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Alpha-fetoprotein producing rhabdomyosarcoma of the adult liver.

An autopsy case of a rare primary tumor in a 76-year-old man is described. The tumor is histologically comprised of rhabdomyoblasts massively infiltrating without any epithelial elements referable to be as hepatoma. There was no evidence that regarded the hepatic tumor as metastatic secondary tumor in histological examination of other organs. Furthermore, interesting was the production of alpha-fetoprotein from the tumor cells that was detected by immunofluorescent antibody technique. Review of the literature on primary liver rhabdomyosarcomas or adult hepatoblastomas shows no similar case.

Aged↗

Embryonal rhabdomyosarcoma in nude mice and in vitro.

A transplantable tumor strain designated YNnu and a cultured cell line designated YN were established from the human paratesticular rhabdomyosarcoma of a 15-year-old boy. In vitro the cells showed ultrastructural features of immature mesenchymal cells, and a few cells were labeled by antibodies to myoglobin and/or desmin. In nude mice, the cells became to contain numerous myofibrils with Z-bands, and considerable number of cells reacted with anti-myoglobin and/or anti-desmin antibodies. We conclude that spindle-shaped or small round cells are the most immature rhabdomyoblast with multiplicity for cellular differentiation.

Adolescent↗

Esophageal carcinosarcoma with basaloid squamous carcinoma and rhabdomyosarcoma components with TP53 mutation.

Carcinosarcoma of the esophagus is a rare tumor with a distinct pathological entity having squamous cell carcinoma as the most described carcinomatous component. This paper reports the first case of carcinosarcoma of the esophagus that showed predominant basaloid squamous carcinoma component in addition to squamous cell carcinoma and poorly differentiated carcinoma and sarcoma component. A 64-year-old male patient consulted for dysphasia and chest pain was examined and found to have gastrointestinal fiber-endoscope and a polypoid growth in the lower third of the esophagus. Partial esophagectomy was performed and the excised tumor showed histological features of carcinosarcoma with heterogeneous carcinomatous components with dominance of basaloid squamous carcinoma and minority of squamous cell carcinoma, poorly differentiated carcinoma, and sarcomatous component, immunohistochemically proven to be rhabdomyosarcoma. Immunohistochemical study and TP53 mutation analysis was carried out to explain the histogenesis of this rare tumor. The distinct immunohistochemical profiles of the carcinomatous and sarcomatous components suggested the possibility of transition from a carcinomatous to a sarcomatous component. The similar TP53 mutation in the carcinomatous and sarcomatous component suggested each of these components had the same origin, that is, the tumor was monoclonal in origin.

Biomarkers, Tumor↗

Rhabdomyosarcoma in a patient treated for metastatic germ cell tumour of the testis containing teratoma--a case report.

A patient who developed a rhabdomyosarcoma following apparently successful chemotherapy for metastatic germ cell testicular carcinoma is presented. This newly recognized association may be seen particularly in patients whose initial germ cell malignancy contains immature teratoma. Possible reasons for this are discussed. The findings in this patient suggest that re-biopsy of recurrent disease be undertaken wherever possible, particularly where immature teratoma was a feature of the initial histopathology. A proportion of relapsing patients as described may not in fact have recurrent germ cell malignancy, but may have developed high grade, and often chemoresistant sarcomas. These second tumours appear to have an extremely poor prognosis, unless amenable to complete surgical resection.

Adult↗

Transcriptomic and proteomic analyses of rhabdomyosarcoma cells reveal differential cellular gene expression in response to enterovirus 71 infection.

