004

A novel t(2;20)(q35;p12) in embryonal rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood, accounting for 5%-8% of all pediatric malignancies. RMS can be categorized into several subtypes, including embryonal RMS (ERMS), the botryoid and spindle cell variants of ERMS, and alveolar RMS (ARMS). The t(2;13)(q35;q14) and the variant t(1;13)(p36;q14) are seen in a majority of ARMS cases. In contrast, the embryonal subtype of rhabdomyosarcoma has not been associated with a recurring chromosomal translocation. We describe here a novel chromosomal t(2;20)(q35;p12) occurring in a case of childhood RMS with embryonal histology. It is notable that this translocation harbors breakpoints at or near the locus of the PAX3 gene, which is involved in the most common recurring translocation associated with ARMS.

Child, Preschool↗
PubMed2005

[Rhabdomyosarcoma of the salivary glands. Report of 3 cases].

Rhabdomyosarcoma of the salivary glands is rare. Tumours develop mostly in children and young patients but can be diagnosed in older people. We report three new cases in women 7, 14 and 75 years old. Tumefaction of the parotid region and facial paralysis were the principle clinical symptoms. Two patients underwent a total parotidectomy and radiotherapy. The third patient had a locally advanced tumour and received chemotherapy followed by radiotherapy. Loco-regional recurrence was observed in all cases and median survival was 12 months. Rhabdomyosarcoma of the salivary glands is locally aggressive. Treatments include surgery and radiotherapy. The role of chemotherapy remains to be discussed.

Adolescent↗
PubMed2005

Novel genes, possibly relevant for molecular diagnosis or therapy of human rhabdomyosarcoma, detected by genomic expression profiling.

Transcriptional profiles of an alveolar rhabdomyosarcoma (RMS) and of a RMS cell line were reconstructed by a computational and statistical approach. Expression data of 29,963 genes in 11 adult human healthy tissues and in 37 tumour tissues were analysed for comparison. We identified 202 genes differentially expressed in at least one RMS sample, as compared with normal skeletal muscle. Among them, 107 resulted specifically overexpressed in RMS, but in no tumour affecting other tissues. Cluster analysis applied to expression data detected a series of genes presumably co-expressed with genes encoding known tumour markers and/or reportedly involved in genesis or development of rhabdomyosarcoma. This study succeeded in identifying a number of genes, which become candidates for in vitro study, thus facilitating discovery of novel tumour markers or targets for drug therapy.

Adult↗
PubMed2005

Expression of T cell receptor alpha gene (TCRA) in human rhabdomyosarcoma and other musculo-skeletal sarcomas.

Expression of the T cell receptor (TCR) genes is not restricted to T lymphocytes. Human prostate and breast express a truncated TCR gamma transcript. In the mouse, TCR alpha (TCRA) and beta partial transcripts are expressed by mesenchymal cells and TCRA transcripts by epithelial cells of the kidney. We show now that TCRA constant region expression is common in normal and neoplastic human cells of mesenchymal and neuroectodermal origin. TCR transcripts are derived from an unrearranged TCRA locus. Moreover, rhabdomyosarcoma cells highly expressed a specific J49-C splicing product deriving from the assembly of J49 segment and constant region. TCRA ectopic transcripts/proteins negatively regulate rhabdomyosarcoma cell growth as suggested by TCRA gene expression downmodulation effects using a specific duplex small interfering RNA.

Base Sequence↗
PubMed2005

Intensity-modulated radiotherapy for head-and-neck rhabdomyosarcoma.

PURPOSE: To determine the preliminary results of intensity-modulated radiotherapy (IMRT) for head-and-neck rhabdomyosarcoma. METHODS AND MATERIALS: Twenty-eight patients underwent IMRT as a part of multimodality therapy. Twenty-one tumors were parameningeal, three were orbital, and four were in other sites. The median age was 8 years (range, 1-29 years). Most (89%) had Group III disease. Intracranial extension was present in 71% of parameningeal tumors. A 1.5-cm margin was used, and the median dose was 50.4 Gy (range, 30-55.8 Gy). RESULTS: The actuarial 3-year survival rate for patients with parameningeal tumors was 65%. The 3-year actuarial freedom from failure rate was 95% locally, 90% in regional nodes, 88% in the central nervous system, and 80% at distant sites. No failures occurred among patients with orbit tumors; a single central nervous system failure occurred in 1 patient with a lip/cheek tumor. Disease-free survival was significantly worse for patients with alveolar histologic features (p = 0.01). Acute radiation toxicity was similar to that reported by the Intergroup Rhabdomyosarcoma Study Group. Late radiation toxicity was recorded and was mild. CONCLUSION: IMRT with image fusion results in outstanding local control despite the use of a reduced margin. However, survival among patients with alveolar histologic findings or intracranial extension remains unacceptably low.

