Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “RHABDOMYOSARCOMA”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 415 records · Page 23Linked to original sources
PubMed1994

Paratesticular rhabdomyosarcoma: delayed effects of multimodality therapy and implications for current management.

BACKGROUND: The combined modalities of surgery, chemotherapy, and radiation therapy have greatly improved the survival rate in childhood paratesticular rhabdomyosarcoma, but the incidence of complications and late side effects is a cause for concern. METHODS: We reviewed the records of 18 patients treated for paratesticular rhabdomyosarcoma at St. Jude Children's Research Hospital between 1962 and 1989. Patients with Group I disease were treated with orchiectomy, retroperitoneal lymph node dissection, and multi-agent chemotherapy; more advanced cases also received radiation therapy with concurrent chemotherapy. RESULTS: Sequelae included esophageal and common bile duct stricture, inguinal nerve entrapment syndrome, and small bowel obstruction. Short stature was found in all children whose spines were irradiated via para-aortic fields (34-37 Gy) prior to puberty. Two of 18 patients died from treatment complications and one from progressive disease. CONCLUSIONS: Multimodality treatment offers an excellent prognosis in paratesticular rhabdomyosarcoma, but is associated with significant morbidity and mortality rates. A discussion of therapy components and their application to disease stages suggests possible approaches to optimizing treatment for this therapy-sensitive malignancy.

Adolescent↗
PubMed1994

Rhabdomyosarcoma of the clitoris.

Genitourinary rhabdomyosarcoma in females usually originates in the vagina or uterus, but rarely the vulva. The authors present a case of rhabdomyosarcoma originating in the clitoris. A 4-year-old with an alveolar rhabdomyosarcoma of the clitoris was treated with radical clitorectomy, radiation, and chemotherapy. Follow-up at 3 years showed no active disease.

Child, Preschool↗
PubMed2004

Vinorelbine and low-dose cyclophosphamide in the treatment of pediatric sarcomas: pilot study for the upcoming European Rhabdomyosarcoma Protocol.

BACKGROUND: Following their previous report on the activity of vinorelbine in the treatment of rhabdomyosarcoma, the authors report the results of a pilot study aimed at defining the optimal dose of vinorelbine when this agent is used in conjunction with continuous, orally administered low-dose cyclophosphamide to treat patients with refractory or recurrent sarcoma. It is hoped that the combination of vinorelbine and low-dose cyclophosphamide can be used as a maintenance regimen in an upcoming European trial involving high-risk patients with rhabdomyosarcoma. METHODS: In the current pilot study, the cyclophosphamide dose was fixed at 25 mg/m2 per day for 28 days. Vinorelbine was administered intravenously on Days 1, 8, and 15, with trial doses escalated from an initial level of 15 mg/m2 in steps of 5 mg/m2; intrapatient dose escalation was not allowed. RESULTS: Between April 2002 and November 2003, 18 patients ages 2-23 years were treated with the study regimen after having received 1-4 (median, 2) other regimens previously. Ninety cycles were administered in total (median, 5 cycles per patient; range, 1-10 cycles per patient). Two cases of dose-limiting toxicity (Grade 4 neutropenia in both cases) were observed among the 5 patients who received vinorelbine at a dose of 30 mg/m2. Of the 41 cycles in which vinorelbine was administered at a dose of 25 mg/m2, Grade > or = 3 neutropenia was observed in 15 (37%); no other major toxicity was documented in association with these cycles. One complete remission and 6 partial remissions were noted among the 17 patients who had measurable disease. Three of the eight assessable patients with rhabdomyosarcoma (which was embryonal in two cases and alveolar in one) had responses to treatment. CONCLUSIONS: Combination therapy involving vinorelbine and low-dose cyclophosphamide was found to be feasible and to possess activity against recurrent sarcomas. The maintenance therapy doses recommended for use in the upcoming European trial are cyclophosphamide 25 mg/m2 per day for 28 days and vinorelbine 25 mg/m2 on Days 1, 8, and 15.

Adolescent↗
PubMed2006

Fine-needle aspiration cytology of alveolar rhabdomyosarcoma utilizing ThinPrep liquid-based sample and cytospin preparations: a case confirmed by FKHR break apart rearrangement by FISH probe.

