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PubMed2003

WT1 expression does not disrupt myogenic differentiation in C2C12 murine myoblasts or in human rhabdomyosarcoma.

WT1 encodes a tissue-specific transcription factor important in early mesenchymal differentiation. Altered expression or mutation of WT1 occurs in malignancies derived from such tissues. These include Wilms tumour, a paediatric kidney cancer that may show heterologous differentiation into primitive skeletal muscle, especially in tumours with WT1 mutation. A putative role for WT1 in inhibiting myogenesis has been suggested by transient transfection of C(2)C(12) myoblasts. However, using a more robust model of stable transfectants of C(2)C(12) expressing inducible WT1 isoforms, we found no inhibition of myogenic differentiation. We also investigated a possible role for WT1 in the disrupted myogenesis seen in rhabdomyosarcoma, a paediatric cancer resembling foetal skeletal muscle. WT1 expression levels measured by quantitative real-time reverse transcription polymerase chain reaction were low or absent in those tumours with a PAX-FKHR fusion gene characteristic of the alveolar subtype, and were higher in cases lacking these fusion genes. Overall, there was a weak positive correlation between expression of myogenic differentiation marker genes and WT1 levels. We conclude that expression of WT1 in C(2)C(12) cells and in rhabdomyosarcoma does not inhibit myogenic differentiation.

Animals↗
PubMed1986

Rhabdomyosarcoma involving the genitourinary organs, retroperitoneum, and pelvis.

We report on 17 patients with rhabdomyosarcoma of the genitourinary organs, retroperitoneal, pelvic, or perineal regions. Treatment was refused by three patients with either bladder or prostatic lesions. The remaining patients were treated with combined modality therapy. Five of 6 patients with bladder lesions are free of tumor 24 to 108 months (mean 51) after diagnosis, while one patient died 4 months postoperatively; two patients with paratesticular lesions are free of disease 3 years and 10 months following diagnosis, respectively. Of two patients with perineal lesions, one patient died, probably due to metastases 21 months after diagnosis, while another patient with local tumor recurrence was treated by reexcision with follow-up for 12 months without evidence of tumor recurrence. The worst results were obtained with the four patients with retroperitoneal pelvic lesions; one patient died 3 months after gross resection of tumor, and the other three patients deteriorated in the 4 months after diagnosis. Overall, 7 of 8 operated patients with bladder or paratesticular lesions are free of disease with follow-up for 10 to 108 months. The biological behavior of embryonal rhabdomyosarcoma, with the sex and age distribution and the management are discussed.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1989

Pulmonary septic emboli mimicking metastatic rhabdomyosarcoma.

Central venous catheters have proven to be an important aid for the care of pediatric patients with malignancies receiving chemotherapy. A rare complication of such catheters is pulmonary septic emboli. This report describes a 15-year-old white girl with rhabdomyosarcoma who developed pulmonary nodules while on chemotherapy. These lesions appeared to be metastatic rhabdomyosarcoma. However, an excisional biopsy showed the lesions to be septic emboli. The patient was placed on antibiotic therapy and responded well. She was able to continue with her "front-line" therapy because the nodules were confirmed not to be metastatic disease.

Adolescent↗
PubMed1996

Extremity sarcomas: an analysis of prognostic factors from the Intergroup Rhabdomyosarcoma Study III.

PURPOSE: Prognostic factors for extremity sarcomas have been reported previously, after analysis of Intergroup Rhabdomyosarcomas Studies (IRS) I and II. This report reviews the experience of IRS III (1984-1992), in light of these reported factors, and the pretreatment factors used in the staging system currently being evaluated in IRS IV. The results of treatment of extremity sarcomas in IRS III are reported. METHODS: The charts of all patients entered in IRS III with an extremity-site tumor were reviewed. This group included patients with shoulder girdle and buttock sites. All patients were treated according to IRS III protocols. Survival rates were estimated by the method of Kaplan and Meier, and comparisons among groups of patients were made using a log-rank test. A multivariate analysis was performed to analyze all pretreatment factors that were significant by univariate analysis. RESULTS: Of the 189 patients entered in IRS III with extremity sites, 88 (47%) had the most common alveolar histology. Fifty-nine patients were in group I at the time of presentation (completely resected disease), 48 in group II (microscopic residual disease), 36 in group III (gross residual disease), and 46 in group IV (metastatic disease). By univariate analysis, the significant prognostic factors affecting survival were clinical group, age at time of diagnosis, tumor size, distant metastases, nodal metastases, and local and distant recurrence. By multivariate analysis of pretreatment factors, age at time of diagnosis, nodal metastases, and distant metastases were significant prognostic factors affecting survival; tumor size approached significance. Both the clinical group system and pretreatment staging system (Lawrence/Gehan) predicted significant differences in survival between groups of patients. The lower survival rate among group II and III node-negative patients with a distal tumor (who, with more extensive surgery, could have been in group I) in comparison to group I patients with a distal lesion, approached significance. In patients without distant metastases, survival was significantly different in those patients that had negative nodes from those in whom nodes were not biopsied. CONCLUSION: This review confirms that both clinical grouping and the new pretreatment staging system used in IRS IV can predict the likelihood of survival of children with extremity sarcomas. By multivariate analysis of the elements included in the staging system, nodal metastases, distant metastases, and tumor size were useful in predicting survival. In addition, age was a significant predictor. This study confirms previous suggestions that complete excision with gross and microscopically negative margins is preferable in the treatment of children with extremity rhabdomyosarcomas.

