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At least 973 records · Page 54Linked to original sources
PubMed2006

Teratoma in an adolescent with malignant transformation into embryonal rhabdomyosarcoma: case report.

BACKGROUND: The somatic type tumors are occasionally found in nonseminomatous germ cell tumors in men. These malignancies are presumed to arise from malignant transformation (MT) of teratoma or by differentiation of totipotential germ cell. OBSERVATION: A case of MT of germ cell tumor in 17-year-old male into embryonal rhabdomyosarcoma is described. The histopathologic diagnosis was that of embryonal rhabdomyosarcoma in which no germ cell elements were found. The germ cell origin of transformed histology is supported by cytogenetic analysis (isochromosome 12p), and elevated alpha(1)-fetoprotein. Despite intensive therapy the patient died. CONCLUSIONS: MT of teratoma is rare entity with poor prognosis.

Adolescent↗
PubMed1989

Congenital alveolar rhabdomyosarcoma presenting as a blueberry muffin baby.

Congenital alveolar rhabdomyosarcoma in a newborn was reported. She had multiple subcutaneous tumors and looked like a blueberry muffin baby. From the clinical signs and the histological pictures, a diagnosis of neuroblastoma was considered. Laboratory data showed no abnormalities. Electron microscopic examination revealed myofilament and incipient stria formation. On the basis of these data, a final diagnosis of congenital alveolar rhabdomyosarcoma associated with multiple skin metastases was made.

Diagnosis, Differential↗
PubMed1998

Characterization of a new human embryonal rhabdomyosarcoma cell line, RMS-GR.

A human tumor cell line designated RMS-GR was established from an embryonal rhabdomyosarcoma. The monolayer cells were polygonal, round or spindle-shaped. The RMS-GR cell line became stable with a doubling time of 42 h. Tumorigenicity of the cells was confirmed by heterotransplantion into nude mice. Electron microscopic images showed typical cytoplasmic inclusion of aggregated intermediate filaments and myofibril-like thin filaments. The expression of desmin, vimentin, actin and human myoglobin was recognized by cytofluorometric analyses, and a large fraction of CK-MM and small fractions of CK-BB and MCK-1 isoenzymes were found. Chromosomal analysis showed that the modal chromosome number was consistently near triploid with structural abnormalities mostly involving chromosomes 1, 3 and 8, and additional unidentified markers. No alteration of chromosome 2 was observed. The RMS-GR cell line may provide a system to identify genes which are involved in the pathogenic mechanism of rhabdomyosarcomas, and to investigate the modulation of myogenic differentiation.

Actins↗
PubMed1999

Multidrug resistance phenotype in the RMS-GR human rhabdomyosarcoma cell line obtained after polychemotherapy.

Classical cytotoxic treatment of rhabdomyosarcoma (RMS), the most common soft tissue malignacy in children, is often accompanied by significant morbidity and poor response. Chemotherapy may induce multidrug resistance (MDR) associated with the expression of P-glycoprotein, a drug efflux pump which modifies the sensitivity of tumoral cells to drugs. To analyze MDR in RMS we used the RMS-GR cell line, obtained from an embryonal rhabdomyosarcoma treated in vivo with polychemotherapy. The RMS-GR cells showed cross-resistance to vincristine, doxorubicin and actinomycin D, the drugs of choice in the conventional treatment of RMS. Polymerase chain reaction (PCR) analysis showed that these RMS cells overexpressed mdr1/P-glycoprotein. The pattern of resistance and the level of P-glycoprotein expression were similar to those found in the resistant RMS TE.32.7.DAC cell line obtained in vitro. Southern blot analysis showed that mdr1 overexpression was not due to amplification of the gene. Our results showed that the in vivo treatment of embryonal RMS may induce an MDR phenotype mediated by mdr1/P-glycoprotein in RMS cells.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed2000

Modulation of myogenic differentiation in a human rhabdomyosarcoma cell line by a new derivative of 5-fluorouracil (QF-3602).

