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PubMed1997

Pretreatment TNM staging of childhood rhabdomyosarcoma: a report of the Intergroup Rhabdomyosarcoma Study Group. Children's Cancer Study Group. Pediatric Oncology Group.

BACKGROUND: The Intergroup Rhabdomyosarcoma Study Group (IRSG) studies began in 1972 and initially used a clinicopathologic system to place patients into prognostic groups. Because of interest in the development of a pretreatment staging system for assessing the posttreatment outcomes of patients with this disease, potential staging elements were retrospectively evaluated in a subset of 505 patients who participated in IRS-II, an IRSG clinical trial. METHODS: Using the IRS-II data, a TNM pretreatment staging system was developed and used to stage prospectively the patients who were entering IRS-III, a subsequent treatment protocol of the IRSG. Failure free survival and overall survival were compared by pretreatment stage in IRS-III as a means of evaluating this TNM staging. RESULTS: The TNM staging system described the tumor (T) in terms of lesion size (< 5 cm or > or = 5 cm) instead of invasiveness, because these two features were not independent of each other. The clinical status of regional lymph nodes (N) was included in the staging system, as was the presence or absence of metastatic disease (M). The latter feature was extremely important, as expected. The anatomic site of the primary tumor also proved to be an important staging element. Classification of patients by tumor size, clinical status of regional lymph nodes, presence or absence of metastatic disease, and location of the primary tumor (at a favorable or unfavorable anatomic site) created four prognostically distinct staging categories that were relatively equal in size. In a prospective evaluation of this staging system with IRS-III patients, the pretreatment staging lost some prognostic impact. The survival of patients with smaller lesions at unfavorable anatomic sites without clinically involved lymph nodes (Stage II) was similar to that of patients with primary tumors at favorable anatomic sites (Stage I). CONCLUSIONS: A pretreatment TNM staging system for childhood rhabdomyosarcoma, developed with data from IRS-II, was not as predictive of patient outcome when applied prospectively to patients treated in the IRS-III trial. These findings could be due to differences in the management strategy used for IRS-III or the statistical variability in the model-fitting process used to develop the staging system. This demonstrates the need for continual reevaluation of staging systems as patient evaluation and treatment innovations are developed.

Child↗
PubMed1997

Clinical relevance of DNA ploidy and proliferative activity in childhood rhabdomyosarcoma: a retrospective analysis of patients enrolled onto the Italian Cooperative Rhabdomyosarcoma Study RMS88.

PURPOSE: Evaluation of the possible clinical relevance of DNA ploidy and proliferative activity assessed as S-phase fraction (SPF) in childhood rhabdomyosarcoma (RMS). PATIENTS AND METHODS: We conducted a retrospective study on 59 RMS patients enrolled onto the ICS-RMS88 protocol (seven botryoid, 35 embryonal, and 17 alveolar RMS), for which formalin-fixed paraffin-embedded (FFPE) tissue was available. Nuclear suspensions for cytometric investigation were obtained using a mechanical disaggregation. Tumors were distinguished according to their DNA index (DI) value as follows: diploid (0.9 < DI < 1.1), hyperdiploid (1.1 < or = DI < 1.8 or DI > or = 2.2), and tetraploid (1.8 < or = DI < 2.2); for analysis of SPF, a cutoff value of 14% was used. RESULTS: DNA histograms were diploid in 19 (33%) cases, hyperdiploid in 29 (49%), and tetraploid in 10 (32%). One patient showed both a hyperdiploid and a tetraploid peak. The 5-year overall survival (OS) rate by ploidy status was 73% in hyperdiploid patients as compared with 33% and 25% in diploid and tetraploid patients, respectively (P = .0012). A striking difference emerged when the 5-year OS for the combined diploid and tetraploid RMS groups was compared with survival of the hyperdiploid RMS group: 30% versus 73%, respectively (P = .0006). In addition, the SPF was prognostically relevant: 5-year OS by SPF less than or greater than 14% was 70% and 36%, respectively (P = .009). Multivariate analysis confirmed the importance of DNA content (P = .0006) and SPF (P = .034) in predicting survival. CONCLUSION: These findings confirm that ploidy and SPF are important new prognostic factors that are able to identify selected groups of patients at high risk of treatment failure, even if the tumor's presentation is favorable according to standard criteria.