Adolescent↗
PubMed1997

Complications of pelvic exenteration in children who have genitourinary rhabdomyosarcoma.

BACKGROUND: Rhabdomyosarcoma is the most frequently encountered soft tissue sarcoma in children younger than 15 years of age. METHODS: The authors reviewed the early and long-term morbidity rate and clinical outcome of pelvic exenteration for genitourinary rhabdomyosarcoma (GU-RMS) in children. Of 43 patients with this diagnosis who were seen at St Jude Children's Research Hospital between 1963 and 1994, 17 underwent pelvic exenteration. These 13 boys and four girls ranged in age from 6 months to 15 years (median, 3.5 years). Their primary sites included prostate (n = 12), bladder (n = 4), and uterus (n = 1). All patients received multiagent chemotherapy and either preoperative or postoperative radiation therapy. Anterior exenteration was performed in 15 patients and total exenteration in two. The complications were classified as early or late (occurring within or after 30 days from the date of surgery) and as major or minor (depending on the risk of death or physical or functional disability). RESULTS: Thirteen of the 17 patients (76%) had 49 surgery-related complications. Wound infection (24%), fistula, abscess, and malnourishment (12% each) were the most common early major complications, with hydronephrosis (35%), bowel obstruction (24%), acute pyelonephritis, fistula, lymphedema, and ureter stenosis (12% each) making up the majority of late complications in this category. Relatively frequent minor complications included protracted paralytic ileus and hematuria (18%), cholelithiasis, chronic diarrhea and, peristoma skin irritation (12%). Disease-free survival was associated with the timing of surgery (P = .002). All but one of the 12 patients who underwent surgery within 6 months of diagnosis are alive, compared with only one of five whose surgery was performed after a longer interval. Pelvic exenteration for GU-RMS was associated with a high rate of serious complications in this series of patients treated over 31 years. CONCLUSION: With recent advances in surgical techniques, as well as improvements in preoperative and postoperative care, this procedure should continue to be applied in cases resistant to conventional therapy.

Adolescent↗
PubMed1979

A therapeutic approach to childhood pelvic rhabdomyosarcoma without pelvic exenteration.

A therapeutic approach using chemotherapy rather than radical surgery as the initial modality of treatment was employed in 13 children with pelvic rhabdomyosarcoma. One patient failed to respond to chemotherapy and required anterior pelvic exenteration. Eight patients are alive and free of disease 24 to 96 months after diagnosis. Limited surgery for minimal residual disease was used in two patients. Radiation therapy at lower than conventional doses was used in seven of eight patients. This experience supports a therapeutic approach to childhood pelvic rhabdomyosarcoma with an initial chemotherapy regimen, resulting in the use of surgical procedures less detrimental to pelvic organs without compromising survival.

Adolescent↗
PubMed2006

Orthotopic continent urinary diversion after radical cystectomy in pediatric patients with genitourinary rhabdomyosarcoma.

PURPOSE: We present our experience with orthotopic continent urinary diversion following radical cystectomy due to genitourinary rhabdomyosarcoma in children not responding to radiotherapy or chemotherapy. MATERIALS AND METHODS: Four children with persistent genitourinary rhabdomyosarcoma underwent radical cystectomy with reconstruction of an orthotopic ileal neobladder. RESULTS: Average followup was 50.7 months. One patient died at 12 months postoperatively due to pulmonary recurrence. All patients were able to void completely at regular intervals. Renal function was normal in all patients. Average reservoir capacity was 250 ml. Daytime and nighttime urinary continence was achieved in all patients. CONCLUSIONS: The orthotopic continent urinary diversion is technically feasible even in small children. It represents a safe long-term option and offers good quality of life after disfiguring surgery. In fact, patients are able to void spontaneously through the urethra.

