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At least 307 records · Page 17Linked to original sources
PubMed2002

Rhabdomyosarcoma arising from a dormant dumbbell ganglioneuroma of the lumbar spine: a case report.

STUDY DESIGN: A case report of a lumbar dumbbell tumor that showed a sequential change in histology from ganglioneuroma into rhabdomyosarcoma after long-term dormancy. OBJECTIVE: To present a case of non-neurogenic malignancy arising from ganglioneuroma. SUMMARY OF BACKGROUND DATA: Ganglioneuromas rarely form the dumbbell variety. Whereas conversion of ganglioneuroma into neurogenic malignancies occurs occasionally, between ganglioneuroma and mesenchymal malignancies it has so far been limited to a case in which ganglioneuroma developed after chemo- and radiotherapy for orbital rhabdomyosarcoma. METHODS: A 61-year-old man was referred with a recurrent retroperitoneal tumor that had been excised as ganglioneuroma. Using imaging, histologic, and immunohistochemical studies, a comparative analysis of the primary and recurrent tumors was performed. RESULTS: A total of 44 histologic sections were examined for the primary and the recurrent tumors that had been excised surgically, with an 8-month interval between resections. Histologic and immunohistochemical findings in the primary tumor were consistent with ganglioneuroma, whereas those in the recurrent tumor indicated rhabdomyosarcoma. CONCLUSIONS: It is assumed that the rhabdomyosarcoma arose from a dumbbell ganglioneuroma as a component of malignant ectomesenchymoma.

Disease Progression↗
PubMed2005

Resveratrol inhibits rhabdomyosarcoma cell proliferation.

Rhabdomysarcoma is the most common soft tissue tumour in children under the age of 15. Although the introduction of multimodal treatment programmes, including chemotherapy, radiation therapy and excision have increased the overall survival, the chemotherapeutic agents currently used for the treatment of rhabdomyosarcoma exhibit considerable toxicity. The aim of this study was to investigate the effects and possible mechanism(s) of action of resveratrol on human embryonal rhabdomyosarcoma (RD) cells. Resveratrol is a natural polyphenolic compound produced in a number of edible plants and has received considerable attention as a potential chemopreventive and/or chemotherapeutic agent against various types of cancers. In the present study, resveratrol was shown to inhibit cell proliferation of RD cells in a dose-dependent manner with an IC50 of 48.1 micromol/l and induce an arrest in the S/G2 phase of the cell cycle. As evident from immunocytochemical data, resveratrol treatment increased the size of the RD cells. Furthermore, resveratrol treatment resulted in a significant downregulation of cyclin B expression as demonstrated by western blot analyses. In conclusion, the present study shows that resveratrol exerts a strong inhibition of rhabdomyosarcoma cell proliferation in part by arresting cells in S/G2 phase of the cell cycle. These findings warrant further investigation to establish potential use of resveratrol as a relatively non-toxic chemotherapeutic agent for the treatment of rhabdomyosarcoma.

Anticarcinogenic Agents↗
PubMed1997

Detection of gene fusions in rhabdomyosarcoma by reverse transcriptase-polymerase chain reaction assay of archival samples.

Alveolar rhabdomyosarcoma is a pediatric soft-tissue tumor that is often difficult to distinguish from other small round-cell tumors. The PAX3-FKHR and PAX7-FKHR gene fusions that result from chromosomal translocations in this tumor provide potential molecular diagnostic markers. To apply these molecular markers to commonly available archival material, we used reverse transcriptase-polymerase chain reaction and oligonucleotide hybridization methodology to develop an assay capable of identifying PAX3-FKHR and PAX7-FKHR fusion transcripts in formalin-fixed, paraffin-embedded tissue. Use of a control assay for wild-type FKHR mRNA indicated that RNA was successfully isolated, reverse-transcribed, and amplified in 15 of 16 archival cases. Comparison of assay results for the PAX3-FKHR and PAX7-FKHR fusions with standard molecular assays of paired frozen material revealed that all eight cases of known fusion-positive rhabdomyosarcoma were correctly identified and distinguished as PAX3-FKHR or PAX7-FKHR. The seven cases of known fusion-negative rhabdomyosarcoma showed no evidence of either product. These results indicate that we have developed a molecular assay that accurately identifies the fusion transcripts characteristic of alveolar rhabdomyosarcoma in archival samples. This assay will be useful for diagnosis and for retrospective clinicopathologic correlative studies.

