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At least 865 records · Page 48Linked to original sources
PubMed1984

Ultrastructural distinctions between adult pleomorphic rhabdomyosarcomas, pleomorphic liposarcomas, and pleomorphic malignant fibrous histiocytomas.

The ultrastructural features of five pleomorphic rhabdomyosarcomas, five high-grade malignant fibrous histiocytomas, and five pleomorphic liposarcomas were studied. Electron microscopy was found to be consistently useful in distinguishing between these tumors. The rhabdomyosarcomas showed thick and thin filaments in complexes and consistently contained glycogen. The malignant fibrous histiocytomas had numerous lysosomes, often in cells with ruffled borders, and contained cells showing "myofibroblastic" differentiation. The liposarcomas showed abundant and coalescing lipid droplets, sparse stroma with condensation of amorphous granular materials surrounding plasma membranes, and prominent vascularity. Fourteen of the 15 tumors could be identified on the basis of ultrastructure; thus, electron microscopic examination is an important diagnostic tool for pleomorphic tumors.

Cytoskeleton↗
PubMed1985

Mesenchymal and muscle-specific intermediate filaments (vimentin and desmin) in relation to differentiation in childhood rhabdomyosarcomas.

Twenty-one childhood rhabdomyosarcomas were divided into three groups on the basis of cytologic composition. The tumors in group P consisted entirely of primitive mesenchymal cells, whereas those in groups M and W were characterized by the additional presence of numerous round rhabdomyoblasts and strap cells, respectively. The tumors were studied for the universal mesenchymal intermediate filament vimentin, and for the muscle-specific intermediate filament desmin. Vimentin positivity, which tended to be more prominent in primitive tumor cells, was found in all tumors, whereas desmin was found especially in round rhabdomyoblasts and strap cells. Desmin-positive primitive cells were found only in groups M and W, not in group P. It was concluded that the differentiation from primitive mesenchymal cells to morphologically recognizable myogenic tumor cells is accompanied by an increase in desmin positivity and, presumably, a decrease in vimentin positivity. Moreover, the observations suggest the existence of a group of "committed" cells that are morphologically primitive, but desmin-positive. These cells might play an important role in the observed further differentiation of rhabdomyosarcomas under chemotherapy.

Adolescent↗
PubMed2003

Alveolar rhabdomyosarcoma of the cervix.

BACKGROUND: Alveolar rhabdomyosarcoma (RMS) has a high risk for local and distal failure. Multimodal management of a patient with alveolar RMS of the cervix uteri is outlined. CASE: A 39-year-old woman suffered from alveolar RMS of the cervix without involvement of uterus and parametrium. She was treated with total hysterectomy and left salpingo-oophorectomy. Systemic chemotherapy and pelvic irradiation were also offered. She remains in clinical remission 3 years after presentation. CONCLUSION: Postoperative chemotherapy and irradiation could be effective treatments for alveolar rhabdomyosarcoma of the cervix in adult patients.

Adult↗
PubMed2000

Reconstructive surgery for children with pelvic rhabdomyosarcoma.

Rhabdomyosarcoma is the most common soft-tissue sarcoma found in children and can arise almost anywhere skeletal muscle is found. It represents 4% to 8% of malignant solid tumors in children, ranking behind central nervous system tumors, lymphoma, neuroblastoma, and Wilms' tumor. Reconstructive surgery has become an integral part of the total plan in patients undergoing radical surgery for rhabdomyosarcoma. Advances in surgical techniques can often provide a reasonable lifestyle for patients.

Child↗
PubMed1979

The treatment of orbital rhabdomyosarcoma with irradiation and chemotherapy.

Fifty-eight patients with orbital rhabdomyosarcoma were treated with irradiation alone (25) or irradiation and chemotherapy (33) with follow-ups of 6 months to 14 years (mean 5.2 yr). At present, 74% are alive and 26% have died. Local control of the tumor was accomplished in 91% of cases. When local sinuses were invaded the survival rate was 55%. Chemotherapy appears to be of greatest value when disease is limited to the orbit. Irradiation or irradiation and chemotherapy should now be the treatment of choice for orbital rhabdomyosarcoma.