The in vitro study of mechanisms involved in drug-induced maturation has made it possible to use differentiation-based therapy in clinical practice. The goal of this new therapy is the development of specific agents to induce cancer cells to stop proliferating and express characteristics of normal cells. Recently, by structural modifications of 5-fluorouracil (5-FU), we synthesized a new pyrimidine acyclonucleoside-like compound, 1-¿[3-(3-chloro-2-hydroxypropoxy)-1-methoxy]propyl¿-5-fluorouracil (QF-3602), which showed in rhabdomyosarcoma cells a low toxicity and time-dependent growth inhibition. In this work, we compared the degree of myogenic differentiation of RD rhabdomyosarcoma (RMS) cells after treatment with QF-3602 and 5-FU. Scanning and transmission electron microscopy (SEM and TEM) and immunocytochemical analyses showed that QF-3602 induced the appearance of myofilaments along the myotube-like giant RD cells, an increase in fibronectin and a decrease in vimentin expression. In contrast, only minor changes were observed with 5-FU. Moreover, polymerase chain reaction (PCR) analyses showed that QF-3602 did not induce overexpression of the mdr 1 gene, a resistance mechanism that frequently appears in classical cytotoxic therapy in these tumors. Compounds obtained by structural modifications of 5-FU may be useful in differentiation therapy as a new approach to the treatment of RMS.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed2002

Expression of cyclin-dependent kinase inhibitor p27/Kip1 and AP-1 coactivator p38/Jab1 correlates with differentiation of embryonal rhabdomyosarcoma.

Cyclin-dependent kinase (CDK) inhibitor p27/Kip1 (p27) is a diagnostic and prognostic marker of various malignancies. Low expression of p27 reflects poor differentiation and poor prognosis, and an inverse correlation between the expression of p27 and degree of tumor malignancy has been reported. Because p27 mutation is extremely rare in human tumors, it is important to study the expression of p27 and its inactivator, p38/Jab1 (JAB1). Here we analyzed the expression of p27 and JAB1 by immunohistochemistry in embryonal rhabdomyosarcoma (E-RMS). We first confirmed the expression of p27 and JAB1 in normal human tonsillar epithelium, and observed a coordinated expression pattern depending on cell differentiation. Subsequently, specimens of eight poorly- and three well-differentiated E-RMS were examined for the expression of p27 and JAB1. The analyses revealed that four out of eight poorly-differentiated E-RMS were negative for p27, with positivity for nuclear JAB (NJAB) (- / + for p27/NJAB) in three and negativity for any JAB-1 expression ( - / -) in one. The remaining four poorly-differentiated E-RMS expressed p27 in the nuclei, together with predominant NJAB (+ / +). In three well-differentiated E-RMS, only one expressed nuclear p27 and all of these three expressed no NJAB (+ / - for p27/NJAB), but expressed predominant cytoplasmic JAB1 (CJAB). These findings suggest that JAB1 may play an important role in determining the differentiation stage of rhabdomyosarcoma cells by modulating the activity of CDK inhibitor p27.

COP9 Signalosome Complex↗
PubMed2004

Paediatric rhabdomyosarcoma of the ear and temporal bone.

The objective of the present study was to review the presentation, management, outcome and morbidity of paediatric patients presenting to a single centre with rhabdomyosarcoma of the ear and temporal region. All patients diagnosed with rhabdomyosarcoma of the ear and temporal region between 1980 and 2000 were entered into this retrospective study. Fourteen patients were identified. The median age at presentation was 4.5 years with a mean time of onset of symptoms to diagnosis of 21 weeks. In many patients, the presentation mimicked that of chronic otitis media, delaying diagnosis. Histological subtype was embryonal in 13 patients and alveolar in 1. All patients underwent multimodality treatment. The 5-year disease-free survival rate was 81%. Regional post-treatment morbidity included chronic aural discharge (6/14), facial palsy (8/14), growth disturbance (4/14) and maxillo-facial deformity occurring in four children. From the results, we conclude that these patients should usually present to an ENT surgeon who should keep the diagnosis in mind when dealing with children with chronic otitis media as early diagnosis with referral to a specialist multidisciplinary team will optimize the chance of survival. Discharge, hearing loss and aural polyp, although commonly because of chronic otitis media, should prompt urgent investigation and biopsy, particularly if associated with facial palsy, lymphadenopathy or an obvious mass.

