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At least 811 records · Page 45Linked to original sources
PubMed1991

A variant (2;13) translocation in rhabdomyosarcoma.

Cytogenetic analysis of a right buttock mass from a 5-year-old boy showed translocation between an inverted chromosome 1 and a chromosome 13 as the sole cytogenetic abnormality. The breakpoint 13q14 appears to be the same as in previously reported cases of rhabdomyosarcoma (mostly of the alveolar type), but does not show involvement of 2q37. We suggest that this translocation may be a variant of the classical t(2;13)(q37;q14) found in rhabdomyosarcoma.

Child, Preschool↗
PubMed1991

Alveolar rhabdomyosarcoma with del(13q14).

We report a case of disseminated alveolar rhabdomyosarcoma, where chromosome analysis showed a deletion of chromosome 13(q14). This breakpoint is involved in the t(2;13)(q37;q14) previously reported in cases of rhabdomyosarcoma, but this is the first reported case in whom this deletion occurs without involvement of chromosome 2. The possible oncogenic role of the retinoblastoma (RB1) gene located at the breakpoint is discussed.

Adult↗
PubMed1992

12q13 abnormality in rhabdomyosarcoma. A nonrandom occurrence?

We describe two cases of rhabdomyosarcoma with a translocation involving 12q13 as the primary cytogenetic abnormality. Literature review of 35 cases has identified 3 other cases with this abnormality, and we speculate that this may be another nonrandom rearrangement in rhabdomyosarcoma.

Adolescent↗
PubMed1992

Embryonal rhabdomyosarcoma with 100 chromosomes but no structural aberrations.

An embryonal rhabdomyosarcoma was analyzed cytogenetically after short-term culturing. The tumor was located in the scrotum of an 18-year-old man who, at the age of 4, had been treated for a rhabdomyosarcoma in the orbita. The chromosome number was 100. No structural aberrations were present. There were 2-7 copies of each chromosome type with particular excess of chromosomes 8, 12, and 22, and a relative deficit of chromosome 15.

Adolescent↗
PubMed1995

Solid alveolar rhabdomyosarcoma with a t(2;13).

We report a child with a progressive "solid alveolar rhabdomyosarcoma" in whom analysis of tumor cells revealed a t(2;13) translocation characteristic of the classical alveolar subtype. Both subtypes of alveolar rhabdomyosarcoma are associated with a poor response to treatment and the occurrence of this translocation in both is suggestive of a common biologic origin.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1996

der(16)t(1;16)(q21;q13) as a secondary change in alveolar rhabdomyosarcoma. A case report and review of the literature.

Alveolar rhabdomyosarcoma is an aggressive childhood tumor that exhibits muscle cell differentiation. Cytogenetically, it is characterized by t(2;13)(q35;q14); no consistent secondary abnormalities have been reported. Cytogenetic analysis of bone marrow in a case of alveolar rhabdomyosarcoma revealed t(2;13)(q35;q14) and der(16)t(1;16)(q21;q13). The present case and a review of the literature suggest that up to 11% of these tumors possess der(16)t(1;16)(q21;q13). This is similar to the incidence observed in the Ewing family of tumors, where unbalanced der(16)t(1;16) translocations, resulting in partial trisomy of 1q, are regarded as a consistent secondary cytogenetic change.

Child↗
PubMed1996

Clinical and cytogenetic studies of two cases of Klinefelter syndrome with hereditary retinoblastoma and rhabdomyosarcoma.

Two children with Klinefelter syndrome (KS), one associated with bilateral hereditary retinoblastoma (RB) and the other with rhabdomyosarcoma (RMS) are reported. Both were boys and chromosomally mosaic for KS. The hereditary retinoblastoma case yielded 46,XY,del(13)(q12q14.2)/47, XXY(c),del(13)(q12q14.2) in PHA-stimulated lymphocytes. The rhabdomyosarcoma case yielded 46,XY/ 47,XXY(c) in peripheral blood cells whereas tumor revealed trisomy 8, trisomy 7, and t(7;13)(q33;q32) in addition to 46,XY/47,XXyc mosaicism.

Child, Preschool↗
PubMed1995

Pediatric rhabdomyosarcoma of the head and neck.

The optimal treatment for most patients with pediatric rhabdomyosarcoma (RMS) of the head and neck has been shown to be a combination of surgery, chemotherapy and radiation therapy. A retrospective review of patients seen at our institution is presented to analyze the impact of multimodality treatment on survival in 32 pediatric RMS patients. Sixty-five percent (21/32) of the patients were disease free at the most recent follow-up. The 5 year disease free survival was 57% (17/30). Combined modality treatment in 25 patients consisting of chemotherapy, radiation therapy and surgical resection (in 5 patients) rendered 18 (72%) patients free of disease. Only 1 of 7 (14%) patients who underwent surgery alone as primary management was cured. Two of these 7 patients were salvaged, both with combined modality treatment. The Intergroup Rhabdomyosarcoma Study (IRS) protocols have resulted in increased survival in pediatric patients with RMS of the head and neck. Our results reflect the significant improvement since the IRS study was completed.

