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At least 685 records · Page 38Linked to original sources
PubMed1996

Detection of the t(2;13) chromosomal translocation in alveolar rhabdomyosarcoma using the reverse transcriptase-polymerase chain reaction.

Rhabdomyosarcoma, a small-, round-cell tumor of skeletal muscle, is the most common soft tissue sarcoma found in children. A specific and unique chromosomal translocation, t(2;13)(q35;q14), has been described cytogenetically in a subset of these tumors and is most often associated with the alveolar histologic subtype. The cloning and sequencing of complementary DNA from fusion transcripts expressed by both cell lines and tumors have shown that this chromosomal translocation results in the fusion of the PAX3 gene on chromosome 2 with a member of the forkhead gene family, FKHR, on chromosome 13. To detect this genetic abnormality we have developed a sensitive method which relies on a reverse transcriptase-polymerase chain reaction with primers designed to be specific for the chromosome 2 and chromosome 13 sides of the translocation. The utility of this approach was tested by analyzing a series of rhabdomyosarcoma cell lines and tumor samples. The data demonstrate that the transcripts derived from the t(2;13) were restricted to tumors having features of the alveolar subtype and that they could be detected with greater ease and sensitivity than with cytogenetic analysis. This approach will facilitate a large-scale group effort to determine the frequency as well as the prognostic and diagnostic significance of this chromosomal rearrangement.

Cell Line↗
PubMed1996

Novel formation and amplification of the PAX7-FKHR fusion gene in a case of alveolar rhabdomyosarcoma.

Alveolar rhabdomyosarcomas frequently exhibit specific translocations, resulting in the fusion of the FKHR gene at 13q14 with either the PAX3 or PAX7 gene at 2q35 and 1p36, respectively. Comparative genomic hybridization revealed amplification at 13q14 and 1p36, suggesting amplification of the PAX7-FKHR fusion gene in two cases of alveolar rhabdomyosarcoma. A PAX7-FKHR fusion transcript was demonstrated in both cases by reverse transcription-polymerase chain reaction followed by sequence analysis. In one case, amplification of the PAX7 gene and 3'-and 5'-FKHR gene sequences was demonstrated by using interphase fluorescence in situ hybridization on tumor imprints. The colocalization, variable copy number, and distribution of signals from the three cosmids was consistent with amplification of these sequences on double minutes, which were present cytogenetically. Chromatin release studies suggested that the amplified sequences correlated with amplification of the PAX7-FKHR fusion gene which resulted from the insertion of PAX7 sequences into the first intron of FKHR gene, in keeping with the absence of cytogenetic evidence for derivative chromosomes.

Child↗
PubMed2000

Issues in the local control of rhabdomyosarcoma.

BACKGROUND: Rhabdomyosarcoma (RMS) is a heterogeneous disease consisting of several different histologies arising from a variety of anatomic sites. Approximately half of the children who die of this tumor have failure at the primary site of involvement, making local control an important component of therapy. PROCEDURE: Published literature and newly analyzed data from the Intergroup Rhabdomyosarcoma Study Group (IRSG) regarding local control of RMS were reviewed. Information regarding the role of various local control modalities for different primary disease sites is presented along with new directions for clinical research. RESULTS: Local control rates for RMS average 80% for group III tumors, with large variations seen for different anatomic sites. Important gains in functional outcome for certain sites such as gynecologic system and bladder/prostate have been achieved by optimizing the use of the various treatment modalities. Local control at other sites such as the chest and extremities remains a problem. CONCLUSIONS: Advances in surgical and radiotherapy techniques coupled with multiagent chemotherapy are providing improved local control with decreasing morbidity. Optimal outcome is dependent on close collaboration between surgical, radiotherapy, and pediatric oncology specialists.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1975

Primary rhabdomyosarcoma of the cerebrum.

A primary cerebral rhabdomyosarcoma is described in a 48-year-old Negro man. At autopsy two metastatic nodules were present in the liver. A review of the literature indicates that this is a rare and highly malignant tumor with no 5-year survival. The multipotentiality of the mesenchymal tissue in the leptomeninges is considered the histogenetic origin of intracranial rhabdomyosarcomas.

Autopsy↗
PubMed1975

Intensive chemotherapy as primary treatment for rhabdomyosarcoma of the pelvis.

