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[Clinicopathological and immunohistochemical study of 118 cases of rhabdomyosarcoma].

118 cases of rhabdomyosarcoma are reported. Specimens from 30 of these cases were stained with Masson Trichrome and phosphotunstic acid haemotoxylin. 36 cases were studied immunohistochemically by PAP, and ABC methods. Specific antibodies against myoglobin, desmin and vimentim were used. Positive immunostaining for myoglobin and desmin was found in 72.7% and 55.5% of the cases studied respectively. The positivity was dependent on the degree of cell differentiation. Results suggest that immunohistochemistry is a useful tool for the diagnosis of poorly differentiated rhabdomyosarcomas. Cross--striations were found in only 6 of the thirty cases (20%). It is now generally accepted that demonstration of cross--striations is not essential for the diagnosis; nevertheless, the characteristic features of fibrillary material arranged in whorls around the nucleus are of diagnostic significance. Histologically, it is also believed that searching for early differentiated rhabdomyoblasts combined with the histological pattern is of vital importance for an accurate diagnosis.

Adolescent↗

Synergistic interactions between cyclophosphamide or melphalan and VP-16 in a human rhabdomyosarcoma xenograft.

Based on previous work demonstrating the activity of cyclophosphamide and melphalan in a series of human medulloblastoma and rhabdomyosarcoma cell lines and transplantable xenografts, investigations were conducted to define the effects of combining cyclophosphamide or melphalan with VP-16. These studies demonstrated a synergistic interaction between cyclophosphamide and VP-16 and melphalan and VP-16 in the treatment of the human rhabdomyosarcoma cell line TE-671 growing in athymic mice. The combination of cyclophosphamide or melphalan with VP-16 may warrant consideration as a therapeutic strategy for solid tumors sensitive to bifunctional alkylating agents.

Alkylating Agents↗

Fine needle aspiration of rhabdomyosarcoma of the heart. Light and electron microscopic findings and histologic correlation.

Fine needle aspiration (FNA) biopsy was performed on a 12-year-old child with intractable cardiac arrhythmias and a mediastinal mass. The cytologic, immunocytochemical and electron microscopic findings on the aspirated material established an initial diagnosis of cardiac rhabdomyosarcoma. Subsequent histologic material confirmed the cytologic diagnosis. The findings are presented, and the use of FNA to definitively establish a preoperative diagnosis of cardiac rhabdomyosarcoma is discussed.

Antibodies, Monoclonal↗

[Ultrastructural characteristics of soft tissue rhabdomyosarcomas].

Seven rhabdomyosarcomas of human soft tissues were studied electron microscopically. Certain ultrastructural features characteristic of tumour rhabdomyoblasts were revealed: atypical fibrillar apparatus, spatially uneven process of myofibrillogenesis, disturbed cyto- and histotypical differentiation ratio and others. These features may be used for electron microscopic diagnosis of rhabdomyosarcomas among soft tissue tumours of low differentiation.

Adolescent↗

Fatal breast rhabdomyosarcoma in a 15 year old primigravida.

A fatal case of rhabdomyosarcoma, occurring in a 15 year old primigravida is described. Difficulties in early and correct diagnosis, the problems of management, and whether this cancer is due to the pregnant condition are discussed. The ethical dilemma is: should the pregnancy be allowed to continue or not, causing delay in the beginning of chemotherapy yet permitting the delivery of a healthy fetus--since the mother was certainly condemned by the widespread diffusion of the rhabdomyosarcoma.

Adolescent↗

Intracardiac rhabdomyosarcoma in a dog.

Intracardiac rhabdomyosarcoma was diagnosed in a 14-month-old Golden Retriever referred for evaluation of syncope. The dog was weak, with irregular heart rate, pulse deficit, heart murmur, and hepatomegaly. Thoracic radiography revealed generalized cardiomegaly, and electrocardiography revealed sinus rhythm with multiform ventricular premature complexes and intermittent ventricular tachycardia. Two-dimensional echocardiography revealed extensive soft-tissue density masses in both ventricles. An intracardiac neoplasm seen at postmortem examination was identified histologically as rhabdomyosarcoma, an uncommon intracardiac tumor in dogs.

Animals↗

[Rhabdomyosarcoma of the prostate in a child: report of a case].

