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At least 883 records · Page 49Linked to original sources
PubMed1996

Parameningeal rhabdomyosarcoma: results of an international workshop.

PURPOSE: A retrospective analysis was performed on children with nonmetastatic rhabdomyosarcomas (RMS) involving a parameningeal site treated by one of the four major cooperative groups: Intergroup Rhabdomyosarcoma Study (IRS), International Society of Pediatric Oncology (SIOP), German Cooperative Group (CWS), and Italian Cooperative Group (ICS) to analyse survival and prognostic factors. METHODS AND MATERIALS: Between 1979 and 1989, 230 children (median age 6 years) were treated in the IRS III, SIOP 84, CWS 81, and ICS 79 studies. All patients received chemotherapy, and 203 were irradiated. Radiotherapy doses were similar in the four studies, although treatment volumes were not similar. The SIOP patients had smaller volumes treated. In addition, the SIOP patients with a low risk of meningeal involvement and children under 5 years of age were not irradiated if they had a complete response (CR) to chemotherapy. Time to initiation of irradiation was earlier in the IRS and Italian studies. RESULTS: Median follow-up was 62 months (range 22-140). The 5-year survival and 5-year event-free survival were better for the IRS study (74% and 71%) than for the other study groups (55% and 36% for SIOP, 47% and 47% for CWS, and 39% and 39% for ICS). The low-risk (LR) patients in the IRS study had improved survival. However, patients with high risk of meningeal involvement had similar survival in all four studies. The most significant prognostic factor was the size of tumor (> 5 cm). CONCLUSION: The improved results from the IRS group, especially among the LR patients, could be related to the IRS treatment employed, particularly the systematic use of radiation, to the inclusion of patients with smaller tumors, and to the routine use of quality control of radiation.

Adolescent↗
PubMed1999

Microvascular and tumor cell alterations during continuous hyperfractionated irradiation: an electron microscopic investigation on the rat R1H rhabdomyosarcoma.

PURPOSE: Conventionally fractionated y-irradiation results in severe damage of tumor capillaries associated with decreasing oxygen partial pressure within the tumor. The present study was undertaken to assess whether vasculo-connective changes are less pronounced after continuous hyperfractionated irradiation, implying better tumor oxygenation and improved radiosensitivity. MATERIALS AND METHODS: Twenty rats with an isotransplanted R1H rhabdomyosarcoma were irradiated for 12 days with 2 daily fractions of 2.5 Gy (delta t = 6 h). After 0, 15, 30, 45, and 60 Gy, tumor tissue of 4 rats each was analyzed histologically and electron-microscopically. RESULTS: Untreated rhabdomyosarcomas were composed of spindle-shaped tumor cells with numerous mitoses. There were many apoptotic nuclei and a large central necrosis. Tumor capillaries showed a continuous lining of flattened endothelial cells with broad overlapping cell contacts overlying a delicate continuous basal lamina. During irradiation, mean tumor volume declined from 1.9 cm3 to 1.2 cm3. The number of atypical mitoses and apoptoses increased and numerous giant tumor cells appeared. The proportion occupied by necrotic tumor tissue rose from 30% to 60%. After 15 Gy (3 days), a marked vasodilatation was apparent accompanied by an interstitial edema. Occasionally, endothelial cells were rounded up and showed indented nuclei, with the underlying basal lamina disintegrated. These changes progressed with increasing radiation doses. After 30 Gy (6 days), leukocytes started to adhere to the endothelial wall. Electron-dense fine fibrillar and basal lamina-like deposits appeared in the perivascular space. Endothelial cell edema was only observed after 60 Gy (12 days). Cell contact areas were shortened, however, the endothelial lining was not interrupted. No signs of radiation fibrosis were observed. CONCLUSION: Continuous hyperfractionated irradiation induces relatively discrete alterations of the vasculo-connective tumor tissue as compared to conventional irradiation. This may be an advantage with respect to tumor blood flow, oxygenation, and thus, radiosensitivity.

Animals↗
PubMed1998

Metastatic rhabdomyosarcoma presenting as intracranial hemorrhage: imaging findings.

The CNS is rarely the first site of metastasis for rhabdomyosarcoma. CNS involvement is uncommon, and usually seen as leptomeningeal spread after development of pulmonary metastases. We present the imaging findings in a 13-year-old boy in whom a large intracranial hemorrhage was the initial presentation of surgically documented metastatic prostatic rhabdomyosarcoma. Multiple chest CTs and radionuclide bone scans had previously shown no evidence of pulmonary or osseous metastases. The significance of this case in relation to the possible role of the need for use of chemotherapeutic agents that cross the blood-brain barriers to prevent brain metastasis is discussed.