Adolescent↗
PubMed1990

Rhabdomyosarcoma of the ciliary body.

Rhabdomyosarcoma is the most common malignant orbital tumor of childhood. It has twice been reported to arise within the globe from the iris. In addition, teratoid medulloepithelioma, a tumor arising from the ciliary epithelium, can contain a rhabdomyoblastic component, often in combination with other heteroplastic elements. The authors report what may be the first recorded case of an embryonal rhabdomyosarcoma of the ciliary body, possibly representing a one-sided differentiation of a malignant teratoid medulloepithelioma.

Actins↗
PubMed2000

Concomitant amplification and expression of PAX7-FKHR and MYCN in a human rhabdomyosarcoma cell line carrying a cryptic t(1;13)(p36;q14).

Alveolar rhabdomyosarcoma (ARMS) is associated with the specific chromosomal translocation (2;13)(q35;q14) or its rarer variant t(1;13)(p36;q14), which produces the fusion gene PAX7-FKHR. Here we describe the human cell line RC2, derived from an ARMS, which harbors a cryptic t(1;13)(p36;q14) and concomitantly shows amplification of the PAX7-FKHR fusion gene and of the MYCN oncogene. The t(1;13) and MYCN oncogene were studied by standard cytogenetic analysis and molecular techniques. The reverse transcriptase polymerase chain reaction demonstrated the expression of PAX7-FKHR mRNA in RC2 cells, although karyotype analysis failed to demonstrate a t(1;13)(p36;q14) chromosomal translocation or a derivative 13 chromosome. Double minute chromosomes were detected in all the metaphases studied. Fluorescence in situ hybridization analysis revealed multiple copies of the PAX7-FKHR fusion gene localized exclusively on a subset of double minutes, whereas multiple copies of MYCN were identified on other double minute chromosomes. Southern-blot analysis demonstrated that RC2 cells contain approximately 20 copies of the MYCN oncogene. So far no continuous RMS cell line carrying the t(1;13)(p36;q14) has been described, and PAX7-FKHR and MYCN amplifications have always been reported to occur separately in rhabdomyosarcoma (RMS). The availability of an ARMS cell line that harbors the t(1;13)(p36;q14) constitutes a useful tool for further understanding the role of the PAX7-FKHR fusion gene in RMS oncogenesis and may improve knowledge of the possible relation between PAX7-FKHR and MYCN amplification.

Chromosomes, Human, Pair 1↗
PubMed2001

Cytogenetic-clinicopathologic correlations in rhabdomyosarcoma: a report of five cases.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children younger than the age of 15 years. Histologically, RMS can be subdivided into two major subtypes; embryonal (E-RMS) and alveolar (A-RMS) rhabdomyosarcoma, with E-RMS being the more common. Although cytogenetic and molecular genetic findings have been reported extensively for RMS, clinicopathologic-genetic correlations among these tumors have not been reported in detail. In this report, we correlate the cytogenetic findings, including fluorescence in situ hybridization and spectral karyotyping, with pathologic findings and outcome for five RMS, including two A-RMS, one E-RMS, one botryoid RMS, and one anaplastic nonclassified RMS (N-RMS). The findings in A-RMS and E-RMS generally were consistent with previous reports; however, gain of chromosome 7 in A-RMS and gain of chromosome 9 segments in E-RMS observed here have seldom been reported in the literature. Importantly, the botryoid RMS had a cytogenetic profile similar to other types of E-RMS. An add(11)(q21) observed in this tumor, together with a t(8;11)(q12 approximately 13;q21) reported previously, indicates that 11q21 rearrangements may be nonrandomly related to botryoid RMS. In addition, the N-RMS expressed a cytogenetic pattern similar to that observed in E-RMS, thus providing genetic evidence that anaplastic N-RMS is a variant of E-RMS. Finally, these cases provide cogent evidence for the diagnostic and prognostic significance of the pathologic-genetic classification of RMS.

