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At least 487 records · Page 27Linked to original sources
PubMed1999

Radiation late effects in children treated for orbital rhabdomyosarcoma.

BACKGROUND AND PURPOSE: The experience resulting from large cooperative studies shows that correct radiation therapy at doses adequate to the tumor bulk are crucial for local control of rhabdomyosarcoma. The aim of the present study was to document the correlation between modalities and doses of radiotherapy and radiation side effects. PATIENTS AND METHODS: Between 1980 and 1997, 19 patients affected by primary orbital rhabdomyosarcoma have been followed at the University Federico II of Naples. All but three patients, who received 45, 54 and 55 Gy respectively, have been treated by immediate radiation at the dose of 60 Gy, delivered in 2 Gy fractions, five times per week, by cobalt 60 megavoltage equipment. Combined chemotherapy using vincristine and vincristine plus dactinomycin on alternate weeks was also administered as part of induction therapy. RESULTS: An overall survival rate of 94.7% was registered. In our patients the majority of radiation late effects were paid by orbit and ocular adnexa. Side effects to lens and ocular structures were fewer and of low grade. CONCLUSIONS: Radiation therapy is still essential for local control of orbital rhabdomyosarcoma, however radiation side effects have to be carefully considered together with the therapeutic goal to be obtained.

Child↗
PubMed1991

HLA class I and II expression in rhabdomyosarcomas.

The expression of class I and II histocompatibility antigens was studied in 43 specimens of skeletal and in 36 specimens of cardiac muscle. Expression was also analyzed in the tumoral counterpart of striated muscle tissue, in 21 rhabdomyosarcomas (13 embryonic, 2 alveolar and 6 pleomorphic). Normal striated muscle showed very weak HLA class I antigen expression and no class II expression. The rhabdomyosarcomas showed increased class I expression, possibly related to malignant transformation of this type of tissue and with the degree of cellular differentiation. In our series of rhabdomyosarcomas, we observed class II neoexpression only in some HLA class I positive specimens, which was unrelated to the degree of cellular differentiation in these tumors.

Cell Differentiation↗
PubMed2004

[Paratesticular rhabdomyosarcoma. A case report].

The rhabdomyosarcomas are rare tumors of mesenchymal tIssue. Whose most frequent localisations are génito-urinary, and occur in the child and young adult. It's an intrascrotal tumour, localised in the tunica vaginalis, epididymis or spermatic cord. The Authors report a case of paratesticular rhabdomyosarcoma in a 15 Year old child, who presented a right painless scrotal mass since two Months. The research of the tumoral markers is negative. Scrotal ultrasound shows a tissular mass in spermatic cord. Abdominal ultrasound and CT show retroperitoneal lymph node mass. Chest X-ray is normal. The intervention consists of a right castration. The histological examination of the spiceman confirms the diagnosis of the paratesticular rhabdomyosarcoma of the spermatic cord. The patient is treated by chemotherapy without improvement. Through this observation, the Authors underline the interest of the imagery and the aspects of the paratesticular tumours in ultrasound and CT.

Adolescent↗
PubMed1985

The rarity of rhabdomyosarcomas in the adult. A morphologic and immunohistochemical study.

An analysis was made of the rhabdomyosarcomas diagnosed in the Dept. of Pathology of the University of Groningen between 1971 and 1983. Ten cases diagnosed in patients over 30 years of age were studied in detail. After review the diagnosis was discarded on morphologic criteria in all cases. In 9 cases it was changed into malignant fibrous histiocytoma (MFH) and in one case this diagnosis was favoured, but inconclusive. In 5 cases immunohistochemical studies could be performed. In all cases staining for the muscle specific intermediate filaments desmin appeared negative and for the mesenchymal intermediate filaments vimentin positive. These cases were also positive for one or more of the histiocytic markers alpha-1-antichymotrypsin, alpha-1-antitrypsin and lysozyme. It is concluded that rhabdomyosarcoma in older patients is extremely rare and the possible relationship between MFH in the adult and rhabdomyosarcoma in childhood is discussed.

