Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “RHABDOMYOSARCOMA”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 955 records · Page 53Linked to original sources
PubMed1992

Rhabdomyosarcoma coexistent with ovarian mucinous cystadenocarcinoma: a case report.

This report describes a case of ovarian mucinous cystadenocarcinoma associated with rhabdomyosarcoma. Several cases of sarcoma-like mural nodules in ovarian mucinous tumor have been described previously, but only three well-documented cases of true sarcoma have been reported. The first case was a fibrosarcoma associated with a mucinous cystadenoma; the second was an undifferentiated sarcoma in a mucinous cystadenocarcinoma; and the third was a fibrosarcoma occurring in a mucinous cystadenocarcinoma. The occurrence of rhabdomyosarcoma in mucinous cystadenocarcinoma, as reported here, is to our knowledge the first case reported of this unusual combination of tumors.

Cystadenocarcinoma↗
PubMed1998

Outcome analysis of rhabdomyosarcoma of the lower urinary tract.

PURPOSE: Multimodal therapy has resulted in a high cure rate for genitourinary rhabdomyosarcoma. We propose that the strategy of induction chemotherapy followed by excision and reconstruction without radiotherapy may provide a high cure rate without the late sequelae of pelvic radiotherapy. MATERIALS AND METHODS: We reviewed the records of 13 patients with a mean age of 3.3 years diagnosed with rhabdomyosarcoma of the urinary tract from 1986 to 1996. The primary site was the bladder in 4 cases and prostate in 9. After biopsy confirmation of the diagnosis patients were treated with chemotherapy and subsequent surgery, while radiotherapy was reserved for those with residual disease. RESULTS: After induction chemotherapy 8 patients underwent tumor excision via an organ sparing approach, which involved radical prostatectomy and partial cystectomy with or without bladder augmentation. In 5 patients the final pathological analysis showed positive margins. Repeat surgery in 6 patients included complete cystectomy and urinary diversion in 2 who had positive margins and recurrence after radiotherapy, urethrectomy and continent diversion for positive margins in 1, partial cystectomy and continent diversion in 1, and radical prostatectomy, partial cystectomy and continent diversion in 1 with stage IV disease who had local recurrence. In the latter case ileocystoplasty had been performed for a small noncompliant bladder. Six patients with microscopic residual or metastatic disease received radiotherapy. At a mean followup of 5.6 years 11 patients are disease-free. Continence was preserved in all reconstructed cases. Erections were reported in 8 patients, and renal function was maintained in 10. CONCLUSIONS: Our results suggest that urinary tract reconstruction may be safely performed at primary excision surgery, radiotherapy induced morbidity may be minimized by limiting radiotherapy to residual and metastatic disease, frozen section results may be false-negative, potency may be achieved and radiological disappearance of the tumor should be confirmed histologically.

Child↗
PubMed1992

Chemotherapy and surgical resection combined with immediate reconstruction in a 1-year-old child with rhabdomyosarcoma of the maxilla.

Parameningeal head and neck rhabdomyosarcomas of childhood are often considered unresectable and are treated with irradiation and chemotherapy. High-dose radiation therapy has a very long-term detrimental effect on the developing face and also results in many other significant long-range complications. With the availability of advanced craniofacial surgical and free-tissue-transfer techniques, one-stage resection and immediate reconstruction, along with the use of effective preoperative and postresection chemotherapy instead of local radiation, may be the logical approach to the treatment of selected patients with chemosensitive parameningeal head and neck rhabdomyosarcomas.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1991

Mediastinal involvement by alveolar rhabdomyosarcoma: evaluation with computerized tomography.

We have reported a case of alveolar rhabdomyosarcoma with mediastinal involvement occurring in a 15-year-old boy. Computerized tomography revealed an anterior mediastinal mass with internal mammary nodal involvement, which had metastasized from a tumor of the abdominal wall musculature. When making the differential diagnosis of a mediastinal mass with concurrent distant soft tissue and muscle involvement in an adolescent, rhabdomyosarcoma should be considered.

Adolescent↗
PubMedDate not supplied

Immunohistochemical detection of p53 protein in rhabdomyosarcoma: association with clinicopathological features and outcome.

PURPOSE: Alteration in the p53 tumor suppressor gene is the most common tumor specific genetic change identified in most major cancer types including rhabdomyosarcomas. To investigate the overexpression of p53 and its relation to clinical features and outcome in patients with rhabdomyosarcoma (RMS), an immunocytochemical study was performed. METHODS: Formalin-fixed paraffin embedded tissue sections obtained from 42 cases of RMS were immunostained with a mouse monoclonal antibody p53-D07. Staining was assessed by evaluating the percentage of p53 immunopositive cancer cell nuclei. RESULTS: Nuclear accumulation of p53 protein was detected in 8 of 42 (19%) samples. Clinical analyses of patients demonstrated no correlation between positive staining and age, sex, histological subtype, stage and overall survival. This analysis, however, was limited by the small number of patients who demonstrated p53 immunostaining. Nonetheless, a statistically significant association was observed between p53 expression and adverse outcome. Nuclear p53 expression was associated with disease progression or recurrence (p <0.001) and with a worse event free survival (p = 0.0015). CONCLUSION: The nuclear p53 immunoreaction rate is low in RMS, but p53 expression appears to correlate with poor prognosis.