Adolescent↗
PubMed1998

Constitutional balanced translocations in alveolar rhabdomyosarcoma.

Chromosomal analysis of tumor tissue from two children with alveolar rhabdomyosarcoma revealed t(1;5)(q32;q31) and t(1;22)(q21;q11.2) in all metaphases examined, respectively. Peripheral blood lymphocytes carried the same cytogenetic abnormality as that of the tumor cells in both patients. Parental lymphocytes were karyotypically normal in the patient with t(1;22), indicating a de novo constitutional translocation, but t(1;5) was paternally inherited in the other patient. The presence of constitutional translocations in these two children might have contributed to the development of alveolar rhabdomyosarcoma.

Child↗
PubMed2000

Orbital rhabdomyosarcoma presenting as an apparent orbital subperiosteal abscess.

We present a patient with acute sinusitis whose CT scan showed a ring-enhancing lesion within the orbit typical of an orbital subperiosteal abscess. On exploration of the orbit, there was no pus present but a tumour was found, which on histological examination was found to be a rhabdomyosarcoma. The clinical signs and CT characteristics of orbital subperiosteal abscesses and rhabdomyosarcomas are discussed. A ring-enhancing lesion within the orbit on CT scan should not be regarded as being pathognomonic for a subperiosteal abscess and the possibility of other diagnoses should be considered.

Abscess↗
PubMed1983

Rhabdomyosarcoma of the head and neck.

A retrospective review of all cases of rhabdomyosarcoma seen at UCLA was done. Survival prior to the advent of cyclic multidrug chemotherapy was compared to survival with this therapy. In the latter group, unlike other published series, survival was 150% better with gross total removal of disease as compared to biopsy only. All received at least 5000 rads. Fifteen percent presented with regional nodal metastases. However 75% of these also had intracranial extension and all died. An additional 19% developed late regional nodal metastases. Eighty percent of these also developed systemic metastases and all died. Survival in the absence of nodal disease was 41%. This is the first study to address the influence of regional nodal metastases, early and late on the outcome of treatment. In rhabdomyosarcoma of the head and neck, regional nodal metastases at any time implies a grave prognosis and more seriously adversely affects the progress than it does in squamous cell carcinoma in adults.

Adolescent↗
PubMed1998

Facial paralysis: a presenting feature of rhabdomyosarcoma.

The purpose of this paper is to present a child with embryonal rhabdomyosarcoma involving the left middle ear, who initially presented with unilateral facial paralysis. A 5-year-old boy presented with a 4-week history of left-sided facial weakness, associated with persistent otitis media on that side. Examination revealed complete left lower motor neuron facial weakness and hearing loss. A myringotomy revealed a soft tissue mass behind the tympanic membrane. Biopsy and oncologic assessment confirmed a stage II, group III left middle ear embryonal rhabdomyosarcoma. Despite debulking surgery, local irradiation and multiple chemotherapeutic courses the child deteriorated quickly. He developed carcinomatous meningitis and died 9 months after his initial presentation. In conclusion, middle ear tumors should be considered in the differential diagnosis of unresolving otitis media, particularly when associated with persistent ipsilateral facial paralysis. An ear mass, discharge, facial swelling, or systemic symptoms may be initially absent despite the presence of this aggressive malignancy. Careful examination of the middle ear is recommended in children with facial weakness. A myringotomy incision may be necessary including a complete assessment of the middle ear cavity, particularly when there is no fluid return.

Child, Preschool↗
PubMed1997

Impact of the interfraction interval on clonogenic cell survival in split-dose irradiation of R1H rhabdomyosarcoma of the rat in vitro.