Base Sequence↗
PubMed2001

Surgery for urogenital rhabdomyosarcoma.

The treatment and prognosis of rhabdomyosarcoma of the urogenital tract have changed dramatically over the past few decades. Until the 1970s radical cystectomy and pelvic exenteration were the only therapeutic options in rhabdomyosarcoma of the bladder/prostate and of the vagina/uterus. Since then, multiagent chemotherapy has achieved remarkable cure rates. Consequently, organ-saving surgical techniques were advocated, with the goal to improve patients' quality of life. The available long-term survival data with these techniques demonstrate excellent survival in rhabdomyosarcoma of the vagina/uterus, with a high percentage of organ preservation. On the contrary, increased rates of local recurrence and death from disease in patients with rhabdomyosarcoma of the bladder/prostate who undergo primary chemotherapy with or without tumour excision, and complications following combined radiotherapy critically question the long-term benefit of these organ-sparing surgical techniques in this group.

Female↗
PubMedDate not supplied

A phase II trial of high-dose methotrexate in previously untreated children and adolescents with high-risk unresectable or metastatic rhabdomyosarcoma.

PURPOSE: The outcome for children with advanced-stage rhabdomyosarcoma remains poor with contemporary treatment regimens. Evaluation of new drugs is important to improve clinical outcome. Because methotrexate has shown promising activity in the treatment of patients with recurrent rhabdomyosarcoma, we conducted a phase II trial in untreated children with advanced-stage disease to evaluate the efficacy and safety of this agent. PATIENTS AND METHODS: Fifteen patients received 1 to 4 courses of high-dose methotrexate (HDMTX, 12 g/m2). Patients then received standard multiagent chemotherapy (vincristine, dactinomycin, cyclophosphamide, ifosfamide, mesna) with cytokine support and local radiotherapy. Patients who responded to HDMTX received four additional courses of this drug during continuation therapy. RESULTS: Twelve patients were evaluable for response after 2 or more courses of HDMTX; 4 achieved a partial response (33.3%). After administration of standard chemotherapy and radiation, the estimated 2-year progression-free survival for all patients was 56% (SD 15%). The drug was well-tolerated and the most common side effects included mucositis, transient elevation of transaminases, and neutropenia. The four patients who received additional courses of HDMTX during continuation therapy had limited toxicity which included mucositis, anemia, and thrombocytopenia. CONCLUSIONS: About one-third of children with previously untreated advanced-stage rhabdomyosarcoma responded to HDMTX. Its different mechanism of action and non-overlapping toxicity with other agents make HDMTX an attractive candidate for incorporation into front-line treatment regimens for rhabdomyosarcoma.

Adolescent↗
PubMedDate not supplied

Hypercalcemia in children with rhabdomyosarcoma.

PURPOSE: Hypercalcemia complicating malignancy is a serious and frequent occurrence in adults but rare in children. The aim of this study was to determine the incidence, symptoms, outcome, and cause of hypercalcemia in children with rhabdomyosarcoma. PATIENTS AND METHODS: Among 93 children with rhabdomyosarcoma, six with hypercalcemia were identified and their clinical course, pathologic findings, treatment, and outcome were evaluated. In addition, serum and intratumor parathyroid hormone-related protein (PTHrP) concentrations were measured to ascertain the potential of hypercalcemia. RESULTS: All six patients with hypercalcemia had bone metastasis, and four with severe hypercalcemia had multiple bone metastases. Serum PTHrP levels were moderately elevated. There were no significant trends detected based on age, gender, primary site, and pathologic type. All patients achieved transient resolution of hypercalcemia after treatment. However, none survived for a long period of time because their original tumor eventually recurred. CONCLUSIONS: Hypercalcemia is not an uncommon complication in patients with rhabdomyosarcoma. Bone absorption seems to be the predominant factor that induces the hypercalcemia in rhabdomyosarcoma. However, PTHrP may be a powerful stimulator of bone absorption, which contributes to the growth of bone metastasis.

Adolescent↗
PubMedDate not supplied

Presence of well-differentiated rhabdomyoblasts at the end of therapy for pelvic rhabdomyosarcoma: implications for the outcome.

