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PubMed2002

Post-chemotherapy microscopic residual prostate rhabdomyosarcoma: long-term conservative follow-up.

In spite of advances in the treatment of childhood bladder and prostate rhabdomyosarcoma (RMS), the ability to detect minimal residual disease correlates imperfectly with the ultimate outcome. We report the long-term follow-up of a child with microscopic residual RMS after chemotherapy. The correct interpretation of the histologic findings spared the child unnecessary additional therapy and raises enigmatic questions about the biology of minimal residual disease.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2003

Anaplastic (pleomorphic) subtype embryonal rhabdomyosarcoma of the cervix.

INTRODUCTION: Embryonal rhabdomyosarcoma (RMS) of the female genital tract usually occurs in the vagina during childhood. In rare cases, RMS can originate in the uterine cervix, with an incidence peak in the second decade. Recent studies have suggested that it is possible to limit surgery to local excision in stage I cases. CASE REPORT: We present the case of a 13-year-old girl diagnosed with an anaplastic (pleomorphic) subtype embryonal RMS of the endocervix, who was treated successfully with polypectomy followed by ifosfamide-vincristine-actinomycin combination chemotherapy and brachytherapy. The patient exhibits no evidence of recurrence and has normal menstrual function 36 months following initial diagnosis.

Adolescent↗
PubMed1998

Laryngeal pleomorphic rhabdomyosarcoma.

A case of an extremely unusual tumor of the larynx, pleomorphic rhabdomyosarcoma, is presented with a review of literature. This is the fifth case of this malignancy described in the larynx in the English language literature. A histopathological diagnosis was made with immunohistochemistry and electron microscopy. In contrast to other reported cases, the tumor in the present case had a very aggressive behavior. Despite radical surgery involving total laryngectomy and neck dissection followed by radiation therapy, the patient died of disease 8 months following treatment.

Aged↗
PubMed2000

Costello syndrome: report of six patients including one with an embryonal rhabdomyosarcoma.

UNLABELLED: Costello syndrome was first described in 1971. Besides papillomata, which were part of the initial description, patients tends to develop benign tumours of ectodermal origin. Aetiology is yet unknown but it is supposed to be the result of a sporadic dominant mutation. We report six patients with typical clinical findings and emphasise the importance of cardiac manifestations and the tendency to develop tumours. One patient developed an embryonal rhabdomyosarcoma, the occurrence of which has been reported twice before in patients with Costello syndrome. CONCLUSION: There might be a causal link between the development of rare tumours and this genetic disorder which may provide a new clue concerning the identification of the gene involved in Costello syndrome.

Cardiomyopathy, Hypertrophic↗
PubMed1999

Experimental induction of rhabdomyosarcoma in mice with fractionated doses of beta-irradiation.

Repeated doses of beta-radiation in the mouse skin model have been reported to produce carcinomas and sarcomas with equal frequency. Among sarcomas, fibrosarcomas and osteosarcomas have been the predominant reported histologies. In this report we describe the beta-radiation induction of rhabdomyosarcoma (RMS), a histology previously undescribed with tumor induction protocols using ionizing radiation in an animal model. Radiation-induced RMS is often seen as a secondary tumor following therapeutic irradiation for retinoblastoma in children. In our experiment the backs of 50 CD-1 mice were irradiated 3 times weekly for 35 weeks using a 90Sr source. The initial dose was 5.5 Gy/application, which was later reduced to 3 Gy after 15 weeks due to severe skin reactions. In all, 27 skin and subcutaneous tumors were seen and collected. Of 12 sarcomas seen, 9 had a rhabdoid histology; cell lines from 3 such tumors as well as a squamous-cell carcinoma (SCC) and a malignant fibrous histiocytoma (MFH) were established. Immunohistochemical analysis of their parent tumors showed that the rhabdoid tumors expressed desmin, which established the diagnosis of RMS. Two-dimensional gel electrophoresis and Western analysis of insoluble protein extracts confirmed that the cell lines from RMS tumors expressed desmin. A screen for molecular alterations identified a mutant p53 phenotype for RMS and MFH cell lines. These radiation-induced RMS cell lines provide a unique opportunity to study the molecular biology of this tumor in an animal model and will help provide insight into the mechanisms of radiation-induced RMS in humans.