Bone Neoplasms↗
PubMed2006

Rhabdomyosarcoma of the mandible in a 6-year-old boy.

INTRODUCTION: Rhabdomyosarcoma is an aggressive malignant tumour composed of neoplastic mesenchymal cells that infiltrate surrounding tissue structures, making their precise site of origin unclear. Although rare, this is highly aggressive and the most common soft-tissue neoplasm of the head and neck in children. Regrettably by the time most cases are initially seen, the patients already have large tumours, due to rapid tumour growth and delayed medical consultation. CASE REPORT: This report describes a 6-year-old presenting with just such symptoms of facial swelling and pain but elicitation of further information and findings, including tooth mobility of 3 days duration, led to prompt referral and early treatment of an embryonal rhabdomyosarcoma. CONCLUSION: General dental practitioners are frequently presented with a child with a swollen face and pain. Experience would suggest a dental abscess to be the most likely cause with treatment as appropriate. However, all swellings in children, should be thoroughly investigated and reviewed as particularly in this age group, tumour growth is rapid while early diagnosis allows successful treatment with multimodality therapy.

Biopsy↗
PubMed1981

Induction by butyrate of differentiated properties in cloned murine rhabdomyosarcoma cells.

A cell line derived from the murine rhabdomyosarcoma BW10139 (Dexter, Cancer Res. 37: 3136, 1977) was subcloned and examined with respect to growth and myogenic characteristics in the presence and absence of 1 mM butyrate. Without butyrate, these cells behave as typical transformed cells: they grow rapidly and chaotically, do not form multinucleated muscle fibers and have little or no creatine kinase activity. In the presence of 1 mM sodium butyrate or butyric acid, growth slows, cells become arranged in whorl patterns, and creatine kinase activities increase to levels comparable to those found in normal chick myoblasts immediately prior to cell fusion. The increase in creatine kinase activity is detectable within 2 h exposure to butyrate, reaches a maximum by 24 h, and the elevated level can be maintained for at least six weeks. The induction is reversible upon sequential addition, deletion, and readdition of butyrate to the culture medium. Isoenzyme analyses demonstrated that only the BB form of creatine kinase is induced; MM creatine kinase was not detected. Although formation of multinucleated cells increases after exposure to butyrate, no typical myotubes form. The results suggest that this rhabdomyosarcoma cell line can, under appropriate conditions, re-express some properties characteristic of skeletal muscle, but not the complete muscle phenotype.

Animals↗
PubMed2006

Auricular rhabdomyosarcoma in a rat.

An ear (auricular) neoplasm from a 1-year-old male rat was removed surgically and examined histologically. Macroscopically, the neoplasm was firm, white and measured (0.5 x 0.5 cm). Microscopically, the neoplasm was expansile, non-encapsulated, and composed of large, pleomorphic, polygonal to spindle-shaped cells containing multiple nuclei. Using immunohistochemical and chemical stains, the neoplastic cells were positive for vimentin, myoglobin, phosphotungstic acid haematoxylin and desmin, but had no immunoreactivity for cytokeratin or alpha-smooth muscle actin. On the basis of histopathological, immunohistochemical and histochemical stains, a diagnosis of auricular rhabdomyosarcoma was made. Although reported infrequently in human, this is, to the author's knowledge, the first report that describes the detailed gross, histopathological, histochemical and immunohistochemical findings of auricular rhabdomyosarcoma in a rat.

Animals↗
PubMed1988

Congenital alveolar rhabdomyosarcoma with multiple skin metastases. Report of a case.