Adolescent↗
PubMedDate not supplied

Pediatric sarcomas of the head and neck other than rhabdomyosarcoma.

INTRODUCTION: The treatment of children with rhabdomyosarcoma (RMS) has been established in multicenter protocols. There are few data available regarding the treatment of other sarcomas in pediatric patients. METHODS: From 1955 to 1988, a retrospective review of 33 children with sarcomas other than RMS of the head and neck was performed to identify prognostic factors and to determine optimal treatment. Grade, tumor size, and RMS grouping of the tumors were found to be of prognostic value. RESULTS: Eighteen of 33 (54%) patients are currently disease free. Only 3 of 16 (19%) patients treated initially with surgery alone were rendered disease free. However, 6 patients were salvaged with an additional surgical procedure, yielding 9 of 16 (56%) patients disease free treated with surgery alone. Of patients receiving surgery plus radiation therapy, with or without chemotherapy, 7 of 14 (50%) have no evidence of disease. CONCLUSION: After salvage therapy, 5-year disease-free survival was 56% (15 of 27). Unlike rhabdomyosarcoma, complete surgical extirpation is the mainstay of treatment in pediatric sarcomas other than RMS of the head and neck. Adjuvant radiation with or without chemotherapy should be administered when complete resection is not possible.

Adolescent↗
PubMed1990

Rhabdomyosarcoma, presenting as a facial swelling in a child. A case report and review of the literature.

A case of the very rare rhabdomyosarcoma as the more common second primary in a young child who had been treated for bilateral, genetic retinoblastoma is described and the importance early diagnosis emphasised. A review of the literature is presented with special reference to the prognosis of head and neck rhabdomyosarcoma and the various treatment alternatives, related to both new primary and secondary tumours in children with retinoblastoma.

Cheek↗
PubMed1993

Biosynthesis of 10 kDa and 7.5 kDa insulin-like growth factor II in a human rhabdomyosarcoma cell line.

In the present study we have analysed the expression of insulin-like growth factor II (IGF-II) in the human rhabdomyosarcoma cell line IN157.IN157 cells express high levels of three IGF-II mRNAs of 6.0 kb, 4.8 kb and 4.2 kb. In contrast, normal skeletal muscle expresses a negligible amount of IGF-II mRNA. Two forms of IGF-II with molecular masses of 7.5 kDa and 10 kDa, corresponding to the mature IGF-II and IGF-II with a C-terminal extension of 21 amino acids (IGF-IIE21), were secreted into the culture medium at amounts of 17 ng/ml (2.3 nM) and 15 ng/ml (1.5 nM), respectively. IN157 cells also produce IGF binding protein-2. The bioactivity of recombinant IGF-IIE21 was compared with human IGF-I and IGF-II. IGF-I, IGF-II and IGF-IIE21 bound with high affinity to human IGF-I receptors (Kd approximately 1 nM), whereas the human IGF-II/mannose 6-phosphate (IGF-II/Man 6-P) receptor bound IGF-II and IGF-IIE21 with Kd values of 0.5 nM and 2 nM, respectively, and IGF-I with about 500 times lower affinity. IGF-II and IGF-IIE21 stimulated DNA synthesis via the IGF-I receptor, whereas the IGF-II/Man 6-P receptor mediated their rapid internalization and inactivation. During culture of IN157 cells about 50% of their IGF-I receptors were occupied by endogenous IGF-II. We conclude that IN157 cells express high levels of bioactive 10 kDa IGF-II and 7.5 kDa IGF-II that may stimulate the proliferation of rhabdomyosarcomas by interaction with IGF-I receptors on the cells.

Animals↗
PubMed1984

Primary rhabdomyosarcoma of the leptomeninx. Clinical, neuroradiological and pathological aspects.

Report of an eight-year-old boy presenting with untreatable focal epilepsy and slowly progressing hemiparesis combined with personality changes. Continuous diagnostic efforts eventually resulted in the diagnosis of a primary intracranial rhabdomyosarcoma several months later. The delay in making the correct diagnosis was due to the fact that the malignant process originated as a diffuse involvement of the leptomeninx, and only in later stages a tumor mass developed. Nosology and histogenesis of primary intracranial rhabdomyosarcomas are discussed.

Arachnoid↗
PubMed1994

Induction of myogenic differentiation in a human rhabdomyosarcoma cell line by phenylacetate.