Child↗
PubMed1994

The role of retroperitoneal lymphadenectomy in localized paratesticular rhabdomyosarcoma.

Between 1985 and 1990, 10 cases of embryonal paratesticular rhabdomyosarcoma were treated at our institution. Patient age ranged from 5 months to 16 years at presentation (mean 8.7 years). Chest and abdominal computerized tomography (CT) was performed on all patients. Lymphangiography was performed on 2 patients, and 6 underwent retroperitoneal lymphadenectomy, due to suspicious CT (2) and a positive lymphangiogram (1). All 6 patients had pathologically negative nodes, and they received vincristine, actinomycin D and cyclophosphamide for 8 to 13 months (mean 10.6 months). Four patients also received doxorubicin. The 10 patients are alive and the disease is in complete remission for a mean 6.02-year disease-free survival. These data support the hypothesis that retroperitoneal lymphadenectomy can be avoided for paratesticular rhabdomyosarcoma after radical inguinal orchiectomy when CT is negative for nodal involvement.

Adolescent↗
PubMed1993

Urogenital rhabdomyosarcoma in children: is a conservative surgical approach justified?

Between 1970 and 1990, 23 patients were treated for embryonal rhabdomyosarcoma of the bladder (8), prostate (7), uterus (4) or vagina (4). One girl underwent exenteration without chemotherapy. Of the patients 22 received chemotherapy as initial treatment. There was 1 treatment-related death. A total of 21 patients completed the first phase: 4 were treated with chemotherapy alone and 17 subsequently underwent an operation. Three boys underwent total cystoprostatectomy and local resection was performed in 14 patients. One boy was lost to followup. Mean followup was 11.2 +/- 6.3 years in 15 survivors. Complete remission was achieved in 19 patients, while 11 (53%) had relapse at an interval of 3 to 102 months. Of 13 patients with bladder and prostate tumors 8 had relapse and 7 survived. Of 8 patients with uterus and vagina tumors 3 had relapse and all survived. Six patients died of recurrent disease (overall mortality rate 32%). The interval from initial diagnosis until death was always less than 5 years. The mortality rate was greater in boys (5 of 12) than in girls (1 of 9). Preservation of pelvic organs in girls was 75%, while of the boys 44% retained the bladder. Since disease relapsed in patients in whom no tumor was found in the resection specimen after chemotherapy, we conclude that a conservative surgical approach combined with chemotherapy towards urogenital rhabdomyosarcoma is justified. Although late relapses do occur, they can usually be salvaged by a combination of chemotherapy, radiotherapy and surgery.

Adolescent↗
PubMed1989

Experimental chemotherapy and radiotherapy to paratesticular rhabdomyosarcoma.

Experimental chemotherapy and radiotherapy were tried in transplanted tumors derived from a paratesticular embryonal rhabdomyosarcoma. There was no significant difference on the therapeutic effect between a combination chemotherapy composed of vincristine, actinomycin D and cyclophosphamide, so-called "VAC" regimen, and a single therapy of radiation. However, morphologic analyses suggest that VAC is effective in embryonal rhabdomyosarcomas in which undifferentiated rhabdomyoblasts predominate, while radiotherapy is preferable for those containing variously differentiated rhabdomyoblasts.

Adolescent↗
PubMed1988

Embryonal rhabdomyosarcoma of bladder and prostate: nuclear DNA patterns studied by flow cytometry.

Nuclear deoxyribonucleic acid ploidy studies with paraffin-embedded archival tumor specimens were performed by flow cytometry on extracted nuclei from 13 embryonal rhabdomyosarcomas of the bladder and prostate. Preparation of embedded tissue specimens into single dissociated nuclei was by the Hedley technique and they were stained with propidium iodide according to the Vindeløv method. Before the era of chemotherapy, 6 of 7 patients died of disease at a median of 5.5 months post-treatment. All 6 deaths occurred in patients with deoxyribonucleic acid aneuploid patterns that were stage II or greater. The 1 survivor had a deoxyribonucleic acid aneuploid pattern and stage I disease and is alive at 12 years of followup. Since 1971, 6 patients were treated with primary polychemotherapy and surgery. All 6 patients are alive without evidence of disease at a mean followup of 75 months (range 12 to 180 months). All 6 patients had deoxyribonucleic acid aneuploid tumors. One patient was stage I and 5 patients were stage III. Thus, all patients with pediatric embryonal rhabdomyosarcoma of the bladder and prostate had deoxyribonucleic acid aneuploid tumors. These patients responded well to treatment with the combination of chemotherapy and surgery.