Adolescent↗
PubMed2001

Up-front window trial of topotecan in previously untreated children and adolescents with metastatic rhabdomyosarcoma: an intergroup rhabdomyosarcoma study.

PURPOSE: To investigate the antitumor activity and toxicity of topotecan, used alone and in combination with conventional therapy, in patients with metastatic rhabdomyosarcoma (RMS). PATIENTS AND METHODS: Forty-eight patients younger than 21 years of age with newly diagnosed metastatic RMS received 2.0 to 2.4 mg/m(2) of topotecan intravenously daily for 5 days every 21 days before standard therapy. Two courses were given in the absence of progressive disease or excessive toxicity and response was assessed. Patients with at least a partial response (PR) to topotecan proceeded to therapy with alternating courses of vincristine 1.5 mg/m(2), dactinomycin 1.5 mg/m(2), and cyclophosphamide 2.2 g/m(2) (VAC) and vincristine 1.5 mg/m(2), topotecan 0.75 mg/m(2) daily x 5, and cyclophosphamide 250 mg/m(2) daily x 5. Patients who did not respond to topotecan received continuation therapy with VAC alone. RESULTS: The overall response rate to topotecan was 46% (complete response, 4%; partial response 42%). Unexpectedly, patients with alveolar RMS had a higher rate of response (65%) than those with embryonal RMS (28%; P: = .08). The most common grade 3 or 4 toxicities were neutropenia (67%), anemia (33%), thrombocytopenia (25%), and infection (21%). Two-year failure-free survival and survival estimates were 24% and 46%, respectively. Response to window therapy did not correlate with survival. CONCLUSION: The high response rate and acceptable toxicity profile of topotecan in children with advanced RMS support further evaluation of this agent in phase III trials. The superior responses in alveolar RMS are of interest.

Adolescent↗
PubMed1993

Spindle cell variants of embryonal rhabdomyosarcoma in the paratesticular region. A report of the Intergroup Rhabdomyosarcoma Study.

We reviewed 173 cases of paratesticular rhabdomyosarcoma (RMS) of Intergroup Rhabdomyosarcoma Studies (IRS)-I, -II, and -III for evaluation of possible histological factors that might account for the good prognosis of these patients. Almost all cases (161 of 173 cases, 93.1%) occurring in this site were of embryonal histology. A spindle-cell subtype of embryonal RMS was identified that presented a storiform growth pattern with abundant collagen between the tumor cells in most cases. Other tumors of this subtype showed an arrangement of tumor cells in bundles with a low to moderate amount of collagen, resembling a leiomyosarcoma. The other embryonal RMS in this site had the classical embryonal cytology. The spindle-cell subtype was highly differentiated by immunohistochemistry and electron microscopy. Lymph node metastasis was found in seven of 43 patients (16.3%) with a RMS of spindle-cell subtype, compared with 40 of 112 patients (35.7%) with RMS of non-spindle-cell type. Clinical data from patients with spindle-cell subtypes of the paratesticular lesions revealed that they almost always had an association with clinical groups of limited disease (32 patients, 74.4%, with Group I; 10 patients, 23.3%, with Group II disease) and a significantly better prognosis (95.5% survival at 5 years) when compared with patients with the classic embryonal variant of RMS (80% survival at 5 years, p < 0.035). The incidence and anatomic distribution of this spindle cell subtype of embryonal RMS was estimated on 800 randomly selected patients from IRS-II. It was found in the head and neck, extremities, orbit, and some other sites, but 30.6% were located in the paratesticular area. Patients with spindle cell RMS of nonparatesticular sites usually had more extensive disease compared with patients having paratesticular lesions; two thirds of the cases had gross residual tumor after surgery or metastatic tumor at diagnosis. We conclude that spindle-cell RMS is a subtype of embryonal RMS with a very favorable prognosis. The site factor of the paratesticular localization may allow earlier diagnosis of the spindle-cell lesions compared with other sites. Other unknown factors may also play a role.