Brain Neoplasms↗
PubMed2001

Sonographic detection of rhabdomyosarcoma of the urinary bladder.

Rhabdomyosarcoma (RMS) is the most common malignant pelvic tumor in the young child, occurring typically in children aged 2-4 years. It arises from the prostate or the trigone of the bladder in boys, and from the vagina or uterus in girls. We report a case of bladder rhabdomyosarcoma and discuss the ultrasonographic images. The mass produced filling defect on the contrast cystogram and also was demonstrated with CT.

Child↗
PubMed1998

Treatment of non-metastatic rhabdomyosarcomas in childhood and adolescence. Results of the second study of the International Society of Paediatric Oncology: MMT84.

The second International Society of Paediatric Oncology (SIOP) study for rhabdomyosarcoma (MMT84) had several goals. The two principal aims were: (1) to improve the survival of children with rhabdomyosarcoma; and (2) to reduce the late effects from therapy by restricting the indications for surgery and/or radiotherapy after good response to initial chemotherapy. A further aim was to investigate the role of high-dose chemotherapy in young patients with parameningeal primary tumours. 186 previously untreated eligible patients entered the study. Patients with completely resected primary tumour received three courses of IVA (ifosfamide, vincristine and actinomycin D). Patients with incompletely resected tumour received six to 10 courses of IVA according to stage. Patients achieving complete remission with chemotherapy alone did not usually receive radiotherapy or undergo extensive surgery, but patients remaining in partial remission received local therapy with surgery and/or radiotherapy. Only patients over 5 years of age with parameningeal disease and patients over 12 years with tumours at any site were given systematic irradiation. Complete remission was achieved in 91% (170/186) of all patients. With a median follow-up of 8 years, the 5-year overall survival was 68% (+/- 3% standard error of the mean (SEM) and the 5-year event-free survival 53% (+/- 4% SEM). These results show an improvement over previous SIOP study (RMS75) in which survival was 52% and event-free survival was 47%. Among the 54 patients who exhibited isolated local relapse, 35% (19/54) survived in further remission longer than 2 years after retreatment, including local therapy (surgery +/- radiotherapy). Analysis of the overall burden of therapy received by all surviving children (including primary treatment and treatment for relapse if required) showed that 24% (28/116) were treated by limited surgery followed by three courses of IVA, 29% (34/116) were treated by chemotherapy alone (after initial biopsy) and 13% (15/116) received chemotherapy plus conservative local treatment (limited surgery or radiotherapy for residual disease). Only 34% (39/116) received intensive local therapy defined as radical wide field radiotherapy or radical surgery or both. Compared with the results obtained in the previous SIOP study, treatment in MMT84 was based on response to initial chemotherapy and, despite an overall reduction of the use of local therapy, significantly improved survival for patients with non-metastatic disease. This trial, also for the first time, provides evidence that retreatment after local relapse can achieve long-term second remissions.

Adolescent↗
PubMed1998

Treatment of intermediate risk rhabdomyosarcoma and undifferentiated sarcoma with alternating cycles of vincristine/doxorubicin/cyclophosphamide and etoposide/ifosfamide.

Over 50% of patients with newly diagnosed rhabdomyosarcoma (RMS) are in the 'intermediate risk' group with a 3-year progression-free survival of approximately 65%. This group consists of stage 1, group III, non-orbit tumours; stage 2, group II and III; and all stage 3 patients utilising the Intergroup Rhabdomyosarcoma Study (IRS) staging system. The role of doxorubicin in the treatment of RMS has been controversial. Ifosfamide, both alone and in combination with etoposide, has significant activity in patients with RMS. The aim of this pilot study was to examine the efficacy and toxicity of a chemotherapy regimen of alternating cycles of vincristine/doxorubicin/cyclophosphamide and etoposide/ifosfamide for intermediate risk RMS. 30 patients with intermediate risk RMS or undifferentiated sarcoma (US) were treated with alternating cycles of vincristine/doxorubicin/cyclophosphamide (VDC) and etoposide/ifosfamide (EI) at planned intervals of 3 weeks. Local treatment of the tumour in most cases was performed after four cycles of chemotherapy, followed by an additional 10 cycles of chemotherapy. At a median follow-up of 37.5 months, the Kaplan-Meier estimate of 3-year event-free survival was 85% (95% confidence interval 72-99%). The overall survival at 3 years was 91% (95% confidence interval 80-100%). No patient died from toxicity. The most common toxicity was febrile neutropenia in 35% of VDC and 26% of EI cycles. No nephrotoxicity or cardiac toxicity was seen. No patient progressed prior to week 12 local therapy. Alternating cycles of VDC and EI are an effective treatment for patients with intermediate risk RMS and US. Toxicity is tolerable. Delaying local treatment until week 12 does not compromise outcome.