Adolescent↗
PubMed2005

Proton radiotherapy for orbital rhabdomyosarcoma: clinical outcome and a dosimetric comparison with photons.

BACKGROUND: Over 85% of pediatric orbital rhabdomyosarcoma (RMS) are cured with combined chemotherapy and radiation. However, the late effects of photon radiation compromise function and cosmetic outcome. Proton radiation can provide excellent tumor dose distributions while sparing normal tissues better than photon irradiation. METHODS AND MATERIALS: Conformal 3D photon and proton radiotherapy plans were generated for children treated with proton irradiation for orbital RMS at Massachusetts General Hospital. Dose-volume histograms (90%, 50%, 10%) were generated and compared for important orbital and central nervous system structures. Average percentages of total dose prescribed were calculated based on the 3 dose-volume histogram levels for normal orbital structures for both the proton and photon plans. The percent of normal tissue spared by using protons was calculated. RESULTS: Seven children were treated for orbital rhabdomyosarcoma with proton irradiation and standard chemotherapy. The median follow-up is 6.3 years (range, 3.5-9.7 years). Local and distant controls compare favorably to those in other published accounts. There was an advantage in limiting the dose to the brain, pituitary, hypothalamus, temporal lobes, and ipsilateral and contralateral orbital structures. Tumor size and location affect the degree of sparing of normal structures. CONCLUSIONS: Fractionated proton radiotherapy is superior to 3D conformal photon radiation in the treatment of orbital RMS. Proton therapy maintains excellent tumor coverage while reducing the radiation dose to adjacent normal structures. Proton radiation therapy minimizes long-term side effects.

Brain↗
PubMed2005

Characteristics and outcomes of rhabdomyosarcoma patients with isolated lung metastases from IRS-IV.

PURPOSE: To better understand outcomes in children with rhabdomyosarcoma (RMS) and lung-only metastatic disease, the authors reviewed the experience from Intergroup Rhabdomyosarcoma Studies IV Pilot and IV. METHODS: Patients with lung-only (n = 46) vs other sites of metastatic disease (n = 234) were reviewed using patient charts and the database of Children's Oncology Group (COG). RESULTS: Sixteen percent of patients with RMS and metastatic disease had isolated lung metastases. Thirty-one (67%) had more than 5 metastatic lung lesions. These were bilateral in 34 (74%). Only 6 patients were biopsied at diagnosis. Sixteen children (35%) did not receive any lung radiotherapy. Patients that received lung radiotherapy had fewer lung recurrences ( P = .04), although this has no significant impact on overall survival (OAS, 47% radiotherapy vs 31% no radiotherapy). Compared with patients with other sites of metastatic disease, patients with lung-only metastases have a greater proportion of favorable histology (67% vs 39%, P = .0017), negative nodal involvement (67% vs 32%, P = .0013), and parameningeal primaries (39% vs 12%) and a smaller proportion of extremity primaries (20% vs 33%, P = .0005 for site of primary tumor). Overall survival at 4 years for lung-only metastases was not significantly different from other single-site metastasis (42% vs 34%). Survival was not improved for unilateral disease or fewer than 5 metastatic lesions. Factors associated with diminished OAS include unfavorable histology (P = .0001) and age >10 years (P = .015). CONCLUSIONS: Children with RMS and lung-only metastases usually present with extensive bilateral disease that is frequently not biopsied nor given protocol-recommended radiotherapy (XRT). However, outcome is comparable, although slightly better, than patients with other single-site metastasis.

Adolescent↗
PubMed2005

Orbital rhabdomyosarcoma and simulating lesions.

The appropriate diagnosis and treatment of orbital rhabdomyosarcoma requires close cooperation and communication between the radiologist, the ophthalmologist, and the medical and radiation oncologists. The clinical presentation, imaging characteristics, and staging of orbital rhabdomyosarcoma are discussed. A discussion of several important simulating lesions and their distinguishing characteristics follows.