A case of prostatic rhabdomyosarcoma in a 5-year-old boy is presented. He was referred to us because of complete urinary retention. Histologically, embryonal rhabdomyosarcoma of the prostate was found. Initially, he was treated with combination chemotherapy consisting of vincristine, actinomycin-D, adriamycin and radiation therapy. This therapy reduced the size of the tumor markedly. Therefore, we performed prostatectomy. No tumor cells were found on the surgical margin of the resected prostate. About 11 months after the operation, rapidly growing recurrent lesions were found near the bladder neck. Bilateral hydronephrosis and an intravesical massive space occupying lesion were found on the excretory pyelograms and cystogram. Then three drug combination chemotherapy consisting of vinblastine, cis-diamminedichloroplatinum and bleomycin (PVB therapy) was administered and the first course of the chemotherapy reduced the size of the recurrent tumor. Drainage of contrast medium from bilateral kidneys became smooth, and no gross hematuria or severe frequency was observed. Total cystectomy with ileal conduit urinary diversion was performed. Four months after this operation, metastatic lesions appeared at the pubic bone, right ischiadic bone and rectum, and they were resected operatively. However, multiple pulmonary metastases soon occurred, and he did on June 30, 1985, approximately 2 years after the first diagnosis. We have discussed the effectiveness of combination chemotherapy, especially PVB therapy for recurrent cases.

Antineoplastic Combined Chemotherapy Protocols↗

[New combination chemotherapy in refractory rhabdomyosarcoma of the prostate: a case report and review of the literature].

A rare case of prostatic rhabdomyosarcoma in a 3-year-old child is presented. He received a multimodal treatment of chemotherapy including vincristine, cyclophosphamide, actinomycin-D plus adriamycin, and linac irradiation followed by total prostatectomy with segmental resection of bladder. Despite no viable sarcoma cells in the surgical specimen, he showed relapse 4 months later. By the combination of cisplatin, vinblastine and peplomycin (PVP therapy), the bulky mass in the pelvis rapidly reduced by 95%. Furthermore, cisplatin, etoposide and peplomycin (PEP therapy), instead of PVP therapy, were administered to the refractory disease. Although the residual tumor was salvaged after 7 courses of PEP therapy, abdominal dissemination and liver metastasis occurred 2 months postoperatively. The combined modality of vincristine, peplomycin, ifosfamide, methotrexate, adriamycin, melphalan plus nimustine, and irradiation of microtron were effective for the abdominal dissemination, but he died of cerebral hemorrhage 31 months after the start of treatment. We emphasize that more aggressive chemotherapy including cisplatin, etoposide and so on is required to manage the advanced, relapsed, or resistant cases compared to the usual modality of rhabdomyosarcoma.

Antineoplastic Combined Chemotherapy Protocols↗

Immunohistochemical study of uterine stromal sarcoma and rhabdomyosarcoma.

The cytoplasmic filament composition of two pure high-grade endometrial stromal sarcomas and five pure endometrial rhabdomyosarcomas was studied using the immunoperoxidase avidin-biotin method. Ultrastructural correlates were performed on the same tissues. While four of the seven tumors presented as high-grade undifferentiated neoplasms, they were readily categorized on the basis of immunocytochemical findings in which the five rhabdomyosarcomas were positive for muscle-specific actin (HHF 35) and the two stromal sarcomas were positive for vimentin only. Ultrastructure on the HHF 35-positive cases showed the presence of thick filaments and Z-band material, whereas the other tumors showed no cytoplasmic differentiation. Muscle-type actin differs stromal cells, and is useful when used in conjunction with a panel of antibodies in the categorization of undifferentiated endometrial sarcomas.

Biomarkers, Tumor↗

[Genitourinary rhabdomyosarcoma in childhood].

We present the result obtained by GICOP in the treatment of 12 genitourinary rhabdomyosarcoma, located in vagina-uterus (5), bladder-prostate (4) and paratesticular (3). The age of the patients ranging from 1 to 15 years. Embryonal rhabdomyosarcoma was the most common histologic type (58 for 100) being the major number of cases in stadium II. The treatment was based in the combination of chemotherapy protocolised with vincristine, d-actinomycin, cyclophosphamide (VAC), vincristine, adriamycin (VAC-VAD) and/or iphosphamide, vincristine, d-actinomycin (IVA), use of radiotherapy, marrow auto-transplantation and radical surgery avoidance. Complete remission was achieved in 11 out of 12 cases (92 for 100), relapsing in 5 cases (38 for 100) of which 4 (80 for 100) achieved a second remission. An 82 for 100 disease free survival was achieved in a 5 year period.

Adolescent↗

Detection of point mutations in N-ras and K-ras genes of human embryonal rhabdomyosarcomas using oligonucleotide probes and the polymerase chain reaction.