Rhabdomyosarcoma is the most common childhood sarcoma that occurs in the soft tissues of the head and neck, genitourinary system, and extremities. While this tumor may also be seen in young adults, it is distinctly unusual to see this neoplasm beyond the fifth decade. The use of fluorescence in situ hybridization (FISH) probes on fine-needle aspiration material for chromosomal analysis to detect chromosomal translocations are not well established. Herein we present a case of metastatic alveolar rhabdomyosarcoma originating in the nasal sinus and recurring in the neck of an adult man 5 yr after his initial presentation. The ThinPrep smear demonstrated isolated malignant tumor cells similar to his previous histologic material. The cytologic findings of alveolar rhabdomyosarcoma were confirmed utilizing a dual color break apart FISH probe which identified the FKHR translocation on the cytospin preparation prepared from a ThinPrep vial and processed as a cytospin preparation. In addition to routine and immunocytologic staining, we demonstrate that aspirated tumor cells can be analyzed using a FISH probe to identify specific chromosomal abnormalities.

Biopsy, Fine-Needle↗
PubMed1992

Fine-needle aspiration biopsy diagnosis of rhabdomyosarcoma: cytologic, histologic, and ultrastructural correlations.

A series of 15 cases of rhabdomyosarcoma diagnosed by fine-needle aspiration biopsy (FNAB) and confirmed by histopathology is reviewed. Cytologically, the tumors were composed of a variable mixture of cells, which according to the degree of differentiation were categorized as early, intermediate, or late rhabdomyoblasts. Histologically, the tumors were divided into embryonal 9, monomorphic round cell 4, and alveolar rhabdomyosarcoma 2. Comparison of histological and cytological features revealed that embryonal types were composed mainly of early rhabdomyoblasts. Recognition of these patterns may be helpful in FNAB diagnosis of rhabdomyosarcoma.

Adolescent↗
PubMed1991

Variant translocations of chromosome 13 in alveolar rhabdomyosarcoma.

In three cases of alveolar rhabdomyosarcoma with variant translocations, two tumors contained an identical translocation, t(1;13)(p36.1;q14); the third tumor contained a t(8;13)(p21;q14). All three patients were 2 years old, markedly younger than the median age for patients with t(2;13)-positive alveolar rhabdomyosarcoma. The alteration of genetic material on chromosome 13 may be of primary importance in the development of alveolar rhabdomyosarcoma.

Child, Preschool↗
PubMed1990

Characterization of an embryonal rhabdomyosarcoma cell line showing amplification and over-expression of the N-myc oncogene.

A parent rhabdomyosarcoma cell line designated SCMC-RM2 was established from bone-marrow tumor cells taken from an 11-year-old girl with an embryonal rhabdomyosarcoma. Subsequently a cloned SCMC-RM2-1 cell line was isolated from a parent line. These cell lines grew as adherent monolayers in liquid culture with a doubling time of 50 and 52 hr, respectively. In addition, colonies were established in soft agar, which grew in a dose-dependent fashion with a cloning efficiency of 0.7 and 0.8%, respectively. Chromosomal analysis showed these cell lines had neither double minutes nor homogeneously staining regions. Chromosome number ranged from 61 to 93, translocation; t(9;13)(p22;q14) was identified, and no alteration of chromosome 2 was observed. Surface membrane antigen profile of parent and cloned lines by using a panel of 24 monoclonal antibodies (MAbs) excluded the possibility of these being neuroblastoma cell lines. In addition, MAbs to the cytoplasmic protein desmin, myoglobin, muscle actin (alpha and gamma) and alpha-sarcomeric actin reacted with these cell lines, SCMC-RM2 and SCMC-RM2-1 being thus identified as rhabdomyosarcoma. Southern blot analyses revealed 8- and 7-fold amplification of the N-myc gene in SCMC-RM2 and SCMC-RM2-1 as compared with the promyelocytic cell line HL60. Over-expression of the N-myc mRNA was noted over control cell lines.

Antigens, Neoplasm↗
PubMed1992

Differential expression of myogenic regulatory genes, MyoD1 and myogenin, in human rhabdomyosarcoma sublines.