Adolescent↗
PubMed1998

A follow-up of rhabdomyosarcoma of the infratemporal fossa region in adults based on the magnetic resonance imaging findings: case reports.

Two adult patients with rhabdomyosarcoma of the infratemporal fossa region were evaluated by computed tomography and magnetic resonance imaging both before and after treatment. Successful treatment accompanied by reduction of the tumor mass as a result of chemotherapy was demonstrated in one patient. In the other patient a subsequent increase in the tumor mass was documented. Information on not only the size but also the internal architecture of the tumor may be useful when tumor response to therapy is being investigated. Magnetic resonance imaging is recommended as a method for outcomes evaluation in adult rhabdomyosarcoma cases.

Adult↗
PubMed2003

Vaginoscopic resection for rhabdomyosarcoma of the vagina: a case report and review of the literature.

Rhabdomyosarcoma is the most common soft-tissue sarcoma found in children. Genitourinary sites comprise 20% of the primary location of these tumors. A polypoid form of the embryonal type of rhabdomyosarcoma, sarcoma botyroides, is often found in girls under age 5. These tumors are usually localized to the anterior vaginal wall. Their superficial location and clinical symptoms lead to early diagnosis, and these tumors are therefore considered to be the easiest to treat and most likely to be cured. In the past 30 years we have seen a shift in treatment from radical surgery to conservative surgery with chemotherapy and radiation, with improved survival and preservation of normal anatomy and improved postoperative body imagery. Conservative excision in the past has been performed by sharp curettage of the anterior vaginal wall. We present a case of a 2-yr-old child with a RMS of the vagina for which we utilized vaginoscopy not only to determine the extent of the tumor but also for precise resection using a bipolar electrode with normal saline as the distension medium.

Child, Preschool↗
PubMed1980

Rhabdomyosarcoma of head and neck in children.

Embryonal rhabdomyosarcoma is a rare condition that tends to occur in the head and neck in children. This disease carried a very poor prognosis until 10 years ago when a multidisciplinary approach (with surgery, therapy and multiple drug chemotherapy) was instituted. In this article a review of the literature on embryonal rhabdomyosarcoma is given and three rare cases have been reported; the first involved the palate, the second affected the ear and mastoid, and the third was a case originating in the nasopharynx with orbital involvement. Clinical and histopathological descriptions are given and the results of therapy are evaluated.

Antineoplastic Agents↗
PubMed1983

Botryoid rhabdomyosarcoma of the nasopharynx.

A case report of a Stage III botryoid rhabdomyosarcoma of the nasopharynx associated with a six-and-a-half-year survival is presented. Treatment consisted of surgery, radiotherapy (6,000 rads TCT) to the nasopharynx and maxillary sinuses bilaterally, and six cycles of polychemotherapy with Vincristine, Adriamycin, Cyclophosphamide and DTIC, without major loss of function or cosmetic deformity. The histology of the lesion is discussed with reference to recent classification and prognosis. The authors suggest that the histological type and prognosis of rhabdomyosarcoma of the nasopharynx in children may be better correlated in future studies.

Child↗
PubMed1988

Embryonal rhabdomyosarcoma of the sphenoid sinus.

Rhabdomyosarcoma is a diagnostic and therapeutic problem in the management of childhood tumours. A case of embryonal rhabdomyosarcoma affecting the sphenoid sinus and involving the cavernous sinus is presented. It has become evident that modern combined modality therapy, including surgery when required, radiation and adjuvant multi-drug chemotherapy offers the best chance of survival.

Child↗
PubMed1993

Rhabdomyosarcoma of the adult head and neck: a clinicopathological and DNA ploidy study.