Sodium phenylacetate (NaPA) at concentrations ranging from 2 to 10 mM promoted myogenic differentiation of the human alveolar rhabdomyosarcoma cell line KFR. These concentrations inhibited DNA synthesis of the cells in a dose-dependent manner without significant effect on cell viability. The morphological differentiation of small mononuclear elements to terminal, elongated multinuclear structures resembling myotubes was accompanied by the expression of skeletal muscle myosin. The proportion of differentiated myosin-positive cells which was around 0.8-1.7% in control cultures 12 days after seeding was increased by NaPA treatment up to 47%. In the cytoplasm of differentiated cells, features of sarcomerogenesis were observed. These results suggest that NaPA is an effective inducer of rhabdomyosarcoma cell differentiation at concentrations that have been achieved in humans with no significant adverse effects.

Cell Differentiation↗
PubMed1989

Binding and internalization of exogenous glycosaminoglycans in weakly and highly metastatic rhabdomyosarcoma cells.

The fate of exogenous glycosaminoglycans in cultures of strongly (RMS 0) and weakly (RMS 8) metastatic rat rhabdomyosarcoma cells was studied. The time course and concentration dependence of binding and internalization of the radiolabeled sulfated glycosaminoglycans were determined. Weakly metastatic cells took up heparin, heparan and dermatan sulfates into their pericellular compartment at a higher rate than the strongly metastatic RMS 0 cells. The RMS 8 cells exhibited about two times more binding sites for these iduronic acid containing glycosaminoglycans, and internalized higher amounts of them than the RMS 0 cells. The uptake of the chondroitin sulfate into the peri- and intracellular compartments of both cell types was about 5-15% of that of the other glycosaminoglycans studied. The specificity of displacement of the pericellular heparin and dermatan sulfate by the unlabeled glycosaminoglycans indicates the involvement of specific structural features of the polysaccharide chains in the interactions of glycosaminoglycans with the surface of rhabdomyosarcoma cells, beside ionic forces due to the polyanionic character of the glycosaminoglycans. Heparin and heparan sulfate degradation products, mainly large oligosaccharides, were recovered from the surface of RMS 0 cells but were absent on the surface of the RMS 8 cells. About 30% of the internalized heparin and heparan sulfate was present in the partially degraded form in both cell types. Oligosaccharides derived from glycosaminoglycans were not released into the medium. The decrease in the amount of iduronic acid containing glycosaminoglycans internalized by the highly invasive cells seems to be correlated with an increased cell-associated degradation and with an apparent loss of glycosaminoglycan binding sites on the cell surface.

Animals↗
PubMed1989

The influence of extensive bone erosion on local control in non-orbital rhabdomyosarcoma of the head and neck.

To investigate a recent report suggesting extensive bone erosion (EBE) as an important prognostic factor in head and neck rhabdomyosarcoma, a retrospective review was performed of 32 patients with gross residual and/or metastatic non-orbital head and neck rhabdomyosarcoma treated between the years 1971-1987. Treatment consisted of surgery, radiation therapy (median primary dose, 5000 cGy) and combination chemotherapy according to the Memorial Sloan-Kettering Cancer Center (MSKCC) T2 and T6 protocols. The MSKCC staging system was used: Stage II, 23 patients; Stage III, 6 patients; and Stage IV, 3 patients. With a median follow-up of 48 months (range 17 months to 13 years), the overall survival and local control rates were 72% (23/32) and 75% (24/32), respectively. Local control was achieved in 11/11 patients without extensive bone erosion (Stages II, 9/9; III, 1/1; IV, 1/1) as compared to 13/21 patients (Stages II, 10/14; III, 2/5; IV, 1/2) with extensive bone erosion (p = 0.02). Our data appear to support the recently reported finding that extensive bone erosion is an important predictor of local failure.

Adolescent↗
PubMed1993

Combined modality treatment of the rhabdomyosarcoma R1H of the rat: influence of sequence of cisplatin and fractionated irradiation.

The effect of irradiation with 30 fractions of 2 Gy in 6 weeks combined with a single dose of 5mg/kg cisplatin was studied in the rhabdomyosarcoma R1H of the rat and tumor response and normal tissue toxicity were assessed for various combinations of radiotherapy and chemotherapy. Cisplatin was given 3 days before, during (after the 5th and after the 10th fraction), or 3 and 17 days after radiotherapy. Five of the 12 tumors treated with cisplatin injected 3 days after radiotherapy were locally controlled (42%; 95% confidence intervals: 14-70%) as compared to 0/10 for radiotherapy alone (0%; 0-21%; p < 0.02). A similar trend was found for cisplatin injected 17 days after irradiation (2/6 local controls; 33%; 0-71%). With cisplatin given 3 days before radiotherapy 1/13 local controls were observed (8%; 0-22%; p < 0.05 when tested vs. cisplatin 3 days after radiotherapy). Tumor cure was dependent upon tumor size at time of cisplatin administration with 7/9 small tumors (< 2 mm3) cured versus only 2/35 cures of larger tumors (> 2 mm3). By contrast, net growth delay and skin damage were the same for combined modality treatment and for irradiation alone. General toxicity as assessed by body weight change was significantly higher for animals treated with cisplatin before or after radiotherapy, whereas cisplatin during radiotherapy showed equal effects as compared to radiotherapy alone. Although for the rhabdomyosarcoma R1H of the rat the combined modality treatment was shown to be more effective than radiotherapy alone when cisplatin was applied after radiotherapy, general toxicity was also higher for this mode of treatment.