Animals↗
PubMed2003

Spinal epidural rhabdomyosarcoma.

We report an extremely rare case of rhabdomyosarcoma in the lower cervical and upper thoracic spine. The MR imaging appearance of the lesion was nonspecific, and different from the one previously reported. The majority of the tumor was removed surgically less than a month from the onset of symptoms. Treatment was continued with spinal irradiation and chemotherapy, however diffuse leptomeningeal metastases were found six months later.

Cervical Vertebrae↗
PubMed1999

Persistence of coxsackievirus B4 infection in rhabdomyosarcoma cells for 30 months. Brief report.

A persistent infection of rhabdomyosarcoma (RD) cells by Coxsackie B4 virus (CBV-4) was established. The persistently infected RD (piRD) cells have been maintained for over 130 passages (30 months) and have released virus continuously without cellular destruction. The production of infectious virus declined three times during the study. After the first decline (third week post infection) a viral variant with a littered cytopathic effect (CPE) and a marked delayed replication cycle on Green Monkey Kidney (GMK) cells, replaced the original viral population. 100-fold diluted cell cultures were recovered from the piRD cells at the 48(th) and 104(th) passage. All 96 cultures from the former whereas 72% from the second dilution showed virus production when tested on GMK cells. Using a streptavidin/biotin immune-staining assay all piRD cells were positively stained. Test for ts mutants showed that the persistence of the CBV-4 strain was not dependent upon incubation temperature and addition of the antiviral compound disoxaril did not cure the piRD cells.

Animals↗
PubMedDate not supplied

Induction of drug resistance in human rhabdomyosarcoma cell lines is associated with increased maturation: possible explanation for differentiation in recurrences?

In rhabdomyosarcoma (RMS) of childhood and adolescence very little is known about interactions of cytotoxic drugs and tumor cells. In recurrent RMS the tumor cells are often more mature than in the primary tumor. The biological properties of these cells are still a subject of controversy. We investigated two human (RD2 and TE 671) cell lines by cultivating them with doxorubicin, cisplatinum, and etoposide. Degree of differentiation and proliferation rate were estimated morphologically and by means of immunohistochemistry and a monolayer proliferation assay. Both morphological and immunohistochemical maturation was measurable in most resistant cell lines. An increase in myosin expression was most marked in the etoposide- and doxorubicin-resistant RD cell lines. The proliferation rate was decreased in almost all resistant cell lines. Nevertheless, the resistant cell lines tolerated high-dose levels of cytotoxic drugs at a higher proliferation rate than parental cell lines cultivated under similar conditions. The maturation seen in some recurrent tumors of RMS can be simulated in vitro by cultivating cell lines with cytotoxic drugs at sublethal doses. Interestingly, the resistance-associated induced maturation was not accompanied by p170 expression. After comparing these in vitro results with the maturation seen in RMS specimens after chemotherapy, we conclude that chemotherapy-induced differentiation in vivo might be a morphological sign of chemoresistance.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2004

Rhabdomyosarcoma arising in the hand or foot: a clinicopathologic analysis.