We investigated the DNA ploidy patterns in thirteen primary and four recurrent rhabdomyosarcomas of the head and neck from thirteen adult patients and correlated the findings with other clinicopathological factors and clinical outcome. Twelve (92.7 per cent) of the primary neoplasms manifested an aneuploid DNA pattern, five had more than one stemline, and one neoplasm displayed a diploid DNA pattern. All recurrent lesions were DNA aneuploid with DNA indices (DIs) corresponding to their primary neoplasms. No correlation between the ploidy pattern and histological subtypes, tumour location, clinical stage and patient's clinical course was found. In this study, only two patients were long-term survivors. Both patients had stage I neoplasms that were located in non-parameningeal sites which manifested an alveolar histological pattern. Our data indicate that adult rhabdomyosarcomas of head and neck are preponderantly DNA aneuploid and are highly aggressive malignant neoplasms. Our results also suggest that tumours which are low stage and in a non-parameningeal location may pursue a less aggressive course.

Adolescent↗
PubMed1984

Embryonal rhabdomyosarcoma of the ear: a review of the literature and case history.

Approximately 50 per cent of all rhabdomyosarcomas in children occur in the head and neck region with the orbit, nasopharynx and ear in order of descending frequency. Embryonal rhabdomyosarcoma is the commonest malignant tumour of the aural region in childhood and its clinical course is usually rapidly fatal, with extensive local disease and or distant metastases (Dehner and Chen, 1978). Other malignant tumours that can occur in children include melanoma and other mesenchymal tumours, including undifferentiated sarcoma, fibrosarcoma, osteogenic sarcomas and Ewing sarcoma. Secondary extension may occur from a meningioma. Osseous disorders of the temporal bone, such as eosinophilic granuloma and Hand-Schüller-Christian disease, should be included as a differential diagnosis (Lewis, 1979).

Child, Preschool↗
PubMed1995

Transglutaminase-catalyzed cross-linking of fibrils of collagen V/XI in A204 rhabdomyosarcoma cells.

Collagens V and XI are thought to form a core around which the major interstitial collagens, I and II, respectively, are organized during fibrillogenesis. We previously reported the presence of a heterotypic form of collagens V and XI, [alpha 1(XI)]2 alpha 2(V), in cultures of A204 rhabdomyosarcoma cells [Kleman, J.-P., Hartmann, D. J., Ramirez, F., & van der Rest, M. (1992) Eur. J. Biochem. 210, 329-335]. This collagen forms a matrix which remains highly insoluble, even when cells were cultured in the presence of beta-aminopropionitrile, an inhibitor of lysyl oxidase and thereby of "classical" collagen cross-linking. When the cells were cultured in the presence of putrescine, a competitive inhibitor of transglutaminase-catalyzed protein cross-linking, a drastic increase in collagen solubility was observed. This result indicates that a transglutaminase contributes to the covalent stabilization of the collagen matrix of these cells. A204 rhabdomyosarcoma cells express tissue transglutaminase as revealed by specific antibodies, and enzyme activity was detected in the cell layer during culture and in cell extracts. Both collagens V and XI are specific glutaminyl substrates for tissue transglutaminase in vitro, as shown by incorporation of [3H]putrescine. The highly homologous alpha 1 chains of collagens V and XI were the major targets for the cross-linking. Trypsin cleaved the [3H] label from the alpha 1 chain of collagen V, demonstrating that the cross-linking occurs in the non triple helical propeptide domains.

Amino Acid Sequence↗
PubMed1998

Primary rhabdomyosarcoma of the brain: observations on a case with clinical and radiological evidence of cure.

Cerebral rhabdomyosarcoma is a highly aggressive tumor with poor prognosis affecting children and, rarely, adults. The authors describe the case of a patient treated for primary fronto-parietal embryonal rhabdomyosarcoma with a long survival (30 months after surgery) and no clinical or radiological evidence of recurrence and discuss the chemotherapy applied in this case.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2002

Primary embryonal rhabdomyosarcoma of prostate in adults: diagnosis and management.

Embryonal rhabdomyosarcoma of the prostate is a common tumour in infants and children. It is rarely seen in adults. The combined modality has improved outcome in children, where as in adults prognosis is poor. The lack of awareness of this entity in adults, delay in diagnosis and a more aggressive behavior of this malignancy are some of the reasons. We report two cases of embroyonal rhabdomyosarcoma of prostate in adults along with review of literature with an aim to define natural history and formulate treatment policy.

Combined Modality Therapy↗