A cell line (SCMC-MM-1) was established from a human abdominal tumor that was initially diagnosed as a malignant mesenchymoma by histological, immunohistochemical and clinical criteria. The cell line was composed of 2 morphologically and immunohistochemically distinct cell types, one with a small polygonal phenotype (P-type), characterized by the immunostaining of vimentin and the presence of a few electron-microscopically visible organelles, and the other with a giant tubular phenotype (T-type), characterized by the immunostaining of desmin, alpha-sarcomeric actin and skeletal-muscle myosin, and the presence of thick and thin myofilaments and Z-line materials. The parental cell line was cloned into 2 sublines, a P-type clone (SCMC-MM-1-19P) and a T-type clone (SCMC-MM-1-1T), which shared both 2q37 and 11p15 translocations, the characteristic chromosomal aberrations for rhabdomyosarcoma, with the parental SCMC-MM-1 cell line. Northern-blot analyses of the myogenic regulatory genes, including MyoD1 and myogenin, demonstrated the expression of MyoD1 in both of these sublines. Myogenin was very weakly expressed in the SCMC-MM-1-19P subline, but strongly expressed in the SCMC-MM-1-1T subline. Chromosomal and myogenic-regulatory-gene analyses revealed that both of these sublines were rhabdomyosarcoma cell lines. Furthermore, the regulatory-gene analyses indicated that these 2 sublines represented 2 distinct differentiation stages of myoblasts, and that MyoD1 and myogenin could serve as the lineage marker and the differentiation marker, respectively, of human rhabdomyosarcoma.

Blotting, Northern↗
PubMed1987

Systemic rhabdomyosarcoma presenting as leukemia: case report with ultrastructural study and reviews.

A 20-year-old white male was initially suspected clinically and pathologically of having an acute lymphoblastic leukemic process because of fatigue, severe anemia, thrombocytopenia, a leuko-erythroblastic peripheral blood picture, and a diffusely infiltrated bone marrow. Subsequent review of the bone marrow material indicated cytologic features consistent with either an embryonal, undifferentiated small cell mesenchymal malignancy or reticulo-endothelial malignancy. Ultimately, the electron microscopic (EM) study of the tumor proved to be diagnostic of rhabdomyosarcoma. An extensive search for a primary site of rhabdomyosarcoma did not show any lesion, although the genitourinary region was clinically suspected. The clinical course was a rapidly downhill one with extensive bone and CNS involvement. The patient died 5 months later. An autopsy permit was not obtained. This case emphasizes the occasional tendency of rhabdomyosarcoma to masquerade as a hematopoietic malignancy at the time of presentation and the usefulness of EM study in confirming a diagnosis.

Adult↗
PubMed1995

Dental abnormalities in long-term survivors of head and neck rhabdomyosarcoma.

To define the long-term dental sequelae of therapy for childhood rhabdomyosarcoma of the head and neck, and to identify factors in their development, we retrospectively reviewed the serial panoramic radiographs and clinical records of 22 survivors of head or neck rhabdomyosarcoma who had been followed for at least 5 years. Patients were divided into four groups based upon age at the time of therapy and three groups based upon radiation doses. All patients had received similar multiagent chemotherapy. Dental sequelae of oncotherapy occurred in over half of the long-term survivors of head and neck rhabdomyosarcoma. The abnormalities comprised root stunting in 54%, microdontia in 23%, and hypodontia in 50% of patients; 36% had multiple abnormalities. Microdontia and multiple abnormalities were more prevalent in patients treated at the earliest age, and abnormalities tended to be more prevalent with increasing doses of radiation. Five patients (23%) developed severe cosmetic and/or functional sequelae necessitating surgical and/or orthodontic intervention. The high frequency of dental sequelae we observed suggests that meticulous long-term dental and radiographic follow-up are needed. Early detection and treatment of the complications of therapy will expedite their correction and minimize morbidity.

Adolescent↗
PubMed2004

Neonatal prostatic rhabdomyosarcoma.

Rhabdomyosarcoma is one of the commonest soft tissue sarcomas of childhood, but neonatal presentation is extremely rare. This limited experience means there are no clear treatment guidelines in this age group. The authors report a boy with recurrent attacks of hematuria commencing in the neonatal period, which were shown to be from a prostatic rhabdomyosarcoma. To the best of our knowledge this is the first reported case of neonatal rhabdomyosarcoma in this site.

Humans↗
PubMed1993

Rhabdomyosarcoma-derived cell lines exhibit aberrant expression of the cell-cell adhesion molecules N-CAM, N-cadherin, and cadherin-associated proteins.