Rhabdomyosarcomas (RMS) arising at different anatomic sites have distinct clinicopathologic features that affect prognosis and outcome. RMS arising in the extremities generally have a worse prognosis. Case reports and anecdotal experience suggests the possibility that RMS of the hand or foot (RMSHF) may behave even more aggressively than RMS elsewhere in the extremities. This study describes clinicopathologic features and outcome of RMSHF. Charts and pathologic material from RMSHF cases from institutional files over a 32-year interval were reviewed. Additional cases from published reports and series over a 42-year interval were identified by a MEDLINE search. Pertinent clinicopathologic features for each case were compiled. Survival analysis and other data from these patients were compared with previously reported statistics in the literature for RMS at other anatomic sites, including elsewhere in the extremities. Six patients from the institutional files and 46 from published reports were identified. A number of adverse prognostic indicators were observed in the majority of these patients including alveolar morphology (86%) and distant metastasis at presentation (63%). Despite the aggressive nature of these tumors, the majority were smaller than 5 cm (61%). A propensity was observed for metastases to unusual sites such as breast, ovary, testis, pancreas, and kidney. The median survival was 19 months, and the 5-year survival was 27%. While these may reflect a constellation of findings related to site, it is also possible that alveolar histology is the main influence. RMSHF can present as a very small primary tumor with clinically evident or occult metastatic disease. Whether this reflects the location, the predominantly alveolar histologic type, or a molecular genetic subtype will require further analysis. Patients presenting with RMSHF should be thoroughly evaluated for occult metastases at the time of diagnosis and followed closely throughout treatment for subsequent involvement of unusual sites.

Adolescent↗
PubMedDate not supplied

Clinicopathologic study of ectomesenchymomas from Intergroup Rhabdomyosarcoma Study Groups III and IV.

Ectomesenchymomas (EM) are rare malignant neoplasms usually consisting of rhabdomyosarcoma (RMS) with a neural component. Only 21 cases have been previously reported. Here we extend the clinicopathologic spectrum of EM by describing our findings in 15 cases. Only 5 patients were infants; 10 were < or =3 years old and 5 were > or =6 years old. No male predilection was observed; 7 were female. The originating institutional diagnoses were; RMS (12), undifferentiated sarcoma (1), or EM (2), suggesting underdiagnosis of this entity. The primary tumor sites included external genital (5), pelvis/abdomen (6), head and neck (3), and extremity (1). The size of the primary neoplasm was usually > or =5 cm at diagnosis but dissemination only occurred in a minority. Local infiltration was not uncommon. These neoplasms were typically multilobate, thinly encapsulated, hemorrhagic, and necrotic. Light microscopic features were highly variable, but embryonal RMS with scattered or clustered ganglion cells, often in lacunae, was characteristic. In some cases, primitive neuroblastic or neuroectodermal areas were found and/or a component of alveolar RMS was seen. Focal anaplasia was occasionally observed. Mitotic activity appears higher than previously appreciated and some necrosis was invariably present. Electron microscopy was performed in 11 cases, which confirmed skeletal muscle +/- neural differentiation. Cytogenetic studies performed in five cases revealed no specific abnormality. Monoclonal neuron-specific enolase was the best marker of ganglion cells and primitive neural elements. MIC-2 (CD99) membrane expression was not definitively present in any of the six cases examined. A number of the above parameters appear to be of some prognostic significance, but overall, these neoplasms appear to have a similar outcome as would be predicted for their RMS element alone (exclusive of any neural component), with respect to the RMS subtype, age of the patient, and anatomic location of the neoplasm.

Abdominal Neoplasms↗
PubMed2004

Surgical management of gynecologic rhabdomyosarcoma.

The multidisciplinary treatment strategy for rhabdomyosarcoma (RMS) of the female gynecologic system is evidence of the progress made in the management of many childhood cancers. Previously, initial radical surgery (eg, complete vaginectomy, hysterectomy, and pelvic exenteration) and less effective single-agent chemotherapeutic agents were used with suboptimal results. Over the past 30 years, there has been an increase in overall survival with gynecologic RMS and a significant decrease in the hysterectomy and vaginectomy rates. The usual recommended treatment strategy for gynecologic RMS is initial biopsy to establish the diagnosis, followed by neoadjuvant chemotherapy. The specific chemotherapy regimen is selected based on the risk stratification of the particular patient, and this will often allow a more conservative surgical approach to be used, with the goal of complete tumor resection. Radiation therapy is considered before definitive surgical resection with minimal response to chemotherapy or after definitive surgical resection with positive margins. Recurrent or persistent RMS, including RMS of the female gynecologic system, represents a very aggressive disease with poor outcome. In these cases, aggressive management, including experimental chemotherapy agents and organ-sacrificing surgery, is often indicated to afford any chance of long-term survival. Continued enrollment of a high proportion of these patients into ongoing prospective clinical trials should allow the outcomes to continue to improve.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2006

Pediatric rhabdomyosarcoma of the head and neck.

