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[A case of primary cerebral rhabdomyosarcoma (author's transl)].

A primary cerebral rhabdomyosarcoma is reported in a 51-year-old female. She noticed progressive weakness of the left leg in September 1977 and underwent craniotomy for a brain tumor in Tokyo Metropolitan Okubo General Hospital. At the operation the tumor was located on the medial aspect of the right frontal lobe, being attached to the falx. The tumor was 6.0 x 5.0 x 4.0cm in size and subtotally removed. Shortly after operation, she developed a left-sided hemiplegia and was referred to our Kanagawa Rehabilitation Center. During rehabilitation she developed disturbance in recent memory, orientation and speaking. A second operation was done in June 1978, revealing obvious recurrence. A well demarcated extramedullary gray and soft tumor, 7.0 x 5.0 x 3.5cm in size, was found in the previously operated site, being attached to the falx. It was subtotally again. Following surgery her clinical condition was temporally improved. Four months following surgery, however, she developed signs of increased intracranial pressure and died in October 1978 in spite of having chemotherapy and irradiation. Autopsy was not permitted. The tumor was diagnosed as rhabdomyosarcoma with light and electron microscopies and several investigation revealed no primary lesion in elswhere except for the cranium. The histological documentation is also presented and discussed.

Brain Neoplasms↗

[Rhabdomyosarcoma of the bladder in a 2-year-old boy].

A case of rarely occurring rhabdomyosarcoma of the urinary bladder in a boy of 2 is presented. The period from the first clinical manifestations (pyuria, macrohematuria) to death was 4 months. Urological examinations were done at the period of manifest clinical signs of disuria. A tumour of the urinary bladder almost completely filling the lumen was found in the form of numerous papillary-polyplike formations, with uropassage disorders. On the materials of operation biopsy the histological conclusion of urinary bladder rhabdomyosarcoma was reached. The death occurred with urogenic infection and manifestations of drug agranulocytosis (cytostatic therapy). The tumour outgrew into the posterior wall, no metastases were found. Histologically the tumour was characterized by random arrangement of mono- and multinuclear polymorphic cells with oxiphilic cytoplasm. The granularity and fibrous pattern of the cytoplasm were discernible. Typically, an argirophilic framework was observed. By its histological structure the tumour belongs to the embryonal type.

Biopsy↗

Rhabdomyosarcoma of the trachea: first reported case.

This report describes the first case of rhabdomyosarcoma of the trachea in a 65 year old man with a five week history of cough, wheezing, and hemoptysis. The tumor, presumably originating in the submucosal tissue, was characterized as an intratracheal polypoid mass showing no local invasion or distant metastasis. Rhabdomyosarcoma of the lower respiratory tract and its histogenesis are briefly reviewed and discussed.

Humans↗

Tc 99m bone scanning in early detection of rhabdomyosarcoma metastases. A case report.

Employing the Siemens Scintimat II, a skeletal scintigraphy was successfully appled in the early detection, localisation and delineation of bone metastases to the knee from rhabdomyosarcoma of the right pectoral region. However repeated radiographs of the knee continued to be negative. As all other treatment approach of the painful knee: physiotherapy (short wave diathermy) had failed to produce relief, radiotherapy was applied as a last resort; the swelling regressed and the pain disappeared. This was indicative of the positive detection of early rhabdomyosarcoma metastases to be bones as a positive scan is seen in all areas of bone accretion whether malignancy is present or not.

Bone Neoplasms↗

[Studies on the cell models of differentiation reversal phenotypes of human rhabdomyosarcoma].

We have observed the features of malignant phenotypes in human rhabdomyosarcoma RD cells. These cells exhibited high growth rate, aberrant relation between cell proliferation and myogenic terminal differentiation, blockage of myofibrillogenesis, inhibition of gap junctional intercellular communication (GJIC) and inhibition of transcriptional expression of gap junction gene, connexin 43 (Cx43). By utilization of single cell subclone separation and cDNA cell transfection techniques, we obtained several RD subclones, among them 3 were representative of differences in phenotypes and were selected for further study. They were single-cell subclone RDL6, RDL3, and a Cx43 transfectant clone of RDL6, the RDL6/C-4. Through determination of cell growth doubling time, co-stain of 3H-TdR autoradiography with myosin heavy chain (MHC) immunofluorescence, immunofluorescent cytochemical examination and slot-blot analysis, it was demonstrated that significant differences existed between each of the 3 subclones. They were differnet in cell growth rate, cell proliferation and terminal differentiation coupling relation, progress of myogenesis, functional expression of Cx43 gene, and expression of proto-oncogenes, c-myc, Ha-ras and c-met products, therefore they could be used as cell models in the study of reverse transformation of rhabdomyosarcomas. The reversal differentiation markers, their examination approaches and practical value were discussed.