Adult↗
PubMed1989

Laryngeal botryoid rhabdomyosarcoma in an adult. Report of a case with electron microscopic study.

Light and electron microscopic studies of botryoid rhabdomyosarcoma of the larynx in an adult are presented. Differences in cellular composition of the central and peripheral regions of the tumor are described. The ultrastructure of the rhabdomyoblasts is discussed on the basis of criteria recently described. The findings support the hypothesis that botryoid rhabdomyosarcoma is a special subtype of rhabdomyosarcoma with low grade malignancy.

Adult↗
PubMed1996

Low concentrations of actinomycin D potentially cause therapeutic differentiation in human rhabdomyosarcoma cell line RD.

Neoplastic transformation may be an alteration in the process of cell maturation that leads to an infinite capacity for proliferation. Because the cytodestruction caused by most drugs available for cancer chemotherapy is often accompanied by significant morbidity and poor response, the induction of differentiation has been proposed as an alternative approach to conventional anticancer therapy. We used human rhabdomyosarcoma cell line RD to analyze the differentiation process induced by actinomycin D, a drug of choice in the conventional treatment of rhabdomyosarcomas. Low concentrations of actinomycin D induced a terminal process of morphological and ultrastructural myogenic differentiation in rhabdomyosarcoma cells, which concluded with cell death. However, this potential therapeutic effect cannot be considered complete because of the presence of tumoral cells that are heterogeneous with respect to actinomycin D chemosensitivity. This heterogeneity led to the appearance of foci of resistant cells which, despite their greater degree of differentiation in comparison with the parental cell line, escaped from terminal myogenic differentiation. This subgroup of tumoral cells may be responsible for the failure of cytotoxic treatment.

Cell Differentiation↗
PubMed1998

Embryonal rhabdomyosarcoma of the nose in an elderly patient, case report and review of the literature.

Embryonal rhabdomyosarcoma of the head and neck is very rare in adults. We report on an embryonal rhabdomyosarcoma of the nose, occuring in a 74-year-old female patient presenting with nasal congestion as the only clinical symptom. Suspected to be an amelanotic melanoma, further biopsies and histological examinations provided the correct diagnosis. She first underwent a course of chemotherapy which led to a partial shrinkage of the tumor mass which was followed by a complete resection of the residual tumor mass. Biologic behaviour of this tumor must be fully appreciated as treatment is interdisciplinary. Rhabdomyosarcoma should be considered in the differential diagnosis of tumors of the nose and paranasal sinuses not only in children, but also in adults.

Age Distribution↗
PubMed1988

Embryonal rhabdomyosarcoma.

Rhabdomyosarcoma is one of the most common malignancies of the mesenchymal tissue in the head and neck region. A case of a 26-year-old male with oral rhabdomyosarcoma is presented. The clinical and pathologic aspects of this malignancy are also reviewed. Furthermore, the histopathologic and ultrastructural features of this neoplasia are described and the important role of electron microscopy in diagnosis of the myogenic tumor is emphasized. Finally, different modes of treatment for the rhabdomyosarcoma are discussed.

Adult↗
PubMed2000

Vascular targeting of solid tumours: a major 'inverse' volume-response relationship following combretastatin A-4 phosphate treatment of rat rhabdomyosarcomas.

