TURKISH JOURNAL OF ONCOLOGY 2017 , Vol 1 , Num 3
Adult Rhabdomyosarcoma: Clinical Features and Radiotherapy Outcomes?The Turkish Oncology Group (TOG) Bone and Soft Tissue Sarcoma Study Group
Esra KORKMAZ KIRAKLI1,Ayça İRİBAŞ2,Arzu ERGEN3,Banu ATALAR4,Fulya AĞAOĞLU2,Fazilet ÖNDER DİNÇBAŞ3,Emin DARENDELİLER2,Yavuz ANACAK5,Serra KAMER5
1Department of Radiation Oncology, Dr. Suat Seren Chest Diseases and Surgery Training and Research Hospital, İzmir-Turkey
2Department of Radiation Oncology, İstanbul University Oncology Institute, İstanbul-Turkey
3Department of Radiation Oncology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul-Turkey
4Department of Radiation Oncology, Acıbadem University Faculty of Medicine, İstanbul-Turkey
5Department of Radiation Oncology, Ege University Faculty of Medicine, İzmir-Turkey
DOI : 10.5505/tjo.2018.1841

OBJECTIVE: Although rhabdomyosarcoma (RMS) is the most frequent sof tissue sarcoma diagnosed in childhood, it represents only 2%–5% of adult sof tissue sarcomas. Te aim of the present study was to better understand the clinical characteristics, treatment approaches, and outcomes of patients with adult RMS who received radiotherapy (RT) as a component of their multidisciplinary management since there are scarce data on adult RMS due to its rarity.

METHODS: Te medical records of patients with adult RMS who were ≥18 years old and treated with RT between January 1995 and August 2016 in four di?erent radiation centers were evaluated in terms of clinical characteristics, treatment, and follow-up data retrospectively.

RESULTS: Tere were 28 patients. Te median age at diagnosis was 28 (19–53) years. Te most common site of involvement was the head and neck (25%), and parameningeal region involvement was prominent (92%) among them. In general, unfavorable site of involvement was markedly higher than favorable ones (82% vs. 18%). Alveolar and pleomorphic subtypes compromised 75% of the cases. Fifeen patients had surgery, 26 chemotherapy, 10 radical intent of RT, 9 adjuvant, 3 preoperative, and 6 palliative. Te follow-up time was from 3 to 235 (median 18) months, disease-free survival was between 2 and 48 (median 12) months, and 5-year overall survival (OS) was 25% (median OS 20 (4–235) months). Tere were signifcant di?erences in terms of survival according to histopathological subtypes (p: 0.017), risk groups (p<0.001), Intergroup Rhabdomyosarcoma Study Group (IRSG) grouping and IRSG staging (p<0.001).

CONCLUSION: Adult RMS has unfavorable clinical presentation and worse outcome compared with pediatric RMS. Histopathological subtype and risk grouping to defne the prognosis used in pediatric cases also might be valid in adult RMS.

Keywords : Adult rhabdomyosarcoma, Sof tissue sarcoma, Treatment, Outcomes