We aimed to describe clinico-pathological features and outcomes of Rahbdomyosarcoma (RMS) in Tunisia.
We assembled a retrospective cohort of 47 patients aged 18 or older diagnosed with, and treated for RMS at our institute between 1994 and 2013. Patient's characteristics, tumor variables and treatment outcomes were evaluated. Tumors were classified according to the Intergroup RMS Study (IRS) staging.
Median age was 39 and 66% were male. At presentation, 33 had localised disease and 14 metastatic. Metastasis site was lung in 17% of cases. Sixteen patients had node positive disease. Median tumor size was 9 cm. Patients presented with swelling or palpable mass in 63.8% of cases, pain in 17%, exophthalmia in 6.4% and bleeding in 4.3%. Less common clinical presentations were functional impairment, cranial nerve palsies, superior vena cava syndrome and nasal obstruction seen in one case each. Tumor site was extremities in 40.4% of cases, trunk in 23.4%, head and neck in 19.1% and genito-urinary tract in 17%. According to favorable/unfavorable prognostic location, tumor site was unfavorable in 65.9% of cases and favorable in 33.9%. RMS histologic subtype was pleomorphic in 36.2% of patients, embryonic in 25.5%, not mentioned in 23.4%, alveolar in 12.8% and combination of embryonic and alveolar in 2.1%. Among 38 patients who underwent surgery, 40.4% had complete resection (IRS stage I). Chemotherapy was adjuvant in 4 patients, neoadjuvant in 6 and 1st line in 13 with a median number of cycle of 3 in each schedule. Radiotherapy (RT) was adjuvant in 19.1% of cases and symptomatic/palliative in 21.1%. Median dose of adjuvant RT was 55 Gray. Median progression free survival after adjuvant treatment was 4.5 months, 47.1% relapsed locally, 35.3% developed distant metastasis and 17.6% presented local recurrence with distant metastases. Five year overall survival (OS) was 46% for localised RMS and 40% for metastatic disease. Among all studied factors, non operated patients and those under 30 years old had worse OS with p values of 0.005 and 0 respectively.
Adults with RMS have poor prognosis. All patients should therefore undergo multimodality treatment comprising of surgery, radiation, and chemotherapy to achieve better outcomes.
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All authors have declared no conflicts of interest.