Many cancer cells show aberrant adhesion properties that likely contribute to tumorigenesis, invasion, and metastasis. Here, we examine three MyoD-expressing, rhabdomyosarcoma-derived human cell lines (RD, A-204, and HS 729) for their expression of the neural cell adhesion molecule (N-CAM), N-cadherin, and the cadherin-associated proteins, alpha-catenin, beta-catenin, and plakoglobin, using specific antibodies and immunoblotting and immunocytochemical methods. Normally, during the formation of skeletal muscle, both N-CAM and N-cadherin are expressed and participate in mediating myoblast adhesion accompanying cell fusion. RD cells express N-CAM, N-cadherin, and the cadherin-associated proteins; however, N-CAM is expressed as a highly sialylated isoform that functions poorly in promoting Ca(2+)-independent cell aggregation. HS 729 cells express N-cadherin and its associated intracellular proteins but have no detectable N-CAM. A-204 cells express no detectable N-CAM or N-cadherin but do express cadherin-associated proteins. Thus, all three rhabdomyosarcoma cell lines exhibit some abnormality in the expression of adhesion molecules known to participate in skeletal myogenesis; however, no common defect was observed that might be considered as a characteristic marker for rhabdomyosarcomas.

Cadherins↗
PubMed1996

cAMP effects on myogenic gene expression in rhabdomyosarcoma cells.

Rhabdomyosarcoma is a tumor of skeletal muscle origin affecting children and young adults. Although relatively undifferentiated, cell lines derived from this tumor express myogenic regulatory factors and so may be useful models of abortive myogenic differentiation. In the present studies, we have determined the effect of increased intracellular cAMP on proliferation, morphologic differentiation, and expression of myogenic genes in the prototypic embryonal rhabdomyosarcoma cell line, RD. Whereas growth in dibutyryl cAMP (dbcAMP), forskolin, or butyrate led to morphologic differentiation, growth in dbcAMP inhibited proliferation, while growth in butyrate slowed but did not stop cell division. Expression of the genes for myogenin and myosin light chain was inhibited by dbcAMP, while butyrate decreased myogenin and increased myosin light chain transcription. MyoD and MRF4 expression was not altered under either condition and no myf5 expression was detected. We also determined the effects of dbcAMP and butyrate on total protein expression, as well as on a panel of muscle- and neural-specific proteins using functional assays, immunohistochemistry, and immunoprecipitation. The total protein levels of cells treated with either agent were double those of untreated cells. DbcAMP increased the activity of acetylcholinesterase (AChE) up to 10-fold compared to untreated cells, while butyrate had a substantially lesser effect. These increases were due to increased AChE protein synthesis and stability in dbcAMP treated cells, compared to butyrate or untreated cells. Finally, cells under all conditions expressed MAP2, a neural-specific microtubule associated protein. Together, these data suggest that intracellular cAMP levels modulate distinct subsets of the myogenic differentiation pathway in rhabdomyosarcoma cells. Moreover, they also indicate that RD cells are able to express markers of different cell lineages, which may help explain some of the paradoxical features of these tumors.

Acetylcholinesterase↗
PubMed1999

Up-regulation of type XIX collagen in rhabdomyosarcoma cells accompanies myogenic differentiation.

Rhabdomyosarcomas are known to recapitulate some of the early events in skeletal muscle embryogenesis, and cultures derived from these tumors have been extensively used to elucidate processes associated with the differentiation of primitive mesenchymal cells. These neoplasms have also provided important systems for studying different collagen types. This aspect is particularly relevant to type XIX collagen, which was originally identified from rhabdomyosarcoma cDNA clones. Although this collagen has been localized in vivo to basement membrane zones in a wide variety of tissues, including skeletal muscle, the tumor cells appear to be a unique source of its expression in vitro. We have found that one particular cell line-derived from a peritesticular embryonal rhabdomyosarcoma-produced relatively large amounts of type XIX collagen, especially in those rare instances in which these cells appear to spontaneously differentiate. To characterize this phenomenon, tumor cells were grown under conditions known to induce differentiation in normal myoblast cultures. In response to this treatment, the typical tumor cell morphology consistently and reproducibly switched from polygonal to round/spindle-shaped with the subsequent appearance of some structures resembling myotubes. Concurrently, the cultures commenced a dramatic up-regulation of type XIX collagen and skeletal muscle myosin heavy chain and alpha-actinin in a time-dependent fashion, whereas protein and mRNA levels of other matrix proteins were either decreased or unchanged. Moreover, immunocytochemical analysis revealed that only a subpopulation of the cells was responsible for the increased synthesis of type XIX collagen, alpha-actinin, and myosin, and that the same cells which stained positive for the collagen also stained positive for the muscle proteins. Taken together, the results suggested that type XIX collagen may be involved in the initial stages of skeletal muscle cell differentiation.