Child↗
PubMed1978

Incidence of meningeal involvement by rhabdomyosarcoma of the head and neck in children: a report of the Intergroup Rhabdomyosarcoma Study (IRS).

141 patients with embryonal rhabdomyosarcoma (RMS) of the head and neck are reviewed. 57/141 had lesions of para-meningeal sites. 20/57 (35%) developed evidence of direct meningeal extension. 18/20 (90%) died of this complication. Radiation portals and doses were limited in 42% and 32%, respectively. All patients had chemotherapy for 6 weeks prior to radiation. The significance of the adequacy of radiation factors and the timing of chemotherapy are reviewed. Recommendations for managing these patients include earlier use of radiation and increased coverage of adjacent meninges by radiation including total craniospinal axis radiation when brain meningeal involvement exists.

Child↗
PubMed1980

Radiation to regional nodes for rhabdomyosarcoma of the genitourinary tract in children: is it necessary? A report from the Intergroup Rhabdomyosarcoma Study No. 1 (IRS-1).

Fifty-eight children with genitourinary rhabdomyosarcoma are reported. Lesions involved the bladder (22), prostate (14), vagina/uterus (6), and paratesticular tissues (16). Fifteen of 58 had positive sampling of regional lymph nodes. Eleven of 15 received radiation to no more than 4500 rad in most cases, and 9 of 11 are diseases free. Two of 15 had no radiation and are disease free also. Twenty-three of 58 had negative nodes. Six of 23 had radiation to these nodal areas and 4 of 6 are disease free. Fifteen of 17 patients had no radiation and are disease free also. Sixteen of 20 patients with no node sampling are disease free; 9 of 16 had radiation but 7 of 16 did not. All patients in the Study had intensive systemic maintenance chemotherapy. One 1 patient failed in regional nodes despite 4500 rad to these node echelons. We suggest chemotherapy and radiotherapy, not to exceed 3500 rad in four weeks, to known residual disease including regions from which positive nodes have been obtained.

Child↗
PubMed1984

Bone marrow metastases at diagnosis in children and adolescents with rhabdomyosarcoma. A report from the intergroup rhabdomyosarcoma study.

Thirty children and adolescents with rhabdomyosarcoma (RMS) entered on IRS-1 in a 5-year period had bone marrow metastases (BMM) at diagnosis. BMM at diagnosis occurred in 30/103 patients (29%) with Clinical Group IV RMS. On the average, patients with BMM were older with more extremity primaries than other IRS-1 patients. Alveolar histology was present in 15/30 patients (50%) with BMM, compared to 19% on IRS-1. Eleven patients received vincristine, dactinomycin, cyclophosphamide (VAC), while 19 patients received VAC with Adriamycin (ADR). The overall complete response (CR) rate was 60%. The median disease-free survival times by treatment were 17 weeks with VAC and 32 weeks for VAC + ADR (P greater than 0.10). Relapse from CR in Clinical Group IV patients with BMM occurred in 16/18 patients (89%) and was significantly higher than in 17/29 Clinical Group IV patients (59%) without BMM (P less than 0.035). BMM at diagnosis in children and adolescents with RMS is frequently associated with alveolar histology and is a poor prognostic sign.

Adolescent↗
PubMed2001

Congenital alveolar rhabdomyosarcoma: clinical and molecular distinction from alveolar rhabdomyosarcoma in older children.

