Joerg Fuchs1, Tobias M Dantonello, Gunnar Blumenstock, Daniel Kosztyla, Thomas Klingebiel, Ivo Leuschner, Andreas Schuck, Felix K Niggli, Ewa Koscielniak, Guido Seitz. 1. *Department of Pediatric Surgery and Pediatric Urology, University Children's Hospital, Tuebingen, Germany †Department of Pediatric Hematology, Oncology and Immunology, Olgahospital, Stuttgart, Germany ‡Department of Clinical Epidemiology and Applied Biometry, University of Tuebingen, Tuebingen, Germany §Department for Pediatric Hematology and Oncology, University Hospital, Frankfurt/Main, Germany ¶Department of Paidopathology, University Hospital, Kiel, Germany ‖Department of Radiotherapy, University Hospital, Muenster, Germany **Department of Oncology, University Children's Hospital, Zurich, Switzerland.
Abstract
OBJECTIVE: To analyze the clinical course, treatment, complications, outcome, and quality of life (QOL) in patients with perineal/perianal rhabdomyosarcoma (PRMS) treated within the CWS-86, -91, -96, and -2002P trials. BACKGROUND: Although multiple international study trials exist for the treatment of rhabdomyosarcoma, only very limited information is given on treatment, outcome, and QOL in PRMS. METHODS: A total of 35 patients suffering from PRMS were treated with neoadjuvant chemotherapy. Local therapy with radiation and/or surgery was performed, followed by adjuvant chemotherapy. Functional long-term follow-up was evaluated by a gastrointestinal/QOL survey. RESULTS: Thirty-two patients were evaluated (exclusion n = 3). Eight patients had embryonal histology, and 24 patients had alveolar histology. The median age was 108 months (median follow-up: 5.8 years). The 5-year overall survival was 47% (95% confidence interval: 29-64). Sixteen IRS (Intergroup Rhabdomyosarcoma Study) III and IV patients had locoregional lymph node involvement at diagnosis. Seven patients were treated with chemotherapy/surgery alone [5-year event-free survival (EFS): 85.7%]. Eleven patients received only radiochemotherapy (5-year EFS: 27.3%). Combined radiochemotherapy/surgery was used in 12 patients (5-year EFS: 63.6%). Two patients were treated only with chemotherapy and they died. Patients with embryonal histology had a significantly better 5-year EFS (87.5%) than patients with alveolar histology (39.1%; P = 0.013). Some patients reported symptoms of fecal incontinence. The median Wexner fecal incontinence score was 9 (possible range: 0-20), and the median QOL score was 90.5 (applicable range: 0-144). CONCLUSIONS: The outcome of these patients remains unsatisfactory. Prognostic factors for a favorable outcome are tumor size of smaller than 5 cm, negative locoregional lymph nodes, age less than 10 years, low IRS group, and embryonal histology. Fecal incontinence seems to be a problem.
RCT Entities:
OBJECTIVE: To analyze the clinical course, treatment, complications, outcome, and quality of life (QOL) in patients with perineal/perianal rhabdomyosarcoma (PRMS) treated within the CWS-86, -91, -96, and -2002P trials. BACKGROUND: Although multiple international study trials exist for the treatment of rhabdomyosarcoma, only very limited information is given on treatment, outcome, and QOL in PRMS. METHODS: A total of 35 patients suffering from PRMS were treated with neoadjuvant chemotherapy. Local therapy with radiation and/or surgery was performed, followed by adjuvant chemotherapy. Functional long-term follow-up was evaluated by a gastrointestinal/QOL survey. RESULTS: Thirty-two patients were evaluated (exclusion n = 3). Eight patients had embryonal histology, and 24 patients had alveolar histology. The median age was 108 months (median follow-up: 5.8 years). The 5-year overall survival was 47% (95% confidence interval: 29-64). Sixteen IRS (Intergroup Rhabdomyosarcoma Study) III and IV patients had locoregional lymph node involvement at diagnosis. Seven patients were treated with chemotherapy/surgery alone [5-year event-free survival (EFS): 85.7%]. Eleven patients received only radiochemotherapy (5-year EFS: 27.3%). Combined radiochemotherapy/surgery was used in 12 patients (5-year EFS: 63.6%). Two patients were treated only with chemotherapy and they died. Patients with embryonal histology had a significantly better 5-year EFS (87.5%) than patients with alveolar histology (39.1%; P = 0.013). Some patients reported symptoms of fecal incontinence. The median Wexner fecal incontinence score was 9 (possible range: 0-20), and the median QOL score was 90.5 (applicable range: 0-144). CONCLUSIONS: The outcome of these patients remains unsatisfactory. Prognostic factors for a favorable outcome are tumor size of smaller than 5 cm, negative locoregional lymph nodes, age less than 10 years, low IRS group, and embryonal histology. Fecal incontinence seems to be a problem.
Authors: Jörg Fuchs; Cristian Urla; Monika Sparber-Sauer; Andreas Schuck; Ivo Leuschner; Thomas Klingebiel; Gunnar Blumenstock; Guido Seitz; Ewa Koscielniak Journal: J Cancer Res Clin Oncol Date: 2018-02-20 Impact factor: 4.553
Authors: Abha A Gupta; Yueh-Yun Chi; James R Anderson; Elizabeth Lyden; Brenda Weigel; Carola Arndt; William H Meyer; Abby Rosenberg; Douglas S Hawkins Journal: Pediatr Blood Cancer Date: 2017-03-23 Impact factor: 3.167
Authors: Simone de Campos Vieira Abib; Chan Hon Chui; Sharon Cox; Abdelhafeez H Abdelhafeez; Israel Fernandez-Pineda; Ahmed Elgendy; Jonathan Karpelowsky; Pablo Lobos; Marc Wijnen; Jörg Fuchs; Andrea Hayes; Justin T Gerstle Journal: Ecancermedicalscience Date: 2022-02-17