Insights into the host antiviral strategies as well as viral disease manifestations can be achieved through the elucidation of host- and virus-mediated transcriptional responses. An oligo-based microarray was employed to analyse mRNAs from rhabdomyosarcoma cells infected with the MS/7423/87 strain of enterovirus 71 (EV71) at 20 h post infection. Using Acuity software and LOWESS normalization, 152 genes were found to be downregulated while 39 were upregulated by greater than twofold. Altered transcripts include those encoding components of cytoskeleton, protein translation and modification; cellular transport proteins; protein degradation mediators; cell death mediators; mitochondrial-related and metabolism proteins; cellular receptors and signal transducers. Changes in expression profiles of 15 representative genes were authenticated by real-time reverse transcription polymerase chain reaction (RT-PCR), which also compared the transcriptional responses of cells infected with EV71 strain 5865/Sin/000009 isolated from a fatal case during the Singapore outbreak in 2000. Western blot analyses of APOB, CLU, DCAMKL1 and ODC1 proteins correlated protein and transcript levels. Two-dimensional proteomic maps highlighted differences in expression of cellular proteins (CCT5, CFL1, ENO1, HSPB1, PSMA2 and STMN1) following EV71 infection. Expression of several apoptosis-associated genes was modified, coinciding with apoptosis attenuation observed in poliovirus infection. Interestingly, doublecortin and CaM kinase-like 1 (DCAMKL1) involved in brain development, was highly expressed during infection. Thus, microarray, real-time RT-PCR and proteomic analyses can elucidate the global view of the numerous and complex cellular responses that contribute towards EV71 pathogenesis.

Cell Line, Tumor↗

Pelvic rhabdomyosarcoma in children.

Twenty children with pelvic rhabdomyosarcoma treated between 1976 and 1983 are reviewed. The survival rate was 55% (11/20), with a bladder salvage rate of 54.5% among survivors. Systemic chemotherapy combined with radiotherapy and/or surgery was most effective in producing cure.

Adolescent↗

Thirty-year review of intrascrotal rhabdomyosarcoma.

Between 1957 and 1987, 6 cases of intrascrotal rhabdomyosarcoma were found in the pathology records for Northern Ireland. Four of these tumours arose from paratesticular tissue and 2 were confined to the testis. Only 1 patient has died of his disease. Two were lost to follow-up after 21 years and are presumed cured. The remaining 3 remain alive and disease-free between 2 and 3 1/2 years after presentation.

Adolescent↗

Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery.

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. Twenty of the patients received primary polychemotherapy (vincristine, actinomycin D, cyclophosphamide, adriamycin, and more recently including etoposide and ifosfamide), two had primary surgery and seven had additional radiotherapy. Fourteen patients had radical cystoprostatectomy, with continent cutaneous urinary diversion with an ileocaecal pouch in seven, in one each a transverse colonic pouch, orthotopic ileocaecal bladder substitution, a rectal reservoir and rectosigmoid pouch and a colonic conduit diversion in two patients. RESULTS: After a mean (range) follow-up of 8.6 (1.0-26) years, 17 patients had no evidence of disease. Five patients presenting initially with advanced tumour stages died from progressive RMS. Two patients with a continent urinary diversion required ureteric reimplantation for stenosis. In two patients severe bladder contraction after radiotherapy required bladder augmentation. CONCLUSION: Primary chemotherapy followed by radical surgery of RMS of the prostate and/or bladder allows complete tumour resection in most cases, and yields excellent cure rates.

Adolescent↗

Alveolar rhabdomyosarcoma arising in the nasal cavity of a 3-year-old child.

A 3-year-old girl had a large exophytic mass protruding from the nares, of seven months' duration. Routine microscopic studies demonstrated a tumor composed of solid sheets of small cells, with scattered foci lining alveola-like spaces. Immunohistochemical studies demonstrated the presence of desmin and myoglobin, and the absence of prekeratin, neuron-specific enolase, and leukocyte common antigen. These observations are consistent with the diagnosis of alveolar rhabdomyosarcoma.

Child, Preschool↗

Acquired ichthyosis in a child with rhabdomyosarcoma.

A generalized ichthyosiform eruption developed in a 13-year-old boy three weeks before presentation with a paravertebral rhabdomyosarcoma. His skin returned to normal shortly after the onset of chemotherapy and remission of the tumor. This case is unusual because the ichthyosis developed acutely, following a generalized urticarial eruption, and also involved the palms and soles.

Adolescent↗

Solid alveolar rhabdomyosarcoma of the hand in adolescence: a clinical, histologic, immunologic, and ultrastructural study.