Previous studies have demonstrated that genes of the ras family (H, K, and N) can be activated by point mutations at codons 12, 13, and 61. In the present study we have used oligonucleotide probes corresponding to these regions to assess the role of ras gene mutations in the genesis of human rhabdomyosarcoma. To increase the sensitivity of this method the appropriate regions of the three ras genes were first amplified using the polymerase chain reaction. The results show that 35% (5/14) embryonal rhabdomyosarcomas investigated contain mutations in the N-ras or K-ras genes. Thus ras gene mutation is implicated in the development of mesenchymal and embryonal tumors in addition to its previously documented role in epithelial and hematological neoplasia.

Adolescent↗

[Embryonary rhabdomyosarcoma of the middle ear. Apropos of a case].

The subject presented for its rarity is a case of rhabdomyosarcoma embryonal ear in girl patient 6 years old. Her clinic first gave a wrong diagnosis of chronic otitis of the middle ear with otorrhea and facial paralysis. A malignant tumour was suspected during the operation. A histopathology study confirmed the diagnosis of rhabdomyosarcoma. A review of the literature is made and details given of how often it occurs, and incidence, histopathology types, treatment and prognosis.

Child↗

[A case of primary rhabdomyosarcoma of the right ventricle].

A 32-year-old man was admitted to our hospital suffering from cough and bloody sputum. After the preoperative examination, the most suspected diagnosis was either a cardiac myxoma or a pulmonary embolism. At operation, the cavity of main pulmonary trunk was occupied with a tumor growing through the stalk from the outflow tract of right ventricle. The invasive tumor was not completely removed from the pulmonary tract. Rhabdomyosarcoma originating from the right ventricle was confirmed histologically. The patient died on the 199th postoperative day. A necropsy was not conducted. Diagnosis, operative indication and chemotherapy of cardiac rhabdomyosarcoma are discussed in this paper.

Adult↗

Human rhabdomyosarcoma cells in nude mice as a model for metastasis and differentiation.

We investigated the ability of alveolar (RMZ-RC2) and of embryonal (RD) human rhabdomyosarcoma cell lines to grow and metastasize in nude mice. Both cell lines produced local tumors, but failed to give rise to spontaneous metastases. When RD cells were injected intravenously into nude mice pretreated with cyclophosphamide (in order to depress primarily natural killer activity), several large lung colonies were obtained. Renal localization was also observed. In vitro cultures obtained from tumors or secondaries retained the ability to differentiate in the myogenic pathway shown by the parental cell lines. Intravenous injection into nude mice could therefore constitute an interesting experimental system to study metastatic ability and differentiation properties of human rhabdomyosarcoma cells.

Adrenal Gland Neoplasms↗

[Genital rhabdomyosarcoma. Diagnostic and therapeutic considerations. Apropos of a case of cavernous origin].

A case of perineal rhabdomyosarcoma possibly originating in the corpus cavernosum penis is reported. Treatment was by surgery first followed by chemotherapy. The poor outcome prompted us to review the literature on the diagnosis and treatment of this condition. We can conclude from data published elsewhere that rhabdomyosarcoma localized to the GU tract is a tumor that is amenable to treatment by chemotherapy combined with radiotherapy in most of the cases. When planning treatment, diagnosis must be well-established histologically and staging must be precise in order to avoid unwarranted radical surgery.

Adolescent↗

Expression of the myc protooncogene in canine rhabdomyosarcoma.

The expression of the protooncogene c-myc in a canine rhabdomyosarcoma was examined. It was found that this highly malignant tumour contained vast quantities of RNA that hybridized with a cDNA probe for c-myc. Restriction fragment length analysis after endonuclease digestion of tumour DNA did not reveal any rearrangements in this gene locus. The potential role of this oncogene in the development of canine rhabdomyosarcoma is discussed.

Animals↗

X-radiation-induced differentiation of xenotransplanted human undifferentiated rhabdomyosarcoma.

A serially xenotransplantable strain of undifferentiated embryonal rhabdomyosarcoma originating from the nasal cavity of a 42-year-old woman has been established in our laboratory. After radiotherapy for the tumor donor, distinct rhabdomyoblastic differentiation of the undifferentiated sarcoma cells appeared in the primary lesion, and it is a reasonable assumption that X-irradiation has a certain potentiality to induce morphologic differentiation of tumor cells. To study this possibility, tissue fragments of undifferentiated embryonal rhabdomyosarcoma that had grown to more than 10 mm after being transplanted to nude mice were selectively irradiated in situ. The degree of rhabdomyoblastic differentiation according to radiation dose was evaluated by light and electron microscopy and by immunostainability for myoglobin, creatine phosphokinase-MM, and desmin. Distinct morphologic differentiation of undifferentiated sarcoma cells could be induced by repeated X-irradiations at several-week intervals.

Adult↗