We have chosen to translate what we believe to be the first publication of a well-documented case of a young patient with embryonal rhabdomyosarcoma. The author, M. Léon Bérard, was a hospital fellow working in the department of M.
View Article and Find Full Text PDFBackground: Distinguishing alveolar rhabdomyosarcoma (ARMS) from embryonal rhabdomyosarcoma (ERMS) is of prognostic and therapeutic importance. Criteria for classifying these entities evolved significantly from 1995 to 2013. ARMS is associated with inferior outcome; therefore, patients with alveolar histology have generally been excluded from low-risk therapy.
View Article and Find Full Text PDFBackground: Desmoid fibromatosis (desmoid tumor, DT) is a soft tissue neoplasm prone to recurrence despite complete surgical resection. Numerous small retrospective reports suggest that non-cytotoxic chemotherapy using tamoxifen and sulindac may be effective for DT. We evaluated the safety and efficacy of tamoxifen and sulindac in a prospective phase II study within the Children's Oncology Group.
View Article and Find Full Text PDFWe believe that this is the first translation into English of the first description, in French, of a disease previously unknown. JL Riopelle and JP Thériault, both pathologists, reviewed clinical and pathologic findings in six young patients with soft tissue tumors, and contributed autopsy information on four of the patients. Only one patient was initially correctly diagnosed with rhabdomyosarcoma; the other five initially had alternative diagnoses.
View Article and Find Full Text PDFBackground: We wanted to ascertain patterns of recurrence, re-treatment, and outcome among 188 eligible patients treated for localized orbital sarcoma on IRSG Protocols III/IV, 1984-1997.
Procedure: Retrospective chart review.
Results: Twenty-four of 188 patients (12.
Purpose: To analyze the effect of reduced-dose radiotherapy on local control in children with low-risk rhabdomyosarcoma (RMS) treated in the Children's Oncology Group D9602 study.
Methods And Materials: Patients with low-risk RMS were nonrandomly assigned to receive radiotherapy doses dependent on the completeness of surgical resection of the primary tumor (clinical group) and the presence of involved regional lymph nodes. After resection, most patients with microscopic residual and uninvolved nodes received 36 Gy, those with involved nodes received 41.
Sarcomas of the head and neck region are a rare group of tumors in children and present challenges with regard to evaluation and treatment. Rhabdomyosarcomas are the most common sarcomas of the head and neck in children. Presence of metastases and complete surgical resectability continue to be the most relevant clinical prognostic factors in patients with sarcomas.
View Article and Find Full Text PDFPurpose: Patients with localized, grossly resected, or gross residual (orbital only) embryonal rhabdomyosarcoma (ERMS) had 5-year failure-free survival (FFS) rates of 83% and overall survival rates of 95% on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III/IV. IRSG D9602 protocol (1997 to 2004) objectives were to decrease toxicity in similar patients by reducing radiotherapy (RT) doses and eliminating cyclophosphamide for the lowest-risk patients.
Patients And Methods: Subgroup A patients (lowest risk, with ERMS, stage 1 group I/IIA, stage 1 group III orbit, stage 2 group I) received vincristine plus dactinomycin (VA).
Background: The local control approach for girls with non-resected vaginal rhabdomyosarcoma (RMS) enrolled onto Intergroup RMS Study Group (IRSG)/Children's Oncology Group (COG) studies has differed from that used at other primary sites by delaying or eliminating radiotherapy (RT) based on response achieved with chemotherapy and delayed primary resection.
Procedures: We reviewed locoregional treatment and outcome for patients with localized RMS of the vagina on the two most recent COG low-risk RMS studies.
Results: Forty-one patients with localized vaginal RMS were enrolled: 25 onto D9602 and 16 onto Subset 2 of ARST0331.
Purposes: The aims of the study were to compare results of clinical/radiographic studies before second-look procedures (SLP) with SLP specimens from patients with gross residual sarcoma at diagnosis and to relate tumor viability to outcome.
Patients: Seventy-three patients underwent SLP before completing chemotherapy, with (n = 59) or without (n = 14) radiotherapy. Tumor sites were bladder/prostate (n = 27), head/orbit/parameningeal (n = 22), extremity/trunk (n = 14), and retroperitoneum/pelvis (n = 10).
Purpose: To assess local control, event-free survival (EFS), and overall survival (OS) rates in 71 patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma (ALV RMS) and their relation to radiation therapy (RT) on IRSG Protocols III and IV, 1984-1997.
Methods: Chart review and standard statistical procedures. PATIENTS AND TUMORS: Patients were 1-18 years at diagnosis (median, 6 years).
We reviewed clinicopathologic features and treatment outcomes in seven patients diagnosed with Stage 4/Group IV orbital sarcoma and treated on IRSG protocols I-III. Three patients had embryonal rhabdomyosarcoma (RMS), and two patients each had alveolar RMS or unclassified sarcoma. Median age at diagnosis was 1.