Cell Transformation, Neoplastic↗

[Cutaneous rhabdomyosarcoma].

OBJECTIVE: The occurrence of a rare alveolar rhabdomyosarcoma in a 16-year old girl seems worth of a case report. OBSERVATION: A large multinodular tumor developed on the buttocks and the external genitalia; systemic symptoms and metastases of lymph nodes and skin occurred very soon and the disease had a lethal outcome within 16 months. A temporary regression was observed after polychemotherapy with daunorubicine, vincritine and 5-fluoro-uracile. COMMENTS: In children the rhabdomyosarcoma is the most frequent sarcoma of soft tissues; the poor prognosis of the alveolar type is assessed by the case of this 16-year old girl; this tumor may progress as a systemic disease and present as a leukemia with rapid impairment of general health, enlargement of lymph nodes, cutaneous nodules and anemia.

Adolescent↗

[Two cases of rhabdomyosarcoma of the bladder in children].

A 1-year-old boy was admitted to our hospital with chief complaint of urinary retention and a lower abdominal large mass. The mass was shown in the bladder by computerized tomography (CT) associated with paraaortic lymph node swelling. Tumor biopsy revealed rhabdomyosarcoma, embryonal type. Complete remission (CR) was obtained by chemotherapy based on STS' 88. He has been healthy 40 months without recurrence. A similar case of rhabdomyosarcoma, embryonal type, in the bladder in a 3-year-old girl with a chief complaint of macrohematuria was verified by CT and tumor biopsy. The same chemotherapy was performed, resulting in partial remission (PR). A complete resection of the tumor was achieved by partial cystectomy. She is alive without recurrence 18 months after the cystectomy.

Antineoplastic Combined Chemotherapy Protocols↗

[Establishment and characterization of a childhood embryonal rhabdomyosarcoma cell line with nonrandom chromosome alterations].

A childhood embryonal rhabdomyosarcoma cell line, designated as TS-RM-1, was established from transplanted tumor in nude mouse. TS-RM-1 cells were small, spindle to polygonal shaped and cytoplasm was rich in glycogen. Estimated population doubling time was 31 hours and the distribution of chromosome number was in the range of 88 to 98. The karyotype of TS-RM-1 cells revealed nonrandom structural chromosome alterations, including der(3)t(1;3)(q12;p12-14),16q-,17q+ and 21q+. In immuno-cytohistochemical study, both TS-RM-1 cells and the primary tumor were positive for desmin and vimentin. TS-RM-1 cell line may be useful for studying the association between embryonal rhabdomyosarcoma and a specific alteration in chromosome 3.

Animals↗

[Rare tumors of the cervix: three case reports: rhabdomyosarcoma, granulocytic sarcoma and lymphoma].

Three rare cases of tumours of the cervix are reported: embryonal rhabdomyosarcoma, granulocyte sarcoma and lymphoma. There were particular problems with diagnosis in each case requiring histochemistry, immunohistochemistry and ultrastructure examinations. Embryonal rhabdomyosarcoma is a uncommon tumour in the adult. Clinically, there is a vegetative formation but no botryoid aspect. Striated muscle cell proliferation, suggested by the morphology of the malignant cells was confirmed by immunohistochemistry which demonstrated desmine and vimentine and by the ultrastructural study which demonstrated Z lines in the cytoplasm of the malignant cells. Granulocyte sarcoma is characterized by a non-tumoural non-destructive infiltration of inflammatory-like cells. Their granulocyte nature is confirmed by histochemistry, the Leder's reaction on frozen samples, immunohistochemistry (expression of NP57). Electron microscopy can also be used. The diagnosis of lymphoma is difficult due to the non-tumoural nature of the lymphomatous infiltration which can simulate inflammatory reaction. In our case, the diagnosis was made on the tumoural aspect of the biopsy made in the deep infiltration zone of an adjacent organ (bladder). Immunohistochemistry demonstrated the lymphoid nature of the tumour and identified a B phenotype. The ultrastructural study gave little information.