Tumour-specific vascularisation may be therapeutically approached in two different ways: by antiangiogenic treatments specifically directed to dividing and migrating endothelial cells, or by agents that target principally the inadequate and ill-structured tumour vasculature. Combretastatin A-4 phosphate (combreAp), a recently synthesised prodrug (OXiGENE, Lund, Sweden), is a vascular targeting agent of the latter kind. We evaluated the effect of a single intraperitoneal (i.p.) combreAp injection on the growth of rhabdomyosarcomas syngeneic in WAG/Rij rats. Different tumour volume groups, ranging between 0.1 and 27 cm(3), were selected to assess the relationship between the size at treatment time and the response to combreAp. A double combreAp treatment (2x25 mg/kg) was investigated within the same overall aim: the relationship between growth delay and tumour size. Our results show that the systemic administration of combreAp induces a clear-cut differential growth delay in the solid rat rhabdomyosarcomas: with very large tumours (>/= 14 cm(3)), a 17.6-fold stronger effect was measured than with very small tumours (<1 cm(3)). This is the 'inverse' of the volume-response seen with the conventional therapeutic approaches (radiotherapy, chemotherapy or surgery). These combreAp antitumour responses were observed without treatment limiting systemic toxicity in the rats. With clinical digital subtraction angiography, using microsurgical cannulation of a major tumour draining vessel, and with histopathology, we demonstrate that growth delay is related to an early (within 3-6 h) and extensive breakdown of tumour blood vessels. The experiments involving a second injection also indicate a volume-dependent effect of combreAp in reducing the regrowth rate of small or large rhabdomyosarcomas. This significant differential volume-response obtained with 'selective' vascular targeting, stronger in larger tumours than smaller ones, suggests the potential of broadening the therapeutic window.

Angiography, Digital Subtraction↗
PubMed1999

Embryonal "Botryoid" rhabdomyosarcoma of the larynx: a clinicopathologic and immunohistochemical study of two cases.

Two cases of embryonal rhabdomyosarcoma of the larynx are reported. The tumors occurred in a 16-year-old boy and in a 66-year-old man. They manifested clinically with nonspecific symptoms, including voice hoarseness and sense of throat fullness. Treatment consisted of total and partial laryngectomy, respectively. Grossly, both lesions had an exophytic growth pattern and microscopically featured a proliferation of small round to oval cells. Cell cytoplasms were occasionally stainable and fibrillary. Quite often, tumor cellularity was denser beneath the covering mucosa, recalling a "cambium layer" pattern. Tumor cells immunoreacted for desmin, actins, myoglobin, and sarcomeric actin; no immunostaining was noted for epithelial markers. No further antitumoral treatment was administered after surgery. There has been no recurrence of tumor at 2 and 10 years, respectively. Based on our series and the available literature, it seems that rhabdomyosarcoma of the larynx pursues a less-aggressive course than that seen in the homonimic juvenile or adult soft tissue lesion. Surgery alone appears to be a valid treatment option, especially when a polypoid, or "botryoid" gross pattern, coupled with the embryonal small cell histotype is encountered. In light of these findings, it is suggested that botryoid rhabdomyosarcoma of the larynx may deserve a specific consideration among the various laryngeal mesenchymal malignancies.

Adolescent↗
PubMed1975

Rhabdomyosarcoma of the larynx.

A case of rhabdomyosarcoma of the larynx in a 54-year-old man is reported. Patient presented dramatically with a large tumour mass obstructing his laryngeal inlet. The patient remains alive and well sixteen months after a total laryngectomy. Only seven previous reports of a rhabdomyosarcoma of the larynx could be found in the literature and these are compared with the case reported. It would seem that a rhabdomyosarcoma of the larynx has a better prognosis than elsewhere in the body, probably because of its earlier recognition and accessibility to radical surgery.

Biopsy↗
PubMed1997

Media from rhabdomyosarcoma and neuroblastoma cell cultures stimulate in vitro aggregation and fibrillization of amyloid beta-protein.