Rhabdomyosarcoma (RMS), a high-grade, malignant, skeletal muscle tumor, represents approximately 5% of neoplasms in children. The poorly differentiated forms of RMS are often not easily diagnosed and classified. Among the four histologic variants, alveolar RMS is the least frequently reported subtype. A poorly differentiated solid variant of alveolar RMS occurred on the right hand of a 16-year-old girl. Because of the tumor size, local invasiveness, and occurrence of cutaneous and breast metastases at presentation, the clinical staging was group IV (T2/NO/M1). Surgical excisions of the primary and metastatic locations were performed and chemotherapy with vincristine, dactinomycin, cyclophosphamide, and doxorubicin was administered. Light and electron microscopy studies revealed a solid proliferation with a focal alveolar pattern of monomorphous, small, round neoplastic cells without easily detectable muscular morphologic features. The skeletal muscle origin was revealed by the positive immunostaining for desmin, alpha-sarcomeric actin, muscle-specific actins, and enolase, and confirmed by immunoblotting for desmin. Despite the age of our patient, which is considered by some authors an independent predictor of outcome, all prognostic variables were unfavorable. However, a disease-free interval during three years of follow-up underlines the importance of multidisciplinary regimens for the treatment of this rare solid tumor of childhood and adolescence.

Adolescent↗

Improvement of Clostridium tumour targeting vectors evaluated in rat rhabdomyosarcomas.

Previous studies have demonstrated the feasibility of using apathogenic clostridia as a promising strategy for hypoxia-specific tumour targeting. The present study shows that the use of the vascular targeting compound combretastatin A-4 phosphate could significantly (P<0.001) increase the number of Clostridium vegetative cells in rat rhabdomyosarcomas with sizes between 0.2 cm(2) and 3 cm(2). Furthermore, this study showed that administration of metronidazole for a 9-day period was sufficient to eliminate systemically administered Clostridium from the tumour. Moreover, previous Clostridium spore administration did not effect tumour colonisation, regardless of the immune response status of the host.

Animals↗

Detection of anti-acetylcholine receptor antibody by an ELISA using human receptor from a rhabdomyosarcoma cell line.

A simple and reliable enzyme-linked immunosorbent assay (ELISA) has been developed for the measurement of anti-acetylcholine receptor (AChR) antibodies. The test utilizes a membrane-bound AChR obtained from a human rhabdomyosarcoma cell line (TE671) as antigen and employs an affinity-purified rabbit anti-human immunoglobulin G alkaline phosphatase-conjugated antibody as labelled antibody. To assess the sensitivity and the specificity of our assay we tested serum samples from 13 anti-AChR antibody-positive myasthenia gravis (MG) patients known to contain between 2 and 120 nmol/l of anti-AChR antibody, three anti-AChR antibody-negative MG patients, and 70 control subjects including patients with other neurological and autoimmune diseases. A panel of six different anti-AChR monoclonal antibodies and membranes from a AChR-negative rat adrenal pheochromocytoma cell line (PC 12) were also used in competitive studies. The test showed to be specific and able to detect as low as 2.0 nmol/l of anti-AChR antibodies. Moreover, we found a good correspondence between anti-AChR antibody levels measured in the serum samples tested by our assay and levels measured by the routinely adopted radioimmuno assay (RIA) using human-AChR (r = 0.96). Cross-reaction phenomena were observed only using serum samples containing high-titer anti-DNA antibodies. The proposed ELISA, circumventing the limitation of the commonly used RIA (radioactivity and amputated legs as source of human antigen), can be considered as an useful screening test for the diagnosis of myasthenia gravis.

Antibodies, Monoclonal↗

In vitro and in vivo consequences of VLA-2 expression on rhabdomyosarcoma cells.

Cloned integrin alpha 2 subunit complementary DNA was expressed on human rhabdomyosarcoma (RD) cells to give a functional VLA-2 (alpha 2 beta 1) adhesion receptor. The VLA-2-positive RDA2 cells not only showed increased adhesion to collagen and laminin in vitro, but also formed substantially more metastatic tumor colonies in nude mice after either intravenous or subcutaneous injection. These results show that a specific adhesion receptor (VLA-2) can markedly enhance both experimental and spontaneous metastasis. In contrast to the metastasis results, there was no difference in either the in vitro growth rate or apparent in vivo tumorigenicity of RD and RDA2 cells.