Pediatric rhabdomyosarcoma is not exclusive to the head and neck. However, the unique anatomy of the head and neck requires special consideration and treatment modifications. The low incidence of these tumors has prevented the development of rigorous treatment protocols. Treatment strategies must be individualized on the basis of histopathologic subtype, prognostic indicators, tumor location, tumor extent, available clinical trial data, and hospital resources. The primary treatment of these tumors typically involves a combination of surgery, radiation, and chemotherapy. Advancements in surgical and radiotherapy techniques have reduced patient morbidity, whereas new chemotherapeutic protocols have improved local disease control and overall survival. Because of the infrequency and complexity of these tumors, patients may benefit from referral to centers with a comprehensive multidisciplinary team that has experience treating these tumors in the pediatric population. If possible, patients should be enrolled and treated on the current Children's Oncology Group protocol.

Biopsy↗
PubMed1994

Orbital rhabdomyosarcoma metastatic to the placenta.

Cancer coexisting with pregnancy is well reported; however, maternal malignancies that are metastatic to the products of conception are rare. We report a case of orbital rhabdomyosarcoma that metastasized to the placenta.

Adolescent↗
PubMed1980

Rhabdomyosarcoma.

The care of patients with rhabdomyosarcoma has undergone significant change in the past decade. Careful staging and multidisciplinary treatment have improved the prognosis for patients with this neoplasm. A review of the medical literature of the past decade documents these changes in staging, therapy and prognosis.

Adolescent↗
PubMed1989

The 34 kilodalton insulin-like growth factor binding proteins in human cerebrospinal fluid and the A673 rhabdomyosarcoma cell line are human homologues of the rat BRL-3A binding protein.

Three members of a family of insulin-like growth factor binding proteins have been identified by nucleotide sequencing of cDNA clones: the binding subunit of the 150 kDa IGF-binding protein complex in human serum, the 30 kDa IGF binding protein in human amniotic fluid, and a 30 kDa binding protein (BP-3A) isolated from the rat BRL-3A cell line. The present study demonstrates by molecular hybridization and immunoreactivity that the human counterpart of rat BP-3A is a 34 kDa IGF binding protein that is present in human cerebrospinal fluid and is synthesized and secreted by the A673 human rhabdomyosarcoma cell line.

Amniotic Fluid↗
PubMed1987

Relationship between binding affinity, retention and sensitivity of human rhabdomyosarcoma xenografts to Vinca alkaloids.