A rare case of congenital alveolar rhabdomyosarcoma revealing multiple skin metastases in a female neonate is reported. At birth, a ping-pong ball-sized tumor on the neck and a tumor the size of a little finger end on the chin were noticed. Then, multiple skin tumors over the whole body occurred soon after birth. A biopsied small skin tumor was at first interpreted as being compatible with metastatic congenital neuroblastoma. However, immunohistochemical and electron microscopic findings revealed positive immunoreactivity for myoglobin in a few tumor cells and the presence of a few rhabdomyoblasts among poorly differentiated tumor cells, resulting in a final diagnosis of alveolar rhabdomyosarcoma. Therefore, it should be emphasized that in cases of round cell tumor, immunohistochemical and ultrastructural studies are imperative in order to identify the tumor and differentiate it from other forms, including rhabdomyoblastoma, neuroblastoma, Ewing's sarcoma, malignant lymphoma, and small cell carcinoma.

Alveolar Process↗
PubMed1991

A testicular teratoma with rhabdomyosarcoma and seminoma.

A case of testicular mixed germ cell tumor with teratoma in a 36-year-old Japanese male is reported. Histologically, it was strongly suggested that the rhabdomyosarcoma had originated from the mesenchymal element of the teratoma. A review of the literature revealed six definitive and two possible previous cases of testicular rhabdomyosarcoma with teratoma, either with or without other germ cell malignancies. However, the present case associated with seminoma as a germ cell tumor is the first of its kind to be reported. In contrast to the poor prognosis observed in the previous cases, the present patient remains free of disease 3 years and 3 months after orchiectomy, chemotherapy and irradiation.

Adult↗
PubMed1992

Rhabdomyosarcoma with focal cartilaginous differentiation (malignant mesenchymoma) of the inferior vena cava.

A sarcoma arising from the inferior vena cava occupied the entire lumen of the inferior vena cava, right atrium, hepatic veins and common iliac veins. Its histological appearance was non-specific sarcoma, except for the presence of a few rhabdomyoblasts and some immature cartilaginous tissue. Immunohistochemically, some tumor cells were positive for myoglobin, desmin, HHF-35, and vimentin. Electron microscopy revealed that some tumor cells contained myofilaments and Z bands in the cytoplasm, which are characteristics of rhabdomyosarcoma. The tumor was diagnosed as rhabdomyosarcoma with focal cartilaginous differentiation (malignant mesenchymoma) of the inferior vena cava.

Aged↗
PubMed2005

Metastatic germ cell tumor of the lung masquerading as primary rhabdomyosarcoma.

Two years after testicular resection was carried out in a 40-year-old man that revealed mixed germ cell tumor of more than one histological type (seminoma, embryonal cell carcinoma, and yolk sac tumor), he presented with an asymptomatic pulmonary nodule in his left lower lobe. Video-assisted thoracoscopic partial resection of the tumor revealed a 24 x 20 mm teratoma with somatic-type malignancy in which pleomorphic rhabdomyosarcoma was a major element. One year later, asymptomatic tumor recurrence occurred at both edges of the stapler line as 22 x 20 mm and 10 x 5 mm nodules composed only of pleomorphic rhabdomyosarcoma. Throughout the course there was no abdominal lymph node swelling detected by computed tomography (CT) and tumor markers were normal. Adjuvant chemotherapy was started after the tumor recurrence. Currently, the patient is still undergoing chemotherapy 5 months after the tumor recurrence. In conclusion, despite the fact that primary pulmonary rhabdromyosarcoma is a rare neoplasm, metastatic pulmonary germ cell tumor with somatic-type malignancy showing predominantly rhabdomyosarcomatous differentiation should be considered in the differential diagnosis of such lesions of the lung.

Adult↗
PubMed2006

Identification of novel genes expressed during rhabdomyosarcoma differentiation using cDNA microarrays.

