Intensive Multiagent Therapy, Including Dose-Compressed Cycles of Ifosfamide/Etoposide and Vincristine/Doxorubicin/Cyclophosphamide, Irinotecan, and Radiation, in Patients With High-Risk Rhabdomyosarcoma: A Report From the Children's Oncology Group Pediatric Oncology

Purpose Patients with metastatic rhabdomyosarcoma (RMS), except those younger than 10 years with embryonal RMS, have an estimated long-term event-free survival (EFS) of less than 20%. The main goal of this study was to improve outcome of patients with metastatic RMS by dose intensification with interval compression, use of the most active agents determined in phase II window studies, and use of irinotecan as a radiation sensitizer. Patients and Methods Patients with metastatic RMS received 54 weeks of therapy: blocks of therapy with vincristine/irinotecan (weeks 1 to 6, 20 to 25, and 47 to 52), interval compression with vincristine/doxorubicin/cyclophosphamide alternating with etoposide/ifosfamide (weeks 7 to 19 and 26 to 34), and vincristine/dactinomycin/cyclophosphamide (weeks 38 to 46). Radiation therapy occurred at weeks 20 to 25 (primary) but was also permitted at weeks 1 to 6 (for intracranial or paraspinal extension) and weeks 47 to 52 (for extensive metastatic sites). Results One hundred nine eligible patients were enrolled, with a median follow-up of surviving patients of 3.8 years (3-year EFS for all patients, 38% [95% CI, 29% to 48%]; survival, 56% [95% CI, 46% to 66%]). Patients with one or no Oberlin risk factor (age> 10 years or
Source: Journal of Clinical Oncology - Category: Cancer & Oncology Authors: Tags: Rhabdomyosarcoma & Soft Tissue Sarcoma, Chemotherapy, Rhabdo & Other Soft Tissue Sarcomas: Pediatric Oncology Source Type: research

Related Links:

Publication date: Available online 28 November 2019Source: Journal of Pediatric Surgery Case ReportsAuthor(s): Imad Aljabban, Christa GrantAbstractPediatric soft tissue sarcomas comprise a small proportion of pediatric solid malignancies and may arise in numerous anatomic locations such as the head and neck, trunk, extremities, and the genitourinary tract. Here, we present a case of rhabdomyosarcoma (RMS) arising from an unusual location, the urachus. The 3-year-old patient initially presented with signs of small bowel obstruction. On diagnostic laparoscopy, an infraumbilical mass was discovered and removed by laparotomy. ...
Source: Journal of Pediatric Surgery Case Reports - Category: Surgery Source Type: research
Conclusions: The proposed nomogram is a reliable and robust tool for accurate prognostic prediction in patients with extremity soft tissue LMS. Introduction Soft tissue leiomyosarcoma (LMS) is an aggressive sarcoma, which originates from smooth muscle cells (1). Soft tissue LMS accounts for about 5–10% of all soft tissue sarcomas (2). It occurs in different sites, including the retroperitoneum, intraabdominal sites, and extremities (3). Extremity LMS comprised about 10–15% of extremity sarcomas, with a preference for the lower limb (4–6). Extremity LMS tends to have a better prognosis than uterine...
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
Rhabdomyosarcoma (RMS) accounts for approximately half of all pediatric soft tissue sarcomas and 15% of all pediatric solid tumors. RMS is divided into 5 major histologic categories: embryonal, alveolar, botryoid embryonal, spindle cell embryonal, and anaplastic. Treatment is determined by the extent of disease, ease of resectability, and precise site. Superficially placed tumors can be managed either with excision and relatively non-toxic adjuvant chemotherapy or by biopsy. More deeply placed lesions generally are not removable and require radiation therapy and very aggressive chemotherapy.
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - Category: ENT & OMF Authors: Tags: CPP - Clinical Poster Presentation Source Type: research
AbstractRhabdomyosarcoma (RMS) is a highly malignant tumor which is thought to originate from the pluripotent mesenchyme. It is the most common soft-tissue sarcoma of childhood. This review article summarizes the recent and older published literature and gives an overview of management of RMS in children. RMS can arise in a wide variety of primary sites, some of which are associated with specific patterns of local invasion, regional lymph nodal spread, therapeutic response and long term outcome, hence requiring physicians to be familiar with site-specific staging and treatment details. Most common primary sites include the...
Source: Indian Journal of Pediatrics - Category: Pediatrics Source Type: research
Conclusion The treatment of pediatric genitourinary RMS has shifted from debilitating pelvic exenteration to a multimodal treatment approach involving surgery, chemotherapy, and radiation therapy in an effort to preserve genitourinary organs and reduce treatment morbidity. Continued research is required to improve post-treatment organ function. Further studies utilizing objective urodynamic evaluation are necessary to better characterize bladder function after treatment for RMS. Exciting recent developments in RMS research of fusion proteins that induce cell transformation and inhibit apoptosis and myogenic diffe...
Source: International Urology and Nephrology - Category: Urology & Nephrology Source Type: research
Authors: Hooper JE, Cantor EL, Ehlen MS, Banerjee A, Malempati S, Stenzel P, Woltjer RL, Gandour-Edwards R, Goodwin NC, Yang Y, Kaur P, Bult CJ, Airhart SD, Keller C Abstract Embryonal rhabdomyosarcoma (eRMS) is one of the most common soft tissue sarcomas in children and adolescents. Parameningeal eRMS is a variant that is often more difficult to treat than eRMS occurring at other sites. A 14-year-old female with persistent headaches and rapid weight loss was diagnosed with parameningeal eRMS. She progressed and died despite chemotherapy with vincristine, actinomycin-D, and cyclophosphamide plus 50.4 Gy radi...
Source: Sarcoma - Category: Cancer & Oncology Tags: Sarcoma Source Type: research
We present 3 cases of children, aged 6 to 14 years, presenting with auricular RMS. Case reports The first child, aged 6, was managed by 4 chemotherapy cycles followed by surgical resection of the tumor bed, completed by 5 further cycles of chemotherapy. The second, aged 14, was managed by 4 chemotherapy cycles followed by external radiation therapy of the tumor bed and lymph node areas, completed by 5 further cycles of chemotherapy. The third, aged 13, was managed by 4 chemotherapy cycles followed by surgery, completed by 5 further cycles of chemotherapy. Discussion In these 3 patients, the ...
Source: European Annals of Otorhinolaryngology, Head and Neck Diseases - Category: ENT & OMF Source Type: research
Rhabdomyosarcoma (RMS) represents the most common soft tissue sarcoma in infants and children and the third most common pediatric solid tumor, accounting for 5% to 15% of all childhood solid tumors. Of these, 15% to 20% arise from the genitourinary tract, with the most common sites originating from the prostate, bladder, and paratesticular regions, followed by the vagina and uterus. Although upfront radical surgery was used at the initiation of Intergroup RMS Study-I (1972–1978), the treatment paradigm has shifted to include initial biopsy with the goal of organ preservation, systemic chemotherapy for all patients, a...
Source: Urologic Oncology: Seminars and Original Investigations - Category: Urology & Nephrology Authors: Tags: Seminar article Source Type: research
Abstract Rhabdomyosarcoma (RMS) represents the most common soft tissue sarcoma in infants and children and the third most common pediatric solid tumor, accounting for 5% to 15% of all childhood solid tumors. Of these, 15% to 20% arise from the genitourinary tract, with the most common sites originating from the prostate, bladder, and paratesticular regions, followed by the vagina and uterus. Although upfront radical surgery was used at the initiation of Intergroup RMS Study-I (1972-1978), the treatment paradigm has shifted to include initial biopsy with the goal of organ preservation, systemic chemotherapy for all...
Source: Urologic Oncology - Category: Urology & Nephrology Authors: Tags: Urol Oncol Source Type: research
Conditions:   Adult Alveolar Soft Part Sarcoma;   Adult Angiosarcoma;   Adult Epithelioid Sarcoma;   Adult Extraskeletal Myxoid Chondrosarcoma;   Adult Extraskeletal Osteosarcoma;   Adult Fibrosarcoma;   Adult Leiomyosarcoma;   Adult Liposarcoma;   Adult Malignant Mesenchymoma;   Adult Synovial Sarcoma;   Adult Unclassified Pleomorphic Sarcoma;   Childhood Alveolar Soft Part Sarcoma;   Childhood Angiosarcoma;   Childhood&...
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
More News: Cancer & Oncology | Chemotherapy | Children | History of Medicine | Pediatrics | Radiation Therapy | Rhabdomyosarcoma | Sarcomas | Soft Tissue Sarcoma | Study | Toxicology