Twenty-six cases of childhood rhabdomyosarcoma involving the pelvis were treated at Childrens Hospital of Los Angeles between 1950 and 1972. The median survival of 17 patients treated up to 1970 was 6 months. Of a group of 9 patients treated with intensive chemotherapy (ICG) prior to surgery and/or x-ray therapy, 5 are surviving from 12 to 60 months and have a relative survival rate of 4.5 times that of the mixed therapy group (MTG) (p = 0.01). Deaths have occurred only in patients with Stage IV disease. The use of preoperative chemotherapy with or without x-ray therapy followed by local surgical excision when necessary can be as effective in prolonging the survival of children with pelvic rhabdomyosarcoma as those obtained with more traditional approaches; this limitation in use of surgery is likely to preserve the quality of life, should the child live to adulthood.

Adolescent↗
PubMed1976

Nonepithelial tumors of the nasal cavity paranasal sinuses, and nasopharynx: a clinicopathologic study. V. Skeletal muscle tumors (rhabdomyoma and rhabdomyosarcoma).

In a study of nonepithelial tumors involving the nasal cavity, paranasal sinuses, and nasopharynx, 19 lesions (three rhabdomyomas, 16 rhabdomyosarcomas) showed skeletal muscle differentiation. The clinical findings associated with these neoplasms are reviewed, the histologic features are illustrated, results of treatment are presented, and clinicopathologic correlations are discussed. The rhabdomyomas grew as localized masses in the nasopharynx, were excised locally, and behaved clinically like benign tumors. The rhabdomyosarcomas were found predominantly in children and were associated with a poor prognosis; most of the patients died within 2 years following diagnosis.

Adolescent↗
PubMed1977

Lymphatic metastasis with childhood rhabdomyosarcoma.

Clinico-pathologic reviews of series of children with rhabdomyosarcoma have yielded conflicting information regarding frequency of lymphatic spread of this disease. The 264 eligible entries in the Intergroup Rhabdomyosarcoma Study (from November 1972 to September 1975) have been categorized by a prospective staging system and pre-treatment characteristics and pathologic findings reviewed. Data accumulated thus far have revealed a higher than expected incidence of lymphatic metastases from extremity (17%) and genito-urinary sites (19%) with a somewhat lower incidence from the orbit (0%), the head and neck region (3%), and trunk (10%). These differences in regard to lymphatic metastases were found to have no relationship to age, sex, tumor size, or histologic type when these factors were simultaneously examined.

Adolescent↗
PubMed1977

Combined treatment modalities of rhabdomyosarcoma in children.

Thirty-nine previously untreated children with rhabdomyosarcoma were managed by a coordinated program of surgery, radiation therapy, and chemotherapy during the years 1960 to 1973. The primary tumor was located in the head and neck (24), chest wall (1), abdomen (1), pelvis (10), and lower extremity (3). Radiation therapy consisted of tumor doses of 5000 to 6000 rads delivered in five to six weeks. Combination chemotherapy with actinomyctin-D, vincristine and cyclophosphamide was used after 1968. Seventeen of 25 cases (68%) treated after 1968 are alive two to five years following treatment. Only four of 14 cases (29%) who received less radical therapy before 1968 are alive. A relatively high incidence of local failure (23%) was noted in spite of adequate doses of radiotherapy. None of the four cases with metastatic disease at the time of diagnosis survived. Major complications were mainly noted in patients with orbital rhabdomyosarcoma. Correlation of absolute survival with dose of irradiation, primary site, extent of disease and histologic subtypes of the tumor are discussed.

Adolescent↗
PubMed1978

Ultrastructural observations on the histogenesis of alveolar rhabdomyosarcoma.

Ultrastructural examination of an alveolar rhabdomyosarcoma revealed that the bulk of the tumor was composed of small polygonal cells containing polyribosomes, short strands of rough endoplasmic reticulum, and variable amounts of glycogen; the cells were joined by small desmosome-like structures. Similar cells, as well as a variety of larger forms which appeared to be derived from the small cells, were observed along the septa; the larger forms developed a copious cytoplasm and occasionally produced myofilaments. Some of these cells were separated from the septa by basement membranes. Giant cells were also present in the alveolar spaces; these formed neither basement membranes nor myofilaments. The septa themselves were composed largely of collagen and fibroblasts. It is suggested that the commonly proposed correspondence of alveolar rhabdomyosarcoma to the myotube stage of muscle differentiation is incorrect; if anything, the overall pattern of the tumor is epithelial-like, and may correspond to the organization of mesenchymal cells at the stage of somite differentiation. The resemblance of the masses of small tumor cells to the cells of Ewing's sarcoma is discussed.

Basement Membrane↗
PubMed1978

Paratesticular rhabdomyosarcoma in childhood.