PURPOSE: In a previous study, the response of the R1H rhabdomyosarcoma of the rat to conventional irradiation (1.83-2.75 Gy fractions once-daily) and hyperfractionated radiotherapy (0.92-1.38 Gy fractions with different time intervals between the two daily fractions) was investigated [Kleineidam, M., Pieconka, A., Beck-Bornholdt, H.-P. Radiotherapy of the rhabdomyosarcoma R1H of the rat: influence of the time interval between two daily fractions during hyperfractionated radiotherapy. Radiother. Oncol. 30: 128-132, 1994]. Compared to once-daily irradiation, interfraction intervals of 2 h led to reduced tumour response, due to recovery from sublethal damage, and intervals of 5-6 h resulted in increased tumour response, possibly due to cell cycle effects. The purpose of the present study was to complement these tumour data by measuring clonogenic cell survival of R1H cells in vitro after split-dose irradiation. METHODS AND MATERIALS: Experiments were performed with 2 x 2.5 Gy and 2 x 3.5 Gy either at 21 degrees C (preventing cell cycle progression) or at 37 degrees C (allowing for cell cycle effects). RESULTS: For 3.5 Gy fractions, a cell survival curve equivalent to the in vivo results was obtained with the lowest surviving fraction observed at time intervals of 6-7 h, but only when cells were incubated at 37 degrees C during the interval. This phenomenon was absent in the 21 degrees C experiments. CONCLUSIONS: Our data provide further evidence to support the hypothesis that cell cycle effects are responsible for such observations. We conclude that the length of the interfraction interval has a considerable potential effect on tumour response to altered fractionation regimens.

Animals↗
PubMed1980

Combination therapy for laryngeal rhabdomyosarcoma.

A case of childhood laryngeal rhabdomyosarcoma is described with a 42 month follow-up and with no evidence of recurrent disease. Treatment included partial excision, postoperative radiotherapy, and chemotherapy with vincristine, actinomycin D, and Cytoxan. Normal laryngeal function has been maintained. Earlier modes of therapy would probably have included total laryngectomy. Recent advances in the combined therapy of childhood rhabdomyosarcoma are reviewed. Resulting cure rates approach 100 per cent in favorable cases. The concept of limited surgical therapy is discussed and is applied to this case.

Child↗
PubMed2001

[Embryonal rhabdomyosarcoma of the prostate. Report of a case and review of the literature].

The rhabdomyosarcoma (RMS) is a tumour derived from the mesenchymal cells that give origin to striate muscle. It appears most frequently at the pediatric age, although some appear in adolescence and in young adulthood. It is a high grade malignancy that metastatisis frequently (most importantly to the lung). There are two principal types: alveolar RMS and embrionary RMS. It can appear in any localization, being the most frequent the periorbitary area and the paranasal sinuses, and the second in frequency the genitourinary system (bladder, prostate and vagina). The therapeutic management is multidisciplinary and local therapies must always be accompanied with polychemotherapy. With combined treatments a curation rate of almost 90% can be reached in good prognosis cases, although it drops to 20% in those with disfavourable prognostic factors. The protocols with higher chemotherapy doses and subsequent rescue with blood-borne progenitor cells have not yet proved their efficiency. We present the case of a fifteen-year old patient with an embrionary rhabdomyosarcoma of the prostate, we analyze the clinical evolution and we revise the present therapeutic management of these neoplasies.

Adolescent↗
PubMed2004

[Paratesticular rhabdomyosarcoma].

Contribution of one case of paratesticular rhabdomyosarcoma in a 10-years old male patient. Following radical orchiectomy it was classified as Group Ia (Intergroup Rhabdomyosarcoma Study). Treatment was completed with 9 polychemotherapy courses of Ifosfamide, Vincristine and Actinomicine D. The patient was disease-free 6 months after the treatment.

Child↗
PubMed1989

Histological and immunohistochemical findings in experimental rhabdomyosarcomas. Comparisons between original tumors, tumor recurrences and allotransplants in nude mice.