In vitro aggregation and fibrillization of synthetic amyloid beta-protein Abeta 1-40 was assessed in the conditioned media from rhabdomyosarcoma (CRL 1598, HTB 82, HTB 153, CCL 136), adenocarcinoma (CCL 218), neuroblastoma (SY5Y), and COS cells cultured in the absence and presence of 10% heat-inactivated fetal bovine serum (FBS). The aggregation and formation of cross beta-pleated sheet structures in Abeta was quantitated by Thioflavin T (ThT) fluorescence spectroscopy, while the morphology of Abeta fibrils was examined in negative staining in the electronmicroscope (EM). In cultures supplemented with 10% FBS, the conditioned media from CRL 1598, HTB 82, CCL 218, and SY5Y cell cultures stimulated Abeta aggregation in a time-dependent manner as compared to that of control (serum-containing medium that had not been exposed to cells). The order of stimulation was SY5Y > CRL 1598 > or = HTB 82 > CCL 218, and the stimulation was higher in 2 week cultures than in 1 week cultures. Similar studies using media from HTB 153, CCL 136 and COS cell cultures showed no effect on Abeta 1-40 aggregation. In serum-free cell cultures, only media from SY5Y and CRL 1598 could promote significant aggregation of Abeta 1-40. Negative staining in EM revealed Abeta fibril formation only with conditioned media from SY5Y and CRL 1598 cultured under serum free conditions; no Abeta fibrils were noticed in media from cell cultures supplemented with 10% FBS. We propose that both the SY5Y neuroblastoma cell line and the CRL 1598 rhabdomyosarcoma cell line may serve as experimental models for in vitro studies of extracellular aggregation and fibrillization of Abeta-protein in cell cultures, while rhabdomyosarcoma HTB 82 and adenocarcinoma CCL 218 may be models for study of Abeta aggregation only.

Adenocarcinoma↗
PubMed2000

Unusual second malignancies following radiation therapy: subcutaneous pleomorphic rhabdomyosarcoma and cutaneous melanoma. Two case reports.

BACKGROUND: Among nonepithelial second neoplasms which are known to be induced by irradiation, rhabdomyosarcomas are extremely rare, and melanomas are infrequent. We report a high-grade sarcoma with rhabdomyoblastic differentiation, which appeared 30 years after megavoltage irradiation for an endometrial adenocarcinoma, and a malignant melanoma which arose after 6 years in the irradiation field of a fibrosarcoma. METHODS: Histology and immunohistochemistry were performed in both cases. In the first case, electron microscopy was also performed. In the second, the previous tumor was re-evaluated. RESULTS: The first case showed histological, immunohistochemical and ultrastructural features of a rhabdomyosarcoma. In the second case, a lentigoid malignant melanoma was histologically and immunohistochemically demonstrated, whereas the previously resected tumor was a fibrosarcoma negative to melanoma markers. CONCLUSIONS: Rare cases of rhabdomyosarcomas and melanomas are induced by irradiation, although in some cases other factors (i.e., genetic risk, chemotherapy) may have a prominent etiopathogenetic role in their development. A close follow-up and a careful examination of the irradiated area should facilitate an early diagnosis of these aggressive postradiation second neoplasms.

Adenocarcinoma↗
PubMed1989

Fetal calf serum and retinoic acid affect proliferation and terminal differentiation of a rat rhabdomyosarcoma cell line (BA-HAN-1C).

We report on the establishment of a model for differentiation induction in sarcomas, using the clonal rhabdomyosarcoma cell line BA-HAN-1C. This rhabdomyosarcoma cell line is composed of morphologically undifferentiated mononuclear stem cells, some of which spontaneously fuse to form terminally differentiated multinuclear myotube-like giant cells. The deprivation of fetal calf serum (FCS) or the exposure to retinoic acid, respectively, resulted in a significant inhibition of proliferation (P less than 0.001) and a marked increase in cellular differentiation as shown by a significant increase in the number of myotube-like giant cells (P less than 0.001) and in the creatine kinase activity (P less than 0.05) used as a biochemical marker of differentiation. Furthermore, after exposure to retinoic acid about 30% of the mononuclear tumour cells exhibited morphological features of rhabdomyogenic differentiation, such as bundles of thick and thin myofilaments, which had never been observed in the mononuclear cells of untreated cultures. These results confirm that the inverse linkage between proliferation and differentiation known from embryonic myogenesis is preserved in our rhabdomyosarcoma cell line. The failure to induce terminal differentiation by exposure to retinoic acid in all the cells of our clonal cell line indicates that some tumour cells might epigenetically be blocked from responding to retinoic acid. The temporary growth retardation observed after FCS-deprivation suggests that autocrine stimulation of proliferation may be operating in our cell line, too.

Animals↗
PubMed1991

Expression of members of the myf gene family in human rhabdomyosarcomas.