The paratesticular region was the primary site in 20 of 289 children (7%) entered on the Intergroup Rhabdomyosarcoma Study. The 20 patients were 1.7- to 19-years-old at diagnosis. Fifteen underwent retroperitoneal node dissection (12) or biopsy (3), and 6/15 (40%) had nodal involvement by tumor. Nineteen of the 20 patients had no gross local or metastatic disease after surgery. All 20 received chemotherapy, and 13 also received radiotherapy. Treatment was effective: 16 of 18 evaluable patients (89%) were free of disease at a median of 23 months from diagnosis (range, 8-43 months). Since the incidence of tumor-involved retroperitoneal nodes is high, a dissection should be performed. If the nodes are free of tumor, retroperitoneal radiotherapy may not be necessary. Reduction of morbidity in patients with paratesticular rhabdomyosarcoma is desirable, because the disease has a good prognosis.

Adolescent↗
PubMed1979

Rhabdomyosarcoma arising in a pineal teratoma.

Pineal teratomas are relatively uncommon intracranial neoplasms. A rhabdomyosarcoma developed in a pineal teratoma in a 14-year-old boy and was rapidly fatal despite radiation therapy. This is the second reported case of a pineal teratoma giving rise to a rhabdomyosarcoma.

Adolescent↗
PubMed1980

Retroperitoneal rhabdomyosarcoma in children. Results of multimodality therapy.

The clinical course of 18 consecutive children treated for primary retroperitoneal rhabdomyosarcoma was reviewed. At diagnosis, 8 patients had regional unresected tumor and 10 patients had disseminated tumor, including 3 patients with documented bone marrow infiltration by tumor. Following combined modality therapy, 14 of 18 patients achieved a greater than 50% tumor response (11 complete and 3 partial responses); 4 patients failed to respond and died of progressive disease within eight months of diagnosis. Among the 14 patients responding, 7 patients had subsequent reextension of active tumor three to 16 months (median, 9 months) following the onset of therapy. Three of the 7 remaining patients died of treatment complications, 2 of intestinal obstruction and 1 of disseminated histoplasmosis, within the first year of therapy and at post-mortem examination had no demonstrable tumor. Four patients are alive and free of active tumor for 10+, 10+, 32+ and 33+ months from diagnosis. Treatment complications have included hematopoietic depression, mucositis, enteritis, intestinal obstruction, excessive weight loss, malnutrition, and life-threatening infection. These results illustrate limitations in current combined modality therapy of retroperitoneal rhabdomyosarcoma and the necessity for future treatment modifications to both reduce morbidity and to improve survival.

Adolescent↗
PubMed1980

Polypoid sarcoma of the pulmonary trunk: analysis of the literature and report of a case with leptomeric organelles and ultrastructural features of rhabdomyosarcoma.

A case of polypoid sarcoma of the pulmonary trunk is described in an 80-year-old woman who had a 21-year history of episodic chest pain and hemoptysis. Ultrastructural examination revealed Z bands, characteristic of rhabdomyosarcoma, and leptomeric organelles. This is the 60th reported case of sarcoma of the pulmonary trunk. Symptoms in reported cases were variable, usually caused by tumor emboli to the lungs or by right ventricular outflow obstruction. Angiography was diagnostic. The tumor was characteristically polypoid, often multicentric, and by definition was fixed to the pulmonary trunk or valves. Non-differentiated sarcoma (often uniquely pleomorphic) was found in 37% of patients, leiomyosarcoma (once confirmed by electron microscopy) in 17%, myxosarcoma in 13%, elements of rhabdomyosarcoma in 8%, fibrosarcoma in 8%, elements of chondrosarcoma in 11%, and 5% were malignant mesenchymomas. The authors suggest that these tumors originate from the undifferentiated tissues of the bulbus cordis and propose the name myenchymoma for the tumor.

Adult↗
PubMed1980

Rhabdomyosarcoma in children: ultrastructural study of 31 cases.

Thirty-one cases of rhabdomyosarcoma in children were studied by electron microscopy. All of the tumors examined fit into a continuum of progressive cellular differentiation mimicking that of normal myogenesis, with increasingly differentiated examples being decreasingly common. We conclude that all childhood forms of rhabdomyosarcoma are essentially embryonal tumors, with many not being sufficiently differentiated at the cellular level to allow positive ultrastructural diagnosis.

Child↗
PubMed1981

Diffuse meningeal rhabdomyosarcoma.