Cellular heterogeneity, a regular property of malignant neoplasms, can constitute the basis for phenotypic shifting in malignant tumors. A disturbance of the balanced interactions as happens as result of all therapy measurements between tumor cell subpopulations and between tumor and host offers the possibility to profound changes on the basis of cellular heterogeneity. As tumor model we used methylcholanthrene-induced murine rhabdomyosarcomas, which were allotransplanted into nude mice. The original as well as the allotransplanted sarcomas were submitted repeated operative tumor mass reductions, comparable to an insufficient tumor surgery in man. The histology and the cellular differentiation were evaluated by light microscopy and immunohistochemical marker expression (vimentin, desmin and myoglobin). Our findings document that primary tumors, their recurrences after incomplete tumor removal, and allotransplants inclusive of their surgically induced recurrences were histologically and immunohistochemically not identical in every case when compared with each other, but despite the experimental procedures they retained basic criteria necessary for the rhabdomyosarcoma diagnosis. Changes in sarcoma histology and cellular marker expression are not only reflected by dedifferentiation but can also comprise differentiation (maturation) processes. After artificial breakdown of intratumoral cellular interactions and tumor/host relations, unknown sarcoma inherent factors directed to retaining basic properties obviously preponderate, at least for the moment, over accidental new cell clones with differing phenotypic characteristics, which could be expected by cellular heterogeneity and would result in a markedly changed tumor.

Animals↗
PubMed2000

Fractionated, three-dimensional, planning-assisted proton-radiation therapy for orbital rhabdomyosarcoma: a novel technique.

PURPOSE: Most children with orbital rhabdomyosarcoma will survive their disease. However, conventional photon-radiation treatment, as part of multimodality therapy, results in varying degrees of long-term functional and cosmetic side effects. This report introduces external beam proton radiation therapy (PRT) as a conformal, three-dimensional planned radiation technique for this disease, analyzes normal tissue dosimetry, and describes the technique's application in the first 2 patients. MATERIAL AND METHODS: Between January 1995 and February 1996, 2 patients underwent PRT following biopsy and chemotherapy for orbital rhabdomyosarcoma. Fifty and 55 Cobalt Gray Equivalent (CGE) were delivered to the gross tumor volume and 40 CGE to clinical target volumes in both patients. A relative biologic effectiveness (RBE) of 1.1 was utilized to correlate proton dose calculations with CGE. To achieve dose conformity, a "patch technique" was utilized, where target regions were divided into segments, each treated by a separate proton field. Dose-volume histograms were obtained for target and nontarget regions, including lens, bony orbit, pituitary gland, optic chiasm, optic nerves, lacrimal gland, and ipsilateral frontal and temporal lobes. RESULTS: At 3.4 and 2.5 years after PRT, both patients are clinically and radiographically free of disease. Visual acuity remains excellent, without signs of cataract formation; pituitary function is normal; cosmetically, only mild enophthalmos is noticeable. Doses to 90%, 50%, and 5% of lens volume were kept at less than 1%, less than 2%, and less than 8%, respectively. Fifty percent of lacrimal gland volume received less than 36% of the prescribed dose and 50% of the volume of the optic chiasm, pituitary gland, and hypothalamus were restricted to less than 2%. Proton conformity to orbital contents resulted in between 9% and 36% of the prescribed dose reaching the ipsilateral temporal and frontal lobes immediately adjacent to bony orbit (5% volume). CONCLUSION: PRT can offer excellent sparing of lens and selected intraorbital and ocular normal structures, while maintaining conformal target-dose coverage. The steep dose gradient beyond the orbit minimizes irradiation of normal brain parenchyma, with almost complete sparing of the pituitary gland. Reduction of integral irradiation exposure of the periorbital region will, hopefully, reduce the risk of second malignancy later in life. Reduced radiation dose to specific organs in close proximity to, but not part of the target region promises improved functional outcome and better cosmesis for childhood cancer survivors.

Child↗