BACKGROUND: Congenital alveolar rhabdomyosarcoma (RMS) is extremely rare and invariably fatal with current therapy. Its clinical presentation is unusual and is distinct from RMS in older children, with > 50% of patients presenting with multiple cutaneous metastases. To the authors' knowledge, the biology of this condition has not been studied to date. METHODS: The clinical and pathologic findings of four cases of congenital RMS are presented along with those of other cases found in the literature. Molecular analysis was undertaken to detect the characteristic chromosomal translocations of alveolar RMS in three cases with suitable material. RESULTS: To the authors' knowledge, there are no recorded survivors of congenital alveolar RMS. Clinical and histopathologic findings in four patients with congenital alveolar RMS are detailed along with treatment responses. It now is recognized that tumor specific translocations can be detected in the majority of cases of alveolar RMS using the reverse transcriptase-polymerase chain reaction (RT-PCR) method. However, detailed molecular analysis using RT-PCR was unable to detect the presence of either the classic t(2;13) or variant t(1;13) translocation in three cases analyzed at the molecular level. CONCLUSIONS: To the authors' knowledge the optimal treatment for this rare tumor is not known. The longest survivor in the current series underwent myeloablative therapy with peripheral stem cell support, suggesting that more intensive treatment may be of value in this rare condition. None of the three tumors analyzed by RT-PCR harbored characteristic RMS translocations. The molecular biology of this highly malignant and aggressive congenital tumor appears to be different from alveolar RMS in older children and warrants further investigation.

Female↗
PubMed2001

Efficacy of ifosfamide and doxorubicin given as a phase II "window" in children with newly diagnosed metastatic rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study Group.

BACKGROUND: The cure rate for children/adolescents with localized rhabdomyosarcoma (RMS) has tripled over the past 25 years, but patients with metastatic disease at presentation have not benefited similarly, and urgently need new therapy. We evaluated a new drug pair, ifosfamide + doxorubicin, for such patients. PROCEDURE: We estimated the complete and partial response rates (i.e., CR and PR) of 152 previously untreated children/adolescents with metastatic RMS entered on the IRS-IV pilot from July 1988 to October 1991 who received an "up-front window" of ifosfamide (1.8 gm/m(2)/day for 5 days) and doxorubicin (30 mg/m(2)/day for 2 days) given every 3 weeks for 12 weeks. This was followed by combination chemotherapy with vincristine, actinomycin D, and cyclophosphamide (VAC), given every 3 weeks for an additional 36 weeks. RESULTS: Of 115 patients evaluable for early response at 12 weeks, 28 (20%) had CR and 66 (43%) had PR. The ultimate CR rate was 52%. Overall, about one-third of patients survived. Prognostic factor analysis revealed that patients < 10 years old (P < 0.001), those with embryonal tumors (P = 0.002), or a GU primary site (P = 0.010), and those who lacked nodal disease (P = 0.041), and those who lacked bone or bone marrow metastasis (P < 0.001) fared better than did others. CONCLUSIONS: The 63% CR + PR rate achieved at 12 weeks and overall 5-year FFS seen with this drug pair is similar to that achieved with previously evaluated drug combinations. We conclude that ifosfamide/doxorubicin is highly active in advanced RMS, and should be considered for inclusion in frontline therapy for children with intermediate or high-risk RMS.

Adolescent↗
PubMedDate not supplied

Bladder/prostate rhabdomyosarcoma: results of the multi-institutional trials of the Intergroup Rhabdomyosarcoma Study.

Rhabdomyosarcomas (RMS) occurring in pelvic sites are almost entirely of the embryonal histologic subtype and are relatively responsive to chemotherapeutic agents. When treated by radical surgery, pelvic irradiation, and intensive chemotherapy for 2 years, survival rates of higher than 85% have been achieved. However, the effects of such therapeutic approaches have resulted in major changes in the quality of life of survivors, because of the loss of pelvic organs, primarily the bladder. Current efforts are directed towards achieving long range survival with procedures that are less extensive than pelvic exenteration or total cystectomy and by regimens that eliminate or reduce the intensity of radiotherapy. Until the past 5 years, these efforts have been relatively unsuccessful in both North America and Europe. However, the most recent studies of the IRS and European cooperative groups suggest that a majority of these patients can survive with intact pelvic organs when treated with currently available chemotherapeutic agents in conjunction with radiotherapy and limited surgery.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1990

Partial cystectomy in the management of rhabdomyosarcoma of the bladder: a report from the Intergroup Rhabdomyosarcoma Study.