View Article and Find Full Text PDFPurpose: To assess the rate of spinal cord toxicity in adolescents resulting from chemoradiotherapy of parameningeal sarcoma.
Methods And Materials: Of 152 patients with parameningeal sarcoma treated per the Intergroup Rhabdomyosarcoma Study Group protocol from 1977 through 1989, eight developed paralyzing ascending myelitis after intrathecal chemotherapy with cytosine arabinoside, methotrexate, and hydrocortisone administered during and after radiation therapy to volumes that included part of the spinal cord. The eight cases include three not previously published.
Purpose: To describe clinical and pathologic characteristics and outcome of patients with renal sarcomas.
Patients/methods: The IRSG database includes newly diagnosed patients <21 years old with rhabdomyosarcoma (RMS) or undifferentiated sarcoma (UDS). We identified patients with renal sarcoma and reviewed their charts.
Purpose: Determine outcome of patients with cranial parameningeal sarcoma and concurrent metastases treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV.
Patients: We identified 91 patients in the database, which includes newly diagnosed subjects <21 years old with rhabdomyosarcoma (RMS) and undifferentiated sarcoma, and reviewed their charts in detail.
Results: The 54 males and 37 females were <1-19 years at diagnosis.
Purpose: We analyzed the outcome of 47 patients with superficial facial rhabdomyosarcoma (RMS) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols-III, -IV-Pilot, and -IV.
Methods: We reviewed patients' records. Clinico-pathologic features, treatment, and outcome were examined to identify prognostic factors.
Purpose: We determined the late sequelae in children and adolescents with rhabdomyosarcoma of the bladder/prostate treated in the United States, Canada and selected Western European countries, primarily France, Germany, Italy and the United Kingdom, from 1979 to 1998.
Materials And Methods: We used a data collection form to record data from patient records available at the group statistical centers.
Results: A total of 164 patient charts had sufficient data available to be included in the study.
J Pediatr Hematol Oncol
October 2006
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.
View Article and Find Full Text PDFBackground/purpose: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Patients with localized disease have a cure rate of 50% to 90%; however, there has been little evidence that aggressive surgical resection for recurrent disease is of benefit. We reviewed our experience with aggressive surgical resection for recurrent RMS.
View Article and Find Full Text PDFBackground: This study was conducted to evaluate the incidence of adverse medical conditions and to assess the risk of developing these conditions in a cohort of long-term survivors of rhabdomyosarcoma (RMS) diagnosed before age 21.
Procedure: Using data from the Childhood Cancer Survivor Study (CCSS), we evaluated the incidence of self-reported adverse medical conditions for 606 RMS survivors and 3,701 siblings of cancer survivors. Cancer and treatment data abstracted from medical records were used to evaluate the effects of primary tumor site and combined modality therapy on the risk of developing sequelae in survivors.
Purpose: To evaluate the impact of radiation treatment parameters on cancer control outcomes for children with parameningeal rhabdomyosarcoma (PM-RMS) treated on Intergroup Rhabdomyosarcoma Study Group protocols II through IV (including IRS-IV pilot).
Materials And Methods: Radiation therapy (RT) treatment quality was assessed by contemporary review of portal radiographs, simulation films, treatment plans, and, in most cases, cross-sectional diagnostic imaging data for patients treated on Intergroup Rhabdomyosarcoma Study Group protocols II through IV. Five hundred ninety-five patients with PM-RMS were registered on these 4 studies between 1978 and 1997.
Background: We reviewed 56 IRS-IV patients with localized rhabdomyosarcoma [RMS] of the retroperitoneum/pelvis to assess outcome and prognostic factors, including the value of initially excising >or=50% of the tumor (debulking) before chemotherapy.
Methods: Patients had embryonal RMS [N=38], alveolar RMS [N = 7], RMS not otherwise specified [NOS, N = 7], or undifferentiated sarcoma [N = 4]. Fifteen patients were debulked; 41 patients were biopsied.
We identified all children enrolled in the Intergroup Rhabdomyosarcoma Study Group-IV with neurofibromatosis type I (NF1) and rhabdomyosarcoma. Among 1025 eligible patients, 5 (0.5 %) had NF1.
View Article and Find Full Text PDFPurpose: We determine patient and tumor characteristics, event-free and overall survival, methods of local control, rate of bladder preservation and proportion with normal bladder function for patients with localized bladder/prostate (BP) rhabdomyosarcoma (RMS) treated on the Fourth Intergroup Rhabdomyosarcoma Study (IRS IV).
Materials And Methods: We reviewed the records of 90 patients with nonmetastatic BP RMS enrolled on IRS IV for presenting characteristics, details of therapy and outcome.
Results: Of the 90 records 88 had sufficient information for review.