Adolescent↗

Differentiation induction in the human rhabdomyosarcoma cell line TE-671. A morphological, biochemical and molecular analysis.

In the clonal human rhabdomyosarcoma cell line TE-671-1A, the expression of genes implicated in myogenic differentiation was determined before and after exposure to the differentiation inducers retinoic acid (RA), sodium butyrate (NaBut) and N-monomethylformamide (NMF). Exposure to NaBut or RA resulted in a significant (NaBut: p < 0.0001; RA: p < 0.05) increase in biochemical differentiation paralleled by a significant (NaBut: p < 0.0001; RA: p < 0.0002) inhibition of proliferation. An increase in the relative number of myotube-like giant cells was observed after exposure to NaBut. Exposure to NMF proved to be least effective and produced a significant (p < 0.0001) inhibition of proliferation without increase in differentiation. On the molecular level, exposure to RA resulted in a moderate increase in RAR a mRNA expression, whereas CRABP mRNA remained constant. RAR beta and RAR gamma mRNA were not expressed. mRNA expression of c-raf, c-myc and c-Ki-ras remained constant before and after exposure to all inducers of differentiation. C-fos mRNA was not expressed. In summary, differentiation can successfully be induced in the human rhabdomyosarcoma cell line TE-671-1A by various inducers of differentiation. In contrast to other myogenic cell lines, however, the proto-oncogenes myc, fos and raf are not involved in the transmission of myogenic differentiation signals in TE-671-1A cells.

Antineoplastic Agents↗

[Urogenital rhabdomyosarcoma in children: analysis of 39 cases].

From 1967 to 1992, 39 patients with urogenital rhabdomyosarcoma were admitted to our hospital. Of them 30 were male and 8 female and their age ranged from 4 months to 14 years (32 were less than 5 years). Primary tumor was located in bladder or/and prostate in 26, paratesticule 9, vagina 3, and penis 1. Among the 31 patients receiving treatment, 23 were followed up. There were 15 (65%) survivors, including 13 who were free from tumor for over 4 years. The eldest two patients were 20 and 21 years of age. Two patients with vaginal tumor and 4 with bladder tumor preserved their organs with normal function. Biopsy were done only in 8 patients. In recent years, conservative surgery combined with chemotherapy for urogenital rhabdomyosarcoma has been justified.

Adolescent↗

Omental rhabdomyosarcoma presenting with pyrexia.

A 45-year-old man was admitted with pyrexia and intermittently palpable lump in the left lumbar region. Laparotomy revealed a primary omental tumor which on histological examination showed alveolar rhabdomyosarcoma of the omentum. Following surgery the fever subsided. Presentation of omental rhabdomyosarcoma with fever has not been reported earlier.

Fever↗

[Rhabdomyosarcoma of the prostate and bladder in a child: radical or conservative surgery?].

Rhabdomyosarcoma is the most common soft-tissue tumor in childhood. In infants it is frequently localized in the pelvis and in the prostata or bladder region. The aim of treatment should be to eradicate the tumor and yet maintain as normal function as possible. Effective preoperative chemotherapy has enabled one to frequently achieve this aim. We report on two patients both with a rhabdomyosarcoma of the bladder-prostata. One of them in 1983 was operated and treated with chemotherapy and cysto-prostatectomy and the other in 1991 with chemotherapy and eradication of the tumor without cystectomy. Both children are now tumor-free after 10 years/2 years resp. Both approaches are discussed.

Antineoplastic Combined Chemotherapy Protocols↗

Cytologic diagnosis of rhabdomyosarcoma in a patient with germ cell tumor. A case report.