Among 154 children with primary vesical rhabdomyosarcoma entered in the IRS (1972 to 1986), tumor excision constituting a partial cystectomy was performed in 33. These procedures were performed in the following situations (1) as an initial operation for localized disease (22); (2) as a secondary procedure for localized disease following chemotherapy (CT) or CT/irradiation response (6); and (3) as an initial operation in the presence of disseminated abdominal disease (5). Partial cystectomy included full-thickness resection of 15% to 80% of the bladder wall. Ureters were reimplanted or revised in two patients. The 33 patients undergoing partial cystectomy were in the following clinical groups: I, total excision (10); II, gross excision with "microscopic residual" or positive nodes (8); III, subtotal excision (10); and IV, tumor dissemination (5). Patients in clinical groups I and II received vincristine and actinomycin-D (VA) or VA + cyclophosphamide (VAC) +/- adriamycin (ADR). Patients in groups III and IV received the same agents +/- cisplatin. Irradiation (2,000 to 5,000 cGy) was administered to 18 patients (55%) in groups II to IV. Six relapses occurred among 28 patients with localized disease, and one among five patients with dissemination; all resulting in progressive disease and/or death. Estimated 3-year survival following partial cystectomy (79%) is similar to that for all patients with primary bladder tumors (78%). In contrast with the total group, in which the majority of survivors have eventually required total cystectomy; 25 of 26 survivors of partial cystectomy have functional bladders from 146 to 686 weeks (median, 356 weeks) from study entry.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Spindle cell (leiomyomatous) rhabdomyosarcoma, a rare variant of embryonal rhabdomyosarcoma.

A rare case of the leiomyomatous variant of embryonal rhabdomyosarcoma is reported. A 13-year-old boy presented with a recurrent painless mass on the ventral side of the right forefoot. Microscopically, the tumor consisted of spindle-shaped and round tumor cells in a fascicular or storiform, focally hemangiopericytoma-like growth pattern. The cytoplasm of the spindle-shaped tumor cells was eosinophilic and fibrillary, in some areas resembling smooth muscle cells. Immunohistologically, all tumor cells were vimentin-positive, most of them also stained with antibodies to desmin and muscle specific actin (MSA). In addition, many tumor cells showed a co-expression of alpha-sarcomeric actin and myoglobin. All tumor cells were negative with alpha-smooth muscle actin. Two years after surgical treatment and chemotherapy the patient is well with no evidence of distant metastases. The clinicopathological features and differential diagnostic problems are discussed.

Adolescent↗
PubMed1997

Radiation therapy for rhabdomyosarcoma: local failure risk for Clinical Group III patients on Intergroup Rhabdomyosarcoma Study II.