The presence of well-differentiated rhabdomyoblasts at the end of therapy for rhabdomyosarcoma has been noted. This study was undertaken to investigate the therapeutic implications of the presence of well-differentiated rhabdomyoblasts at the end of therapy for pelvic rhabdomyosarcoma. Six patients with pelvic rhabdomyosarcoma (bladder-prostate, 4; vulvovaginal, 2) with disease diagnosed between the years 1974 and 1992 were sequentially investigated by cystoscopic or vaginoscopic examination and biopsy during and after completing therapy. All six patients received treatment according to prevailing therapeutic protocols. Biopsy material from all six patients at the end of therapy documented the presence of well-differentiated rhabdomyoblasts. Repeated biopsies demonstrated the presence of rhabdomyoblasts; however, they appeared to decrease in number with time. Mitotic activity was not observed in the biopsy materials obtained. All six patients are alive without evidence of disease from 37 to 233 months after therapy ended. The presence of well-differentiated rhabdomyoblasts at the end of therapy for pelvic rhabdomyosarcoma is a common finding. The biologic nature of these well-differentiated rhabdomyoblasts is not completely known, but they do not appear to connote the persistent presence of malignant disease and are not an indication for the continuation of therapy.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2003

Effects of therapy on dentofacial development in long-term survivors of head and neck rhabdomyosarcoma: the memorial sloan-kettering cancer center experience.

PURPOSE: To describe potential effects of multimodality therapy on dental and facial development in long-term survivors of head and neck rhabdomyosarcoma. PATIENTS AND METHODS: The medical records of all patients aged 20 years or less presenting between 1985 and 1996 with a diagnosis of rhabdomyosarcoma and treated by protocol were reviewed. Head and neck rhabdomyosarcoma patients who were followed in the Dental Service and were alive and free of disease with at least a 5-year follow-up were included in the review. Ten patients satisfied the inclusion criteria and form the basis of this report. The median age at diagnosis of the 10 patients was 4.3 years (range 10 months to 19.5 years). All patients were treated with chemotherapy, two patients underwent surgery, and all but one patient received external beam radiation therapy. RESULTS: Clinical or radiographic dentofacial abnormalities were observed in 8 of the 10 (80%) patients. Abnormalities included enamel defects, bony hypoplasia/facial asymmetry, trismus, velopharyngeal incompetency, tooth/root agenesis, and disturbance in root development. Bony hypoplasia and disturbance in root formation were the most common findings. CONCLUSIONS: Multimodality therapy for head and neck rhabdomyosarcoma can result in dentofacial abnormalities that affect the patient's quality of life. The care of the long-term survivor requires a multidisciplinary approach, including early involvement of the dental team.

Adolescent↗
PubMed2003

Cyclooxygenase-2 expression does not correlate with outcome in osteosarcoma or rhabdomyosarcoma.

PURPOSE: To determine if expression of cyclooxygenase (COX)-2, an inducible enzyme with known tumor-promoting activity, correlates with outcome in pediatric sarcomas. COX-2 overexpression correlates with more aggressive disease in a variety of adult solid tumors. METHODS: Archived human osteosarcoma, rhabdomyosarcoma, and Ewing sarcoma tumors were retrospectively evaluated, blinded to outcome, for COX-2 expression by immunohistochemistry and correlated with patient characteristics and survival. RESULTS: COX-2 expression was detected in 94 of 142 (66%) tumors (osteosarcoma, 66/99 [67%]; rhabdomyosarcoma, 21/35 [60%]; Ewing sarcoma, 7/8 [88%]) and 51 of 85 (60%) diagnostic biopsies (osteosarcoma, 26/45 [58%]; rhabdomyosarcoma, 21/35 [60%]; Ewing sarcoma, 4/5 [80%]). COX-2 expression did not vary with clinicopathologic features and was not predictive of prognosis in these cases. CONCLUSIONS: This study does not support the use of COX-2 expression as an upfront prognostic variable in patients with osteosarcoma or rhabdomyosarcoma. Results from the small number of patients studied with Ewing sarcoma suggest a similar lack of predictive value for COX-2 expression. However, COX-2 inhibitors are not entirely dependent upon enzyme expression for their antitumor effects; this study does not necessarily preclude the use of COX-2 inhibitors for the treatment of pediatric sarcomas.

Adult↗
PubMed2004

Parameningeal rhabdomyosarcoma in a patient with Costello syndrome.