Rhabdomyosarcomas (RMS) are highly aggressive tumors that are thought to arise as a consequence of the regulatory disruption of the growth and differentiation of skeletal muscle progenitor cells. Normal myogenesis is characterized by the expression of the myogenic regulatory factor gene family but, despite their expression in RMS, these tumor cells fail to complete the latter stages of myogenesis. The RMS cell line RD-A was treated with 12-O-tetradecanoylphorbol-13-acetate to induce differentiation and cultured for 10 days. RNA was extracted on days 1, 3, 6, 8 and 10. A human skeletal muscle cDNA microarray was developed and used to analyze the global gene expression of RMS tumors over the time-course of differentiation. As a comparison, the genes identified were subsequently examined during the differentiated primary human skeletal muscle cultures. Prothymosin alpha (PTMA), and translocase of inner mitochondrial membrane 10 (Tim10), two genes not previously implicated in RMS, showed reduced expression during differentiation. Marked differences in the expression of PTMA and Tim10 were observed during the differentiation of human primary skeletal muscle cells. These results identify several new genes with potential roles in the myogenic arrest present in rhabdomyosarcoma. PTMA expression in RMS biopsy samples might prove to be an effective diagnostic marker for this disease.

Adult↗
PubMed1998

Prognostic value of proliferating cell nuclear antigen immunostaining in pediatric rhabdomyosarcomas.

BACKGROUND: The levels of proliferating cell nuclear antigen (PCNA) are almost negligible in long-term quiescent cells and increase dramatically during the cell cycle. Recently, the monoclonal antibodies to PCNA have been used to demonstrate the proliferative component of paraffin-embedded tumor tissues. It has been shown to be available as a simple histological marker of proliferative activity and the PCNA labeling index has been correlated with the prognosis of several malignant neoplasms. METHODS: Formalin fixed, paraffin embedded tissue specimens of 29 primary pediatric rhabdomyosarcomas were immunostained by using an anti-PCNA monoclonal antibody (DAKO PCNA PC10). The relationship between the PCNA index and prognosis, clinicopathological features and survival were assessed retrospectively. RESULTS: The mean PCNA index for the whole series was 54%. There was no correlation between PCNA index and any of the clinicopathological characteristics. However, patients having tumors with a high (> 54%) PCNA index demonstrated significantly lower survival rates than tumors with a low (< 54%) PCNA index (P = 0.01). Moreover, there were significantly more patients with relapse or progressive disease in the high PCNA index group (P = 0.005). CONCLUSION: The PCNA labeling index can be a useful prognostic factor and a good indicator of recurrence and/or survival in patients with rhabdomyosarcoma.

Adolescent↗
PubMedDate not supplied

Embryonal rhabdomyosarcoma of the tongue.

We report a 10-month-old girl who, at 4 months, developed a small, reddish, plaquelike lesion on her tongue. This lesion began to enlarge rapidly, resulting in difficulty in swallowing and breathing. On physical examination, there was a large, red, friable, hard in consistency, irregular tumor over the distal portion of her tongue. The histopathology and immunohistochemical findings were consistent with an embryonal rhabdomyosarcoma. The patient was assigned clinical stage I, group III, and began treatment according to the Intergroup Rhabdomyosarcoma Study guidelines, on triple-agent chemotherapy consisting of vincristine, actinomycin D, and cyclophosphamide. She only received four cycles because her parents then refused this treatment. After the tumor size was reduced by chemotherapy, a partial anterior glossectomy was performed. After 30 months of follow-up she has had no recurrences.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

Carcinosarcoma of the parotid gland: an unusual case with large-cell neuroendocrine carcinoma and rhabdomyosarcoma.

We report a case of carcinosarcoma of the parotid gland in a 72-year-old Japanese man. The patient noticed a rapidly enlarging hard mass in the right parotid gland. He underwent radical parotidectomy with cervical lymph node dissection. The resected tumor measured 3.5 x 4.5 cm and histopathologically showed carcinomatous and sarcomatous components. The carcinomatous component consisted of large-cell neuroendocrine carcinoma (LCNEC), squamous cell carcinoma and adenocarcinoma not otherwise specified, while the sarcomatous component included spindle cell sarcoma not otherwise specified, so-called myxosarcoma and rhabdomyosarcoma. The LCNEC component was predominant within the whole tumor. The diagnoses of LCNEC and rhabdomyosarcoma were also confirmed immunohistochemically. With regard to histopathogenesis, based on the lack of histopathological evidence and antecedent history of pleomorphic adenoma, we considered the present case to be de novo, not expleomorphic adenoma.

Aged↗