Adolescent↗
PubMedDate not supplied

Phosphotyrosyl proteins in childhood rhabdomyosarcomas: phosphorylation of catenins and components of the insulin-like growth factor type I receptor signaling cascade.

PURPOSE: Rhabdomyosarcomas (RMS) are heterogeneous in their clinical presentation, histology, and cytogenetics. The growth of some RMS cells has been found to be regulated by the tyrosine kinase insulin-like growth factor (IGF) type I receptor. However, RMS cells exhibit variable sensitivity to inhibitors of tyrosine kinases and IGF receptors. Collectively, these heterogeneous features suggest that differences exist in the growth regulatory pathways of RMS. The objective of this study is to identify active tyrosine kinase signal transduction pathways in embryonal and alveolar RMS cells. METHODS: RMS tumor samples and cell lines representing both embryonal and alveolar histologic subtypes have been analyzed by immunoprecipitation and immunoblotting techniques to characterize phosphotyrosyl protein patterns and to identify tyrosine phosphorylated proteins. RESULTS: RMS cells can be characterized based on the patterns of phosphotyrosyl proteins, including the phosphorylation status of the catenin-like protein Cas1 and the signal adapter protein SHC, and the activation of IGF type I receptor signaling cascades including the formation of SHC-GRB2 signal protein complexes and MAP kinase activation. CONCLUSIONS: Rhabdomyosarcomas, especially the embryonal histologic subtype, are heterogeneous at the level of tyrosine kinase signal transduction. It will be important to characterize the growth regulatory pathways active in individual RMS tumors before targeting molecular therapies to this malignancy.

Adaptor Proteins, Signal Transducing↗
PubMedDate not supplied

Recurrent rhabdomyosarcoma after 25 years: a possible association with estrogen and progestogen therapy.

PURPOSE: We describe a patient with a late recurrence of rhabdomyosarcoma and review the relevant literature. PATIENT AND METHODS: Recurrent rhabdomyosarcoma occurred in a young woman 25 years after initial presentation, with the onset of symptoms 3 months after commencing hormonal replacement therapy with estrogen and progestogen. The primary and recurrent tumors were immunocytochemically identical. The primary tumor was steroid receptor negative but the recurrent tumor was estrogen and progesterone receptor positive. DISCUSSION: Estrogen priming can stimulate synthesis of progesterone receptors which may modulate mitotic activity, which suggests a functional role for receptor positive cells in modulating cell growth when sex hormone primed, with a possible tumor induction role of sex hormone replacement therapy. CONCLUSION: The late tumor recurrence may have been induced by estrogen and progestogen treatment.

Adult↗
PubMedDate not supplied

Rhabdomyosarcoma in a patient with cardio-facio-cutaneous syndrome.

A boy with characteristic facial features, pulmonary valvular stenosis, ectodermal abnormalities, growth failure, and mental retardation was admitted for intestinal occlusion at 20 months of age. Clinical findings were consistent with a diagnosis of cardio-facio-cutaneous syndrome (CFC-s), and a huge abdominal mass was evident on computed tomography scan. A biopsy was performed, and embryonal rhabdomyosarcoma was diagnosed. Molecular analysis was performed by reverse transcription (RT) polymerase chain reaction (PCR) on tumor RNA to seek the chimerical transcript of the most common soft tissue sarcoma translocations and analyze neurofibromatosis 1 (NF1) gene expression. Translocations involving 1;13, 2;13, and 11;22 were not found, and the specific transcripts of the NF1 gene were present. Chemotherapy was implemented, but the child died 7 months later of tumor progression. Few patients with CFC-s have been described, and their follow-up is not well known. The association of CFC-s with rhabdomyosarcoma has not been reported previously, but other neoplasms have been reported in patients with Noonan syndrome, a condition similar to CFC-s. More observations are needed, but this and other reports suggest there could be a higher risk of malignancy in patients with syndromes in the Noonan phenotype category.

Adult↗
PubMedDate not supplied

Role of high-dose chemotherapy with hematopoietic stem cell rescue in the treatment of metastatic or recurrent rhabdomyosarcoma.