Diagnosis, Differential↗
PubMed2004

Embryonal rhabdomyosarcoma of the tongue in old age.

Rhabdomyosarcoma represents the most common soft tissue sarcoma of patients under 15 years of age, but it is definitely rare in subjects older than 45. A very rare case of embryonal type rhabdomyosarcoma of the tongue in a 65-year-old man is reported. The adult patient was treated with surgery and post-operative adjuvant radiotherapy and is disease-free at 48 months of follow-up. TUNEL analysis showed that apoptosis was present in 10% of the cells, thus suggesting that an high number of apoptotic cells may represent an indicator of good prognosis for this malignancy.

Aged↗
PubMed2005

Rhabdomyosarcomas of the nose and paranasal sinuses: treatment results in 15 cases.

OBJECTIVE: Primary rhabdomyosarcomas (RMS) of the nose and the paranasal sinuses occur very rarely. Treatment of these tumors usually is conducted according to standardized therapy protocols like the German Cooperative Soft Tissue Sarcoma Study (CWS) or the Intergroup Rhabdomyosarcoma Study (IRS). The role of surgery still remains controversial. STUDY DESIGN AND SETTING: A retrospective analysis of 15 patients with an RMS of the nose or paranasal sinuses treated between 1979 and 2000 is presented. Patients' age ranged from 2 to 60 years, with an average of 22 years. Histologic subtypes encompassed 9 embryonal (e) and 6 alveolar (a) RMS. Resection of the tumor with subsequent radiochemotherapy (RCT) was performed in 6 cases, and primary RCT, in 9 cases. The average duration of follow-up was 4 years and 10 months. RESULTS: Overall 5-year survival was 40%. In the patient group subjected to tumor resection with subsequent RCT, 5-year survival was 66%, compared with 33% after exclusive primary RCT. With respect to histologic subtype, 5-year survival was 55% for eRMS, as compared with 33% for aRMS. Moreover, infiltration of the skull base and the presence of a residual tumor after primary therapy constituted factors associated with an unfavorable clinical course. CONCLUSIONS: Surgical removal of tumor with subsequent radiochemotherapy can be recommended if a complete resection and functionally and cosmetically satisfactory results appear possible. Patients with eRMS showed an overall more favorable clinical course than patients with aRMS.

Adolescent↗
PubMed2005

Rhabdomyosarcoma of tunica vaginalis masquerading as hydrocele.

Paratesticular rhabdomyosarcomas are rare tumors with aggressive growth patterns. Multimodal therapy with surgery, chemotherapy, and radiotherapy provides the patient with an excellent long-term prognosis. These tumors often present in the first two decades after birth. We report on the case of an 18-year-old man with a paratesticular rhabdomyosarcoma.

Adolescent↗
PubMed2004

Embryonal rhabdomyosarcoma (sarcoma botryoides) of the cervix presenting as a cervical polyp treated with fertility-sparing surgery and adjuvant chemotherapy.

BACKGROUND: Embryonal rhabdomyosarcoma (sarcoma botryoides) of the female genital tract is rare in the cervix. It tends to occur in children and young women, and appears to have a better prognosis than sarcoma botryoides of the vagina and uterus. CASE: We report a case of embryonal rhabdomyosarcoma (sarcoma botryoides) presenting as a cervical polyp in a 19-year-old female. The tumor was composed of rhabdomyoblasts of varying differentiation dispersed within a loose, myxoid stroma, and formed a distinct 'cambium' layer beneath the epithelium. The patient was successfully treated with fertility-sparing surgery and adjuvant chemotherapy. CONCLUSIONS: Awareness of this uncommon lesion in this site and its clinical implications is important to avoid misdiagnosis. Therapy has recently inclined towards conservative treatment.

Adult↗
PubMed1994

Visual acuity after radiation for orbital rhabdomyosarcoma.

PURPOSE/METHODS: We reviewed follow-up data on 32 patients with orbital rhabdomyosarcoma, who were treated with external beam radiation with and without adjunctive chemotherapy between 1960 and 1992. RESULTS/CONCLUSIONS: Patients were followed up from five to eight years. Of the 32 patients, six (19%) had best-corrected visual acuity of 20/70 or better; five patients (15%) had best-corrected visual acuity of light perception to 20/300; 21 patients (66%) had no light perception. The reported long-term visual acuities should be factored in the equation for selecting treatment for patients with orbital rhabdomyosarcoma.

Combined Modality Therapy↗