Aneuploidy↗
PubMed1986

Paratesticular rhabdomyosarcoma in children.

The clinicopathological features of 10 children with paratesticular rhabdomyosarcoma treated between 1965 and 1984 are reviewed. Of the patients 9 had embryonal rhabdomyosarcoma and 1 was pleomorphic. Median age was 4 years (range 2 to 11 years). Staging was based on clinical findings, chest x-ray, lymphoangiography, computerized tomography and histological studies. The disease was stage I in 5 patients, stage II in 2 and stage IV in 3. Treatment included radical orchiectomy in all patients, chemotherapy in 8 and lumboaortic radiotherapy in 5. No retroperitoneal node lymphadenectomy was performed. Of the 10 children 7 are free of disease after 2 to 19 years (median 7 years) of followup, including all of those with stages I and II disease.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1981

Embryonal rhabdomyosarcoma of the genitourinary organs.

We report on 14 patients with embryonal rhabdomyosarcoma of the genitourinary organs treated with combined modality therapy. Six patients with paratesticular lesions are free of tumor 7 to 72 months (mean 44) after diagnosis, while 6 of 8 with pelvic lesions are free of tumor 18 to 132 months (mean 61) following diagnosis. Two patients died of metastases. Over-all, 2-year survival free of relapse was obtained in 9 of 11 patients (81 per cent). Of 4 patients with pelvic lesions who were treated with primary radiation-chemotherapy without exenteration 3 were managed successfully but 1 required urinary diversion for vesical fibrosis. Current concepts in the management of embryonal rhabdomyosarcoma are discussed.

Adolescent↗
PubMed1977

Five-year survival after disseminated paratesticular rhabdomyosarcoma.

A 5-year survivor of disseminated metastatic rhabdomyosarcoma arising from the paratesticular tissues is reported. Each of the 6 patients with paratesticular rhabdomyosarcoma treated at this hospital has been free of disease from more than 2 months to more than 7 years. The unusually good prognosis associated with this site may be owing to early detection, predictable pathways of initial lymphatic dissemination and aggressive surgical treatment followed by radiotherapy and multiple agent chemotherapy.

Adolescent↗
PubMed1975

Embryonal rhabdomyosarcoma of the bladder and prostate in childhood.

Of 30 children treated for embryonal rhabdomyosarcoma the primary site of tumor was the bladder in 14 cases (9 boys and 5 girls) and the prostate in 16 cases. The mean age at diagnosis was 3 years in patients with bladder sarcoma and 6.5 years in patients with sarcoma of the prostate. Over-all survival rate has been 23 per cent--5 children with rhabdomyosarcoma of the bladder and 2 with rhadbomyosarcoma of the porstate are alive from 1 1/2 to 23 years postoperatively. Aggressive coordinated treatment with surgery, radiotherapy and cyclic combination chemotherapy is recommended.

Adolescent↗
PubMed1997

Rhabdomyosarcoma. Biology and treatment.

Rhabdomyosarcoma is the most common soft-tissue sarcoma of childhood. Recognition of specific genetic changes in the two most common subtypes of rhabdomyosarcoma has allowed better understanding of the pathogenesis of this disease. In addition, identification of prognostic factors and the use of risk-directed multimodal therapy have improved the outcome for these patients significantly, with cure rates approaching 70%.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1981

Primary pulmonary rhabdomyosarcoma: a case report and review of the literature.

Although several primary pulmonary neoplasms containing striated muscle fibers have in the past been described as rhabdomyoma or rhabdomyosarcoma, it was not until 1939 that McDonald and Heather described the first acceptable case of this neoplasm. Since then 13 such cases have been reported in the world literature. Previously reported cases of the neoplasm are reviewed and the salient features described. The origin of the striated muscle fibers has been much debated. Some believe that they develop from myoblastic differentiation of primitive mesenchymal cells or metaplastic transformation of pleuripotential mesenchymal cells present in the bronchial walls and pulmonary interstitium, whereas others claim that they develop from misplaced striated muscle tissue from the pharyngeal or esophageal region. A case report of the pleomorphic type of primary pulmonary rhabdomyosarcoma with multiple hematogenous metastases is presented as well as a review of the literature with a discussion of the histogenesis.

Aged↗