Xenografts of human rhabdomyosarcoma (RMS) have been derived that differ in their degree of sensitivity to Vinca alkaloids. Lines Rh12 and Rh18 demonstrated, respectively, high and moderate sensitivity to vincristine (VCR), but showed little responsiveness to vinblastine (VLB) in vivo. Rh18/VCR-3, a subline of Rh18 selected for resistance to VCR under in situ conditions, was insensitive to further challenge with VCR. Resistance was associated with elimination of the agent in a biphasic manner, whereas sensitivity to VCR corresponded to very prolonged drug retention in sensitive neoplastic tissues. The initial half-times for drug retention in tumors in vivo (t1/2 alpha) correlated with the degree of sensitivity of tumors to Vinca alkaloids, decreasing t1/2 alpha being associated with decreased sensitivity. A single binding species was observed when membrane-free supernatant fractions were incubated at 37 degrees for 15 min with 10.4 nM [3H]VCR and analyzed by gel filtration HPLC. The protein eluted with a retention time of 57 min and corresponded to a molecular weight (Mr) of approximately 113,000 daltons, agreeing very closely with the Mr of dimeric tubulin (approximately equal to 110,000 daltons). Two fractions were collected and eluted on a one-dimensional denaturing gel. Proteins were transferred subsequently to nitrocellulose and probed with an 125I-labeled monoclonal antibody specific for beta-tubulins. Only the fraction containing bound [3H]VCR contained tubulin. Estimates for the dissociation constants (Kd) for the binding affinity of VCR and VLB in crude, membrane-free supernatant fractions from RMS xenografts were obtained by computer curve fitting using a mathematical binding model. Data fitted a two-site binding model, with Kd values for the high-affinity site ranging from 61 to 160 nM, and for the low-affinity site, from 42 to 94 microM. At physiologically achievable drug concentrations, the relationship between binding affinity, drug retention and tumor sensitivity was examined further. A close relationship was apparent between the Kd values for VCR in Rh12, Rh18 and Rh18/VCR-3 tumor supernatant fractions and VLB in Rh12 preparations, and t1/2 alpha values for drug retention. Prolonged drug retention correlated with a low binding constant. As t1/2 alpha decreased, binding affinity also decreased, as demonstrated by an increase in the Kd value. Consequently, the tightness of drug binding in tumors also correlated with the degree of sensitivity of the xenografts to Vinca alkaloids.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗
PubMed1989

Laminin-induced capping and receptor expression at cell surface in a rat rhabdomyosarcoma cell line: involvement in cell adhesion and migration on laminin substrates.

The present study reveals the dynamic distribution of membrane laminin receptors induced by laminin binding in a rat rhabdomyosarcoma cell line RMS S4. The treatment of the cells with soluble laminin did not modify cell adhesion to laminin-coated substrates in in vitro attachment assays. Fluorescent labeling of membrane-bound laminin revealed that occupied receptors were induced to cluster and cap. New free membrane binding sites were made evident after capping of bound laminin by a double labeling technique. Cytochalasin D (CD) treatment prevented the capping process. The adhesion of CD-treated cells to laminin-coated substrates was inhibited by cell preincubation with soluble laminin. Cycloheximide treatment had no effect on the ability of RMS S4 cells to adhere to adsorbed laminin after preincubation in the presence of soluble laminin. These results taken as a whole suggest that free receptors may arise from an intracellular pool that could be maintained by membrane receptor recycling. Since capping and motility seem related events, migration of RMS S4 cells on laminin was studied in the agarose drop assay. Immobilized laminin stimulated basic cell motility by more than 200%. E8 laminin fragment retained partially the motility stimulating property of laminin while P1 pepsinic fragment had no effect. The presence of constantly available receptors at the cell surface could be determinant in the ability of cells to migrate on laminin substrates.

Animals↗
PubMed1992

Tumorigenicity-associated expression of protein kinase C isoforms in rhabdomyosarcoma-derived cells.

Cell sublines were derived from Ni-induced rat rhabdomyosarcoma which differed in their degree of tumorigenicity. We compared the expression of protein kinase C isoforms alpha, beta, gamma and epsilon in three sublines developing tumors in syngeneic rats (Sy+) and in two sublines devoid of tumorigenicity in these animals (Sy-), with that of normal skeletal muscle. Northern and Western blotting experiments showed that PKC alpha was dramatically overexpressed in Sy- cells whereas it was underexpressed in Sy+ cells. Southern blot analysis provided evidence for a 3-fold increase of PKC alpha-related DNA fragments in Sy- cells. Steady-state levels of the PKC epsilon-related transcript were also markedly decreased in Sy+ cells. However, the expression of PKC beta-related RNA was increased in these cells. Our data support the concept that the differential expression of the PKC isoforms may play a critical role in determining the neoplastic phenotype.

Animals↗