PURPOSE: This review summarizes the published data on the use of high-dose chemotherapy and hematopoietic stem cell rescue (HSCR) in the treatment of recurrent or metastatic rhabdomyosarcoma (RMS). PATIENTS AND METHODS: Three hundred eighty-nine patients were identified from 22 articles selected by computer generated searching of MEDLINE (1979-present). One hundred seventy-seven patients had stage 4 disease and were treated during first complete remission (CRI). The remaining patients were treated during CR1/first partial remission (PR1) (110 patients), CR2/PR2 (53 patients), CR2 (12 patients), CR3 (1 patient), or treated with disease (36 patients). RESULTS: Patients treated during CR1 or CR1/PR1 had event-free survival (EFS) rates ranging from 24% to 29% at 3 to 6 years from diagnosis and overall survival (OS) rates ranging from 20% to 40% at 2 to 6 years after diagnosis according to data provided as Kaplan-Meier estimates. Studies without Kaplan-Meier estimates (n = 32) indicate that 12 patients (38%) with stage IV RMS treated during CR1 or CR1/PR1 were surviving 7 to 60 months from diagnosis, similar to patients with stage IV RMS treated on Intergroup Rhabdomyosarcoma Studies II or III. Patients treated during CR2, CR3, or with evidence of disease had a worse outcome with an estimated 3 years OS of 12% (n = 51). Studies without Kaplan-Meier estimates (n = 27) indicate that four patients (15%) treated during CR2, CR3, or with disease were surviving 17 to 33 months after transplant. CONCLUSIONS: Based on these data, there does not appear to be a significant advantage to undergoing high-dose chemotherapy with HSCR for patients with relapsed or refractory high-risk RMS. Clearly, there is a need for incorporating new treatment strategies for patients with high-risk RMS.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2003

Orbital rhabdomyosarcoma in Noonan syndrome.

The incidence of various benign and malignant tumors is increased in patients with Noonan syndrome compared with the general population. We present a 9-year-old boy with the typical features of Noonan syndrome and an acute nonaxial proptosis of the right eye. An ultrasound scan, a computed tomography scan, and magnetic resonance imaging raised suspicion of rhabdomyosarcoma. Biopsy confirmed the diagnosis. The tumor was consequently treated with combined chemotherapy and radiotherapy. Rhabdomyosarcoma is a rare but important differential diagnosis of tumor formation in children with Noonan syndrome and may arise in the orbit.

Brachytherapy↗
PubMed2003

Embryonal rhabdomyosarcoma presenting as conjunctival tumor.

The authors report the case of a 10-year-old girl who presented with a focal conjunctival lesion of the right eye. Conjunctival biopsy was performed and revealed an embryonal rhabdomyosarcoma. She received chemotherapy alone according to the Malignant Mesenchymal Tumor 95 Study (stage II nonalveolar orbital tumors) and is still in complete remission 6 months after the completion of treatment. Rhabdomyosarcomas of the conjunctiva without orbital extension are rarely reported. The authors discuss the prognosis and differential diagnosis of these lesions.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2003

Intensive induction chemotherapy and delayed irradiation in the management of parameningeal rhabdomyosarcoma.

PURPOSE: To evaluate local control, event-free survival, and overall survival for patients with parameningeal (PM) rhabdomyosarcoma (RMS) treated with intensive chemotherapy and delayed irradiation. PATIENTS AND METHODS: Thirteen consecutive patients with PM RMS were treated with an institutional protocol from 1992 to 1998 at the University of Washington/Children's Hospital and Regional Medical Center and Deaconess Medical Center. Patients received intensive chemotherapy consisting of vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide prior to radiotherapy. Irradiation was delayed, in contrast to current Intergroup Rhabdomyosarcoma Study Group (IRSG) recommendations. RESULTS: Median follow-up was 39 months. Eleven patients had high-risk features, including five with intracranial extension. All patients responded to the intensive chemotherapy, with 38% exhibiting a complete response and the remaining 62% a partial response. Radiation was administered a median of 21 weeks from initiation of chemotherapy. The Kaplan-Meier estimate of 5-year local control was 92%, with event-free survival and overall survival rates of 83%. CONCLUSIONS: With intensive induction chemotherapy, delayed irradiation for PM RMS does not compromise local control. Event-free survival and overall survival rates compare favorably with recently IRSG trials employing early irradiation. Delaying irradiation allows for intensification of chemotherapy and could permit response-based radiation volume and/or dose modifications, which could decrease treatment-related morbidity.

Adolescent↗
PubMed2005

Does the less aggressive multimodal approach of treating bladder-prostate rhabdomyosarcoma preserve bladder function?