Animals↗
PubMed1995

A phase II trial evaluating selective use of altered radiation dose and fractionation in patients with unresectable rhabdomyosarcoma.

PURPOSE: Between 1987 and 1991, 25 children with advanced rhabdomyosarcoma (20 with IRS Group 3 disease and 5 with Group 4 disease) were entered on a prospective study evaluating selective use of hyperfractionated irradiation (HFI) and reduced-dose conventionally fractionated irradiation (CFI), based on disease status following induction chemotherapy (ifosfamide or melphalan, followed by vincristine, adriamycin, and cyclophosphamide combination) with or without delayed surgery. METHODS AND MATERIALS: Patients with gross disease following induction chemotherapy with or without delayed surgery, and whose primary tumor sites did not involve the central nervous system, received HFI (n = 12) at 1.1 Gy twice-a-day to 59.4-63.8 Gy total. Patients with parameningeal primaries and intracranial disease extension received HFI with initiation of therapy (n = 2). Those with microscopic disease following induction chemotherapy with or without delayed surgery (n = 11) received reduced-dose CFI to 40 Gy. Active follow-up ranges from 28-75 months (median = 43 months) with no patient lost to follow-up. RESULTS: Eighteen patients (72%) are alive and without evidence of disease, including 8 of the children with gross residual disease postinduction therapy. The absolute 2-year continuous local tumor control rate is 86% for all patients. Among the 14 who received HFI, the absolute 2-year continuous local tumor control rate is 75% at 33 to 67 months (median = 38 months) postirradiation. Hyperfractionated irradiation was associated with expected enhancement of acute reactions, which all resolved with conservative medical management. Grade 4 or 5 acute toxicities were not seen. Significant late radiation morbidity has, thus far, been minimal and limited to Grade 1 and 2 events. Among the 11 who received reduced-dose CFI, the absolute 2-year continuous local tumor control rate is 100% at 25 to 70 months (median = 40 months) postirradiation. CONCLUSION: This limited experience suggests that HFI to a dose level of 60 Gy can be used selectively in children with advanced rhabdomyosarcoma left with gross disease following induction chemotherapy, with or without delayed surgery, with an apparent improvement in local control, and minimization of potential late radiation toxicity. Concurrently, those left with microscopic disease following induction therapy can be selectively treated with reduced-dose CFI with excellent local control.

Adolescent↗
PubMed1995

Tumor oxygenation in a transplanted rat rhabdomyosarcoma during fractionated irradiation.

PURPOSE: To quantify the changes in tumor oxygenation in the course of a fractionated radiation treatment extending over 4 weeks. METHODS AND MATERIALS: Rhabdomyosarcomas R1H of the rat were irradiated with 60Co-gamma-rays with a total dose of 60 Gy, given in 20 fractions over 4 weeks. Oxygen partial pressure (pO2) in tumors was measured at weekly intervals using polarographic needle probes in combination with a microprocessor-controlled device (pO2-Histograph/KIMOC). The pO2 measurements were carried out in anesthetized animals under mechanical ventilation and in respiratory and hemodynamic steady state. Tumor pO2 values were correlated to the arterial oxygen pressure paO2, arterial pCO2, and pH determined with a blood gas analyzer. RESULTS: Tumor oxygenation did not change significantly during the 3 weeks of irradiation (up to 45 Gy), from a median pO2 of 23 +/- 2 mmHg in untreated controls to 19 +/- 4 mmHg after the third week. The decrease of the number of pO2 values between 0 and 5 mmHg indicated that an improved oxygenation in the tumors occurred. However, with increasing radiation dose (fourth week, 60 Gy) a significant decrease in tumor oxygenation to a median pO2 of 8 +/- 2 mmHg and a rapid increase in the frequency of pO2 values (35 +/- 4%) between 0 and 5 mmHg was found. CONCLUSION: Improved oxygenation in rhabdomyosarcomas R1H was only present in the early phase of the fractionated irradiation. Radiation does above 45 Gy led to a considerable decrease of tumor oxygenation in the later phase of irradiation.

Animals↗