Costello syndrome, a rare entity with multiple congenital anomalies, has been associated with a high incidence of benign and malignant tumors, particularly rhabdomyosarcoma. Although the head and neck is the most common site for rhabdomyosarcomas in children, only one other case in the literature has been reported of a head and neck rhabdomyosarcoma in a Costello patient. The authors describe a 3-year-old with a parameningeal infratemporal fossa rhabdomyosarcoma. Management is discussed with particular attention to the difficulties encountered due to the congenital heart abnormalities associated with the syndrome.

Abnormalities, Multiple↗
PubMed2003

Primary perineal alveolar rhabdomyosarcoma metastatic to an extraocular muscle.

A 17-year-old boy diagnosed with an alveolar rhabdomyosarcoma involving the perineum and with extensive lymphadenopathy was treated with chemotherapy yet developed metastases to the head and neck 6 months into therapy. Ten months after initial diagnosis, while receiving salvage chemotherapy and radiotherapy, he returned with pain on movement of his left eye, proptosis, and ptosis of the left upper eyelid. Computed tomography (CT) revealed a mass within the left lateral rectus muscle that biopsy confirmed to be metastatic alveolar rhabdomyosarcoma. Despite continued chemotherapy and radiotherapy, he ultimately died of the disease. Alveolar rhabdomyosarcoma from distant sites rarely metastasizes to the extraocular muscles. However, our case shows that alveolar rhabdomyosarcoma may metastasize to the orbit and involve a single muscle.

Adolescent↗
PubMed1988

Rhabdomyosarcoma with bone marrow metastasis simulating acute leukemia. Report of two cases.

Rhabdomyosarcoma manifested as a systemic disease is very rare and cases showing diffuse metastasis in the bone marrow are most unusual. Recently we encountered two cases of rhabdomyosarcoma with diffuse bone marrow metastasis which were clinically manifested as acute leukemia. The first patient was a 15-year-old female, who was admitted in 1982 with pancytopenia and many large primitive cells in bone marrow aspirates, hematological malignancy being diagnosed. Thereafter the bilateral breasts showed rapid swelling and a biopsy specimen revealed the histological features of typical alveolar rhabdomyosarcoma. The primary site of the neoplasm remained undetermined during the course. At autopsy, it was disclosed that the neoplasm originated from the left thigh and showed generalized metastasis. The second patient was a 38-year-old man, who was admitted in 1986 because of a nasal polyp obstructing the nasal cavity, and persistent nasal bleeding. Peripheral blood samples showed leukoerythroblastosis and thrombocytopenia, and large primitive cells were found in bone marrow aspirates, so that hematological malignancy was initially diagnosed. A biopsy specimen of the nasal polyp showed proliferation of large round cells and electron microscopy demonstrated the ultrastructural features of rhabdomyosarcoma.

Acute Disease↗
PubMed1989

Diffuse pleural rhabdomyosarcoma with persistent pleural effusion.

A unique case of embryonal rhabdomyosarcoma arising at the left pleura of a 7-year-old Japanese girl is reported. The present case was characterized by persistent pleural effusion, and the malignant cells incidentally found in it were the first diagnostic clue. The tumor showed a rare growth pattern involving diffuse thickening of the parietal pleura. Biopsy of the thickened parietal pleura upon thoracotomy revealed embryonal rhabdomyosarcoma largely composed of immature mesenchymal cells. Immunohistochemical demonstration of creatinine phosphokinase-MM was most helpful among several types of immunostain for the histopathological diagnosis. Ultrastructurally, thin filaments with primitive Z bands could be seen in some tumor cells. Intensive clinical examination revealed only diffuse thickening of the parietal pleura, which was reduced by chemotherapy. This is the first documented case of rhabdomyosarcoma arising at the pleura. Previous reports of rhabdomyosarcoma arising at unusual sites are reviewed and the histogenesis of this tumor is briefly discussed.

Child↗
PubMed1994

Transrectal ultrasound in the evaluation of rhabdomyosarcoma involving the prostate.

OBJECTIVE: To evaluate transrectal ultrasound as a means of diagnosis and of monitoring patients with rhabdomyosarcoma involving the prostate. PATIENTS AND METHODS: Serial transrectal ultrasonography was utilized to evaluate prostatic rhabdomyosarcoma in three patients. RESULTS: Unlike prostatic adenocarcinoma and transitional cell carcinoma involving the prostate, which are predominantly hypoechoic, the echogenicity of rhabdomyosarcoma is similar to that of the normal prostate. Transrectal ultrasound provided a simple means of monitoring prostate size and sampling tissue in these patients. CONCLUSION: Transrectal ultrasound imaging can be useful in both diagnosis and evaluation of treatment response as well as provide easy access for biopsies in patients with rhabdomyosarcoma involving the prostate.