Animals↗

Persistent Varicella-Zoster virus infection in a human rhabdomyosarcoma cell line and recovery of a plaque variant.

Varicella-zoster virus (VZV) has been found to persistently infect the human rhabdomyosarcoma cell line A204. Infectious center assays and fluorescent antibody staining demonstrated continuous production of infectious VZV and viral antigen. The level of infection determined by fluorescent antibody staining was variable, and usually only a small percentage of the cells were capable of producing plaques in permissive fibroblasts. The extent of infection was similar in cell cultures passaged at split ratios of 1:2 or 1:10 and grown at 33 or 37 degrees C. VZV recovered from A204 cells several months after establishment of the persistent infection had markedly increased syncytia-forming activity as compared with the parental VZV grown in human diploid fibroblast cells and the three monkey kidney-derived cell lines Vero, CV-1, and MA104. The expression of this altered phenotype continued after serial passage of the cell-associated virus in human diploid fibroblast and Vero cells. Consequently, we designated the reisolated VZV as plaque variant A. The buoyant densities of VZV plaque variant A and VZV DNAs in CsCl gradients were indistinguishable.

Antigens, Viral↗

Sensitivity of rhabdomyosarcoma and guinea pig embryo cell cultures to field isolates of difficult-to-cultivate group A coxsackieviruses.

Forty-two difficult-to-cultivate group A coxsackieviruses (i.e., group A types other than A7, A9, and A16), collected primarily from throat swab specimens of patients suffering from fever, pharyngitis, lymphadenopathy, and cough during the 1986 enterovirus season, were isolated in less than 24-h-old suckling mice. Thirty-six moribund mice were sacrificed and autopsied, and then their brains and back musculature were inoculated into rhabdomyosarcoma (RD), guinea pig embryo (GPE), rhesus monkey kidney (RhMk) and human carcinoma of the larynx (HEp-2) cell cultures. Twelve of the 36 suckling mice isolates were adapted to grow in RD and GPE cells after two passes and have been identified in RD cells by type-specific antisera as group A coxsackievirus types A2, A4, and A8. Three passes in RhMk or HEp-2 cell cultures were insufficient to affect a discernible cytopathic effect. Coxsackievirus types A1, A19, and A22, unable to grow in any of the four cell cultures tested, were identified by virus neutralization in suckling mice. These data denote the efficacy of suckling mice for the isolation of difficult-to-cultivate group A coxsackieviruses.

Animals↗

Comparison of shell vials and conventional tubes seeded with rhabdomyosarcoma and MRC-5 cells for the rapid detection of herpes simplex virus.

Shell vials (SV) and conventional tubes (CT) were seeded with rhabdomyosarcoma (RD) and MRC-5 cells and inoculated with clinical specimens, and the systems were evaluated for the rapid diagnosis of herpes simplex virus (HSV) infections by detection of cytopathic effects (CPE) (for CT, for 7 days) and by using fluoresceinated monoclonal antibodies (for SV, 16 h postinoculation). Of 245 genital specimens (16 from males and 229 from females) 56 (23%) seeded with MRC-5 cells (14 type 1 and 42 type 2) and 55 (22%) seeded with RD cells were detected in CT; however, CPE were recognized in only 26 (46%) of the total HSV-positive cultures 1 day postinoculation. Forty-eight (86% sensitivity, MRC-5) and 46 (84% sensitivity, RD) HSV strains were detected immunologically in SV 16 h postinoculation. Early CPE in CT or fluorescent foci in SV were easier to detect in MRC-5 than in RD cell cultures. MRC-5 and RD cells were equally sensitive to infection with HSV. CT cell cultures were more sensitive than SV but less rapid for the detection of HSV infection (P less than 0.01). We recommend using SV for the rapid diagnosis of HSV infections, but in addition, CT must be inoculated with MRC-5 or RD to ensure maximum detection of this virus.

Animals↗

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