Actinin↗
PubMed1995

Correlation between cell differentiation stage, types of invasion, and hematogenous metastasis in experimental rhabdomyosarcomas.

Cancer malignancy is directly related to invasiveness and metastasis and inversely related to the degree of tumor differentiation. The relation between the stage of cell differentiation and the types of invasion leading to metastasis is not entirely clear. Intramuscularly transplanted rat rhabdomyosarcomas are good models to study cell differentiation, invasion, and metastasis. Rat rhabdomyosarcoma cell lines (SMF-Ai, SMF-Da, and RMS-B and its clones) with defined invasive and metastatic potentials have been established. The stage of myogenic differentiation was evaluated morphologically and by immunohistochemistry. Invasiveness was evaluated according to the infiltration of muscle fibers and basal lamina. The SMF-Ai line is highly invasive and metastatic. It is composed of premyoblasts that were involved in intercellular, translaminar, and transcellular invasion of muscle fibers. The SMF-Da line is noninvasive and nonmetastatic. It is composed of myoblasts. The RMS-B line and its clones were at different stages of differentiation and they differed in their invasiveness and metastatic potentials. In highly invasive and metastatic clones (RMS-Bg and RMS-Bc), premyoblasts were involved in translaminar invasion. Clones composed of myoblasts, rhabdomyoblasts, and myotubes only showing intercellular invasion did not present hematogenous metastasis. Our results demonstrate a correlation between premyoblastic stage of differentiation and translaminar invasion. The presence of translaminar invasion is directly related to hematogenous metastatic ability of rat rhabdomyosarcomas.

Animals↗
PubMed1993

Uterine mixed embryonal rhabdomyosarcoma and fetal rhabdomyoma.

A polypoid uterine tumor, occurring in a 31-year-old woman, with histopathologic features of both embryonal rhabdomyosarcoma and fetal rhabdomyoma is reported. The clinical and pathologic differences between pure fetal rhabdomyoma and embryonal rhabdomyosarcoma are detailed and theories concerning the histogenesis of such a mixed, or intermediate, tumor are discussed. Most likely, this neoplasm represents one example in a spectrum of tumors with varying proportions of immature and mature skeletal muscle elements. Its good prognosis is in keeping with that of uterine (cervical) embryonal rhabdomyosarcomas in general, although the presence of mature skeletal muscle elements may signify an even better prognostic group of tumors.

Adult↗
PubMed1996

Alveolar rhabdomyosarcoma of the uterine cervix.

Alveolar rhabdomyosarcoma is an uncommon tumor occurring mostly in extremities of children and adolescents. This type of tumor located in the uterine cervix has not been reported previously. A case of pure alveolar rhabdomyosarcoma arising from the uterine cervix in a 45-year-old patient and having a very aggressive clinical course is described. The patient presented with rapidly progressive cervical tumor, which was originally diagnosed as mesonephroid adenocarcinoma. She was treated with a radical two-stage surgical procedure and postoperative external beam and intracavitary irradiation. Despite treatment, she developed metastatic disease and died 3 months after the surgery. The aggressive course of the disease at this location was consistent with that of the majority of alveolar rhabdomyosarcoma cases of other sites reported in literature. No standard effective treatment is known for this tumor in adult patients.

Combined Modality Therapy↗
PubMed1998

Integrated multimodality therapy for embryonal rhabdomyosarcoma of the lower genital tract in postpubertal females.

Embryonal rhabdomyosarcoma of the female lower genital tract is generally regarded as a neoplasm occurring in childhood, but has also been reported in adults. The philosophy of therapy, largely based on data obtained from pediatric patients, has evolved slowly from ultraradical surgery, without adjuvant therapy, to neoadjuvant chemotherapy followed by less radical surgery and postoperative radiation. We report here three cases of lower genital tract rhabdomyosarcoma in postpubertal females. A failure to observe complete responses from any single treatment modality suggests that for embryonal rhabdomyosarcoma in adult and adolescent women a multimodality approach to therapy is essential.

Adolescent↗