Northern analysis of tumour RNA has been used to examine the expression of members of the myf family of muscle determining genes (myf3, myf4, myf5 and myf6) in a series of 20 rhabdomyosarcomas. A 2.0 kb myf3 transcript was observed in 85% of tumours, a 1.8 kb myf4 transcript was detected in 70% of tumours and a 1.7 kb myf5 transcript was observed in 55% of tumours. Transcription of myf6 occurred in 28% of tumours, but there were several transcript sizes (1.2, 1.5, 2.0 and 3.5 kb) and in some individual tumours two or more transcripts were observed. Only two rhabdomyosarcomas, one classified as embryonal and one as pleomorphic, failed to exhibit transcription of members of the myf gene family. We were unable to detect transcription of myf genes in neuroblastomas, Wilms' tumours, hepatoblastomas, paediatric non-Hodgkin's lymphoma and leiomyosarcomas. When considered together these observations suggest that expression of myf genes could provide an extremely useful marker in the diagnosis of rhabdomyosarcoma.

Adolescent↗
PubMed1993

Uncoupling of growth inhibition and differentiation in dexamethasone-treated human rhabdomyosarcoma cells.

The effects of dexamethasone, a synthetic glucocorticoid, and of N,N-dimethylformamide on in vitro growth and differentiation and on proto-oncogene expression of human rhabdomyosarcoma cells were studied. RD/18 clone cells (derived from the embryonal rhabdomyosarcoma cell line RD) treated with 100 nM dexamethasone showed an almost complete block of differentiation: about 5% myosin-positive cells were observed after 2 weeks of culture in dexamethasone-supplemented differentiation medium, compared to 20% of untreated cultures. Dexamethasone also induced a 20-30% growth inhibition and a more flattened morphology. The treatment with N,N-dimethylformamide induced a significantly increased proportion of myosin-positive cells (reaching about 30%) and a 40% growth inhibition. Induction of differentiation inversely correlated with the levels of c-myc proto-oncogene expression: after a 2 week culture dexamethasone-treated cells showed the highest c-myc expression and N,N-dimethylformamide-treated cells the lowest. Culture conditions per se down-modulated c-erbB1 and up-regulated c-jun expression, with no relationship to the differentiation pattern. Other proto-oncogenes were not expressed (c-sis, N-myc, c-mos, c-myb) or were not modulated (c-fos, c-raf). Therefore dexamethasone and N,N-dimethylformamide, both causing a decreased growth rate, showed opposing actions on myogenic differentiation and on c-myc proto-oncogene expression of human rhabdomyosarcoma cells.

Cell Count↗
PubMed1995

Redundancy of autocrine loops in human rhabdomyosarcoma cells: induction of differentiation by suramin.

Three human rhabdomyosarcoma cell lines were used to investigate the presence of autocrine loops based on the production of insulin-like growth factor (IGF)-II, basic fibroblast growth factor (bFGF) and epidermal growth factor (EGF)/transforming growth factor (TGF)-alpha and of their corresponding receptors, and whether these loops affect cell proliferation and myogenic differentiation. Two cell lines, RD/18 and CCA, deriving from tumours of the embryonal histotype, showed the presence of both growth factors and receptors which make possible three different autocrine loops, while the alveolar RMZ-RC2 cell line lacked that based on the EGF receptor. Culture of rhabdomyosarcoma cells in the presence of specific blocking antibodies, directed to a component of single autocrine loops, inhibited cell proliferation (up to 50%), without inducing myogenic differentiation. Suramin, a drug which non-selectively interferes with the binding of growth factors to their cellular receptors, was used to block all the autocrine loops simultaneously. In CCA and RMZ-RC2 cells suramin was able to induce a significant increase (up to 3-fold) in the proportion of myosin-positive cells over control cultures. Therefore rhabdomyosarcoma cells of embryonal and alveolar histotype can show a redundancy of growth-sustaining autocrine loops. Suramin could interfere with them by acting on both growth inhibition and induction of myogenic differentiation.

Base Sequence↗