PURPOSE: A subset of 362 pediatric patients with rhabdomyosarcoma was selected from a total of 532 eligible IRS-II patients in Clinical Group III to assess the local and regional failure rates following radiotherapy and to determine patient, tumor, and treatment factors contributing to the risk for local and regional failure. METHODS AND MATERIALS: The study population was selected from all eligible IRS-II Clinical Group III patients. Excluded patients were those with "special pelvic" primary sites whose protocol management restricted radiotherapy (n = 123), and those who were removed from the study before radiotherapy was to begin, or because it was omitted (n = 47). A binary recursive partitioning model was used to identify subgroups of the remaining 362 patients at risk of local or regional failure. RESULTS: The local (only) failure rate was 17% (95% confidence interval, 13-21%), and the local (all) failure rate was 20% (95% confidence interval, 16-24%). The 5-year actuarial risk of local (all) failure was 22% (95% confidence interval, 18-27%). The risk of regional (nodal) failure was between 2% and 23%. Increasing tumor size predicted an increased local failure risk. Primary tumors located above the clavicle had a reduced risk of local failure. The binary recursive partitioning model identified a subset of patients at high risk of local failure. Those patients had primary tumors in the chest, pelvic region, extremity, or trunk, or tumors > 10 cm in diameter. Their local failure rate was 35% (compared to 15% for the remaining patients). The subset of patients at high risk for regional (nodal) failure had node involvement at diagnosis and a primary tumor originating at a site other than orbit, parameningeal, or trunk. Compliance with radiation treatment guidelines approached but did not achieve statistical significance as a predictive factor for local failure. By univariate analysis, factors not influencing local failure risk were age, race, gender, adenopathy, and histology. CONCLUSION: Radiation therapy and chemotherapy administered to Clinical Group III patients entered into the IRS-II protocol produced sustained local control in most cases. Knowledge of the factors which predict an increased risk of local or regional failure will facilitate the design of new treatment strategies.

Adult↗
PubMed1989

Immunohistochemical spectrum of rhabdomyosarcoma and rhabdomyosarcoma-like tumors. Expression of cytokeratin and the 68-kD neurofilament protein.

Twenty-five rhabdomyosarcomas (RMSs), including 12 alveolar and 13 embryonal types, were immunohistochemically studied for the presence of different classes of intermediate filament proteins and muscle actins (MAs). For the most part, formaldehyde-fixed and paraffin-embedded tissue was used in immunostaining. All RMSs showed desmin and MAs, usually in a major portion of tumor cells. The number of MA-positive cells was sometimes higher than that of desmin-positive cells. Vimentin was present in all tumors studied in frozen sections. Eight of 12 alveolar RMSs showed small number of cytokeratin-positive neoplastic cells. Cytokeratin-positive cells were present less commonly in embryonal RMS (3/13 cases). The 68-kD neurofilament protein was found in frozen sections of two embryonal RMSs. The cytokeratin and neurofilament immunostaining could be reproduced by immunofluorescence technique. In addition, we studied three childhood sarcomas, which showed abundant desmin and MA immunostaining but did not conform to the ultrastructural criteria of RMS. Scattered cytokeratin-positive cells were found in two of these tumors, and neurofilaments were found in the two cases for which frozen sections were available. The results show that typical RMS may demonstrate immunohistological pleomorphism with cytokeratin and neurofilament immunoreactivity suggesting the presence of multidirectional differentiation. In addition, there are tumors that by morphology look like RMS and have muscle cell markers but cannot be verified as RMS by electron microscopy; also, these tumors seem to show immunohistological pleomorphism. The presence of nonmyoid markers in RMS should be considered when making immunohistological diagnosis of soft tissue sarcomas.

Adolescent↗
PubMed1987

Pathology of fatal rhabdomyosarcoma. Report from Intergroup Rhabdomyosarcoma Study (IRS-I and IRS-II).

Patterns of tumor spread were studied by examining clinicopathologic and postmortem data on 274 tumor-related deaths from patients in IRS-I and IRS-II. The activity of the primary lesion and presence or absence of distant metastases at death were related to the histologic subtype and anatomic site of origin, individually and/or in combination. The alveolar subtype had the highest proportion of distant metastases and the lowest occurrence of local progression alone from the primary site. More distant metastases were observed among the patients with extremity primary, and the frequency increased significantly with the alveolar histologic subtype. Parameningeal tumors, irrespective of their histologic subtypes, showed a high incidence of direct extension from the primary lesion especially into the CNS. The lung was the most common site of metastasis at death, followed by the regional lymph node, bone, liver, and brain. Regional node involvement was observed more commonly in patients with lower extremity primary and alveolar subtype.

Autopsy↗