The development of sarcomas in patients with germ cell tumors is rare but has been reported previously. Theories about its pathogenesis include derivation of the tumor cells from pluripotential germ cells and malignant transformation from teratomatous elements. This report describes the occurrence of rhabdomyosarcoma in a patient with a history of mixed germ cell tumor of the testis who developed a malignant pleural effusion. Cytologic evaluation of the fluid revealed single malignant cells with high nuclear/cytoplasmic ratios and prominent nucleoli. While these features initially suggested a diagnosis of metastatic seminoma, careful cytologic and immunohistochemical examination revealed features consistent with metastatic rhabdomyosarcoma. The exfoliative cytologic findings of other germ cell tumors are also discussed.

Adult↗

Multiplex RT-PCR assay for the differential diagnosis of alveolar rhabdomyosarcoma and Ewing's sarcoma.

Cytogenetic analysis has defined specific translocations associated with two of the most common small round cell tumors of childhood, t(11;22) in Ewing's sarcoma and t(2;13) in alveolar rhabdomyosarcoma. We and others have previously demonstrated the diagnostic utility of a reverse transcriptase polymerase chain reaction (RT-PCR) assay for the detection of the t(11;22) encoded EWS/FLI-1 chimeric message in Ewing's sarcoma. More recently, we have cloned the t(2;13)(q35;q14) translocation and have shown that it results in the fusion of the PAX3 gene on chromosome 2 to FKHR, a novel member of the fork-head family of transcription factors on chromosome 13. To define the morphological spectrum of childhood sarcomas that express the t(2;13) encoded PAX3/FKHR chimeric message, we have performed RT-PCR analysis on samples from 44 primary pediatric sarcomas and 8 sarcoma cell lines. PAX3/FKHR chimeric messages were detected in 24 of 27 alveolar, 2 of 12 embryonal, and 0 of 1 pleomorphic rhabdomyosarcoma and in 1 of 2 ectomesenchymomas. In contrast, none of 8 Ewing's sarcomas or 2 undifferentiated sarcomas expressed this message. Chimeric transcripts were detected in all cases with cytogenetic evidence of the (2;13) translocation, and in each case the chimeric PAX3/FKHR message had the identical junction sequence, suggesting that genomic chromosome breaks were clustered in a single intron in both genes. By combining the PAX3/FKHR RT-PCR assay with primers for detection of the Ewing's sarcoma t(11;22) encoded EWS/FLI-1 chimeric transcript, we have developed a multiplex RT-PCR reaction that allows the rapid and accurate identification of either translocation in a biopsy sample.

Base Sequence↗

Actinomycin D causes multidrug resistance and differentiation in a human rhabdomyosarcoma cell line.

The emergence of drug-resistant tumor cells remains a major problem in cancer chemotherapy. Resistance to multiple unrelated antineoplastic drugs may be related, in part, to expression of the P-glycoprotein. The cell line RD, derived from an embryonic rhabdomyosarcoma tumor, was used as an in vitro model to examine the development of drug resistance. A cell line resistant to actinomycin D (RD-DAC) was developed by growing RD in increasing concentrations of the drug. The ID50 (concentration of drug needed to induce a 50% reduction in cell growth) of the resultant line to actinomycin D was more than 15 times that of the parental line. The resistant line was cross-resistant to vincristine and doxorubicin. Resistance to actinomycin D resulted in increased P-glycoprotein expression, which was associated with a change in desmin and vimentin expression. These results suggest that exposure to chemotherapeutic drugs can induce not only classical multidrug resistance, but also a process of cellular differentiation in rhabdomyosarcoma cells.

ATP Binding Cassette Transporter, Subfamily B, Mem↗

[Rhabdomyosarcoma of the thoracic wall in an adult. Apropos of a case].

Rhabdomyosarcoma is a malignant tumour generally observed in children or adolescents; thoracic localization is rare. The authors report a case of embryonary rhabdomyosarcoma of the thoracic wall in a 36-year-old subject. Pathology examination of surgical specimens confirmed the diagnosis. Treatment included surgical exeresis together with pre- and post-operative chemotherapy combining doxorubicine (40 mg/m2/cycle), ifosfamide (6 g/m2/cycle) and dacarbazine (900 mg/m2/cycle). Local recurrence, requiring radiotherapy, was observed a few months later and led to the patient's death after a 9-month clinical course.

Adult↗