Treatment and Outcome of Patients Suffering From Perineal/Perianal Rhabdomyosarcoma
| Autor: | Tobias M. Dantonello, Ivo Leuschner, Daniel Kosztyla, Thomas Klingebiel, Guido Seitz, Joerg Fuchs, Gunnar Blumenstock, Ewa Koscielniak, Andreas Schuck, Felix Niggli |
|---|---|
| Přispěvatelé: | University of Zurich, Seitz, Guido |
| Rok vydání: | 2014 |
| Předmět: |
Male
medicine.medical_specialty Time Factors medicine.medical_treatment Anal Canal 610 Medicine & health Perineum Disease-Free Survival law.invention Randomized controlled trial Quality of life law Germany Surveys and Questionnaires Rhabdomyosarcoma Humans Medicine Fecal incontinence Child Pelvic Neoplasms Retrospective Studies Chemotherapy business.industry Retrospective cohort study Anal canal medicine.disease Combined Modality Therapy 2746 Surgery Surgery Survival Rate Treatment Outcome medicine.anatomical_structure 10036 Medical Clinic Child Preschool Quality of Life Female medicine.symptom business Switzerland Follow-Up Studies |
| Zdroj: | Annals of Surgery. 259:1166-1172 |
| ISSN: | 0003-4932 |
| Popis: | OBJECTIVES: 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. |
| Databáze: | OpenAIRE |
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