Adolescent↗
PubMed1993

Deficiency in rhabdomyosarcomas of a factor required for MyoD activity and myogenesis.

Rhabdomyosarcoma cells express the myogenic helix-loop-helix proteins of the MyoD family but do not differentiate into skeletal muscle cells. Gel shift and transient transfection assays revealed that MyoD in the rhabdomyosarcoma cells was capable of binding DNA but was relatively nonfunctional as a transcriptional activator. Heterokaryon formation with fibroblasts resulted in the restoration of transcriptional activation by MyoD and the differentiation of the rhabdomyosarcoma cells into skeletal muscle cells. These results suggest that rhabdomyosarcomas are deficient in a factor required for MyoD activity.

Animals↗
PubMed2006

Liver tumor model with implanted rhabdomyosarcoma in rats: MR imaging, microangiography, and histopathologic analysis.

In compliance with institutional regulations for care and use of laboratory animals, the aim of this study was to establish and characterize a rodent liver tumor model to provide a platform for preclinical assessment of new diagnostic and therapeutic strategies. A rhabdomyosarcoma tumor was implanted in the right and left liver lobes of 20 rats, for a total of 40 tumors. T1- and T2-weighted magnetic resonance (MR) images, diffusion-weighted images, and dynamic susceptibility contrast agent-enhanced perfusion-weighted images were obtained up to 16 days after tumor implantation and were compared with postmortem three-dimensional computed tomographic (CT) images, digital microangiograms, and histopathologic findings. Fifteen tumors were examined with proton ((1)H) MR spectroscopy. All tumors grew, with a mean volume doubling time of 2.2 days +/- 0.9 (standard deviation) and a final size of 591 mm(3)+/- 124. The rhabdomyosarcoma tumor showed hypervascularity at MR imaging, three-dimensional CT, microangiography, and histologic analysis. On dynamic susceptibility contrast-enhanced perfusion-weighted images, the maximum signal intensity decrease differed in time and extent between the tumor and the liver, with a significantly (P < .001) higher relative blood volume, relative blood flow, and permeability value in the tumor than in the liver. With (1)H MR spectroscopy, the rhabdomyosarcoma tumor and the liver featured significant (P < .001) choline and lipid peaks, respectively. Implantation of a rhabdomyosarcoma tumor in the livers of rats is feasible and reproducible, and this animal model seems promising for future testing of new diagnostic and therapeutic strategies.

Angiography↗
PubMedDate not supplied

Rhabdomyosarcoma of the adult nasopharynx.

Rhabdomyosarcoma of the adult head and neck is rare, particularly beyond 40 years of age. In this region, the nasopharynx is an unusual site. Most nasopharyngeal rhabdomyosarcomas are of an alveolar variety. We report a case of embryonal or spindle cell rhabdomyosarcoma in the nasopharynx of a 47-year-old man. The histology of this tumour revealed clear cells that have not been described in embryonal rhabdomyosarcomas occurring in the head and neck. The pathology is discussed and current literature reviewed.

Humans↗
PubMed2006

Botryoid-type pleomorphic rhabdomyosarcoma of the renal pelvis in an adult. A rare case report and review of the literature.

Rhabdomyosarcoma of the renal pelvis is an extremely rare lesion for which only two reports are available. Here, we report another case of botryoid-type pleomorphic rhabdomyosarcoma in the renal pelvis. The tumor produced mild hydroureteronephrosis and right nephrectomy was performed after the needle aspiration cytology revealed abnormal epithelial clusters. The surgical specimen disclosed a polypoid mass attached to the wall of the lower pole of the renal pelvis. Light microscopy examination revealed a large polypoid tumor protruding from the inner surface of the renal pelvis with intact surface urothelium. Immunohistochemical stains for actin, myoglobin, and vimentin were positive. Although rhabdomyosarcoma has formerly been reported to occur at the renal pelvis, to our knowledge, this is the first case of renal pelvis botryoid-type pleomorphic rhabdomyosarcoma diagnosed by light microscopy and immunohistochemical stains.

Aged↗