PURPOSE: The treatment of bladder-prostate rhabdomyosarcoma has evolved into multimodal therapy, including chemotherapy, radiotherapy and organ sparing surgery with bladder preservation. We investigated bladder function in children who underwent multimodal therapy at our institution and retained the original bladder for at least 6 months after treatment ended. MATERIALS AND METHODS: We evaluated 8 children with bladder-prostate rhabdomyosarcoma treated at our institution between 1999 and 2003 according to inclusion criteria. All patients underwent history, physical examination and urodynamic study at least 6 months after completion of treatment (range 6 to 39 months). RESULTS: All patients were treated following the same chemotherapy and radiotherapy scheme. Three patients (37.5%) were asymptomatic and had normal urodynamic studies, and 1 had only dysuria (this patient later underwent continent urinary diversion with transverse colon). The 4 remaining patients had urological complaints, and the urodynamic findings were reduced bladder capacity in 4, overactivity plus sensory urgency in 2, sensory urgency only in 1 and suprapubic pain during filling in 1. CONCLUSIONS: Among 8 patients 3 had normal urinary function and 4 had minor tolerable alterations. Cystectomy and urinary diversion were later necessary in only 1 patient due to disabling dysuria. The fact that the original functioning bladder was preserved in 7 of 8 patients suggests the feasibility of multimodal therapy. Long-term followup will still be necessary for definite conclusions, since we recognize that the deleterious effects, mainly of radiotherapy, may take longer to become evident.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

Radiation therapy for non-rhabdomyosarcoma soft tissue sarcomas in adolescents and young adults.

Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are less responsive to radiotherapy than the more common pediatric soft tissue sarcomas, rhabdomyosarcoma and Ewing sarcoma of soft tissue. However, radiation therapy does play an important role in the treatment of NRSTS, including synovial sarcoma. Little data exists regarding the behavior or treatment of these tumors in adolescents and young adults compared to older populations. Limb-preservation with adjuvant radiation thereby is standard. There is a greater incentive to reduce long-term complications of radiation in younger patients and thus smaller margins, lower doses, and conformal techniques should be considered. Results of anticipated cooperative group studies promise to guide future therapy for the various types of NRSTS.

Adolescent↗
PubMed2006

Intraocular rhabdomyosarcoma in the sibling of a patient with a cerebellar medulloepithelioma.

A 12-year-old girl with a three-year history of right eye disease was referred to our service after enucleation of her right eye. Histology showed a pleomorphic high-grade rhabdomyosarcoma. As there was extrascleral extension, we elected to treat her with local radiotherapy and adjuvant vincristine, dactinomycin, cyclophosphamide, and adriamycin. She remains disease free 35 months from diagnosis. Notably, a younger sibling died in early childhood as a result of a cerebellar medulloepithelioma. Although there were no teratoid elements in the rhabdomyosarcoma, our patient's unusual disease course would be consistent with rhabdomyosarcomatous differentiation in a teratoid medulloepithelioma.

Cerebellar Neoplasms↗
PubMed2006

Orbital metastasis in patients with rhabdomyosarcoma: case series and review of the literature.

We present 3 cases of children who developed recurrent rhabdomyosarcoma with metastases to the orbit and review the medical literature. Eight additional cases have previously been reported. Alveolar rhabdomyosarcoma was diagnosed in 7 cases. All 11 patients had stage 3 or 4 tumors at diagnosis, but none had orbital involvement initially. The mechanism of orbital recurrence is hematogenous in nature. Ten of the patients died; 7 of them succumbed with progressive disease within 6 months of the orbital recurrence.

Adolescent↗
PubMed2006

Significance of persistent mature rhabdomyoblasts in bladder/prostate rhabdomyosarcoma: Results from IRS IV.

Terminally differentiated rhabdomyoblasts are common after or during therapy for rhabdomyosarcoma (RMS). Case reports have suggested that their presence after therapy does not indicate a poor prognosis, but significance and relationship to outcome has not been systematically studied. Management of patients with this finding can cause confusion. Slides and pathology reports from 44 patients with bladder/prostate RMS treated on Fourth Intergroup Rhabdomyosarcoma Study were examined by a pathologist experienced in RMS, and findings compared with institutional reports. Details regarding patient characteristics, outcome, and management were reviewed. Outcome of patients with various pathologic findings was assessed. One of 10 patients with only mature rhabdomyoblasts at their last procedure recurred, versus 4 of 17 patients with viable tumor and 2 of 17 patients with no viable tumor and no rhabdomyoblasts. Sixteen of 42 cases reviewed had results differing from our review. Mature rhabdomyoblasts are a discrete entity which may not be predictive of recurrence, but should be evaluated by a pathologist experienced with this entity. The presence of mature rhabdomyoblasts at the end of therapy for bladder/prostate RMS does not justify radical surgery. Sequential biopsies are subject to sampling error and should only be performed in the context of protocol-directed therapy to avoid unnecessary radical surgeries.

Antineoplastic Agents↗