Pure rhabdomyosarcomas are extremely rare as primary tumors in the cranial vault. In this instance, a 25-year-old man had symptoms and signs of chronically increased intracranial pressure. A biopsy of the meninges of his posterior fossa was performed revealing a spindle-cell sarcoma. Ultrastructural evidence of striated muscle differentiation and cross striations was present. The patient died of pulmonary emboli. Complete autopsy failed to disclose tumor anywhere except the leptomeninges. The presence of this tumor indicates that a rhabdomyosarcoma can arise from the meninges without a preexisting glioma or teratoma.

Adult↗
PubMed1982

Botryoid rhabdomyosarcoma of the gallbladder in a child.

The first case of botryoid rhabdomyosarcoma of the gallbladder in a child is described and compared with previously reported cases from the world literature. Despite its rarity, rhabdomyosarcoma of the liver and biliary system should be considered when an obstructive jaundice is seen in children. Radical surgery with additional chemotherapy and radiotherapy are necessary for long-term survival.

Adolescent↗
PubMed1985

Soft tissue sarcomas arising in the retroperitoneal space in children. A report from the Intergroup Rhabdomyosarcoma Study (IRS) Committee.

One hundred one eligible children with soft-tissue sarcomas arising within the retroperitoneal space have been registered on Intergroup Rhabdomyosarcoma Study Committee (IRS) studies I and II and followed for at least 2 years or until death. The most common presenting symptoms and signs were pain in the abdomen or lower extremities, and/or an abdominal mass, usually noted by a parent or a physician seeing the child for other complaints. The median age at diagnosis was 6.5 years and the sex ratio (M/F) 1.7:1. Histologic types were embryonal or botryoid rhabdomyosarcoma (RMS) in 58 patients, alveolar RMS in 8, pleomorphic RMS in 2, undifferentiated sarcoma in 20, extraosseous Ewing's sarcoma in 4 and unspecified sarcoma in 9. Median tumor size was 10 cm, significantly larger than the 7.5 cm noted in the IRS studies as a whole (P less than 0.05). One patient had complete tumor removal (Group I); 12 had grossly complete removal with microscopic residual tumor (Group II). Fifty-one patients had residual local tumor after biopsy or partial resection (Group III) and 37 patients had distant metastases at diagnosis (Group IV). Treatment included surgery, radiation therapy (RT) and combination chemotherapy with vincristine and actinomycin D with or without cyclophosphamide and Adriamycin (doxorubicin) according to protocol. Thirty-nine of 99 patients (39%) had major difficulties in the delivery of specified RT. Seventy patients received sufficient therapy to be evaluable for treatment response. Forty-one (58%) achieved a complete remission and 16 (23%) achieved partial remission. Twenty-four of 41 children (59%) achieving a CR have relapsed. The proportion of children who remained relapse-free at 2 and 3 years of follow-up was 44% or 42%, respectively. Overall, 40 children have developed recurrent sarcoma and the median disease-free interval and overall survival times were 54 and 88 weeks, respectively. Most children experienced severe myelosuppression; there were three early deaths from infection which occurred during granulocytopenic periods. One third of the patients experienced cystitis. Children with soft-tissue sarcomas arising in the retroperitoneal space have a poor prognosis associated with large tumors which, because of location and/or extent (Groups III or IV), are unresectable and difficult to treat.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1986

Infants younger than 1 year of age with rhabdomyosarcoma.

Of a total of 1561 patients registered in the Intergroup Rhabdomyosarcoma Study (IRS) as of May 1983, 78 patients (5%) were younger than 1 year of age. These infants did not differ from the older children (1-20 years of age) in male/female ratio, clinical grouping, or survival rates. Infants younger than 1 year of age had a significantly greater frequency of undifferentiated sarcoma, 18% versus 7% in older children (P less than 0.005). Infants also had a significantly greater proportion of cancers with botryoid pathology, 10% versus 4% in older children (P less than 0.005). When reviewed by the newly proposed IRS cytopathologic classification, there was no difference in pathologic types between the two age groups. Infants younger than 1 year of age had a higher rate of bladder-prostate-vagina primary tumor sites than older children, 24% versus 10% (P less than 0.05). In addition, infants tended to receive less of the prescribed doses of chemotherapy and radiation therapy than older children, and to develop more toxicity to treatment than older children. Despite these differences, the overall survival curve for the two age groups appears to be similar. In contrast to Wilms' tumor and neuroblastoma, in which age (less than 1 year) is a favorable prognostic factor, age does not appear to be an important prognostic factor in rhabdomyosarcoma.

Antineoplastic Agents↗