Clinical characteristics and the prognosis of childhood rhabdomyosarcoma in 60 patients treated at a single institute

Rhabdomyosarcoma [RMS] is the most frequent soft tissue sarcoma in children. The aim of study was to retrospectively review the treatment results of childhood rhabdomyosarcoma and identify prognostic factors. 60 children with rhabdomyosarcoma treated between 1996 and 2002 in Shafa Hospital were reviewed. The data were analyzed for clinico-epidemiological factors. Age, gender, race, histology type, primary site, tumor size and intergroup rhabdomyosarcoma study [IRS] group were evaluated. The primary site of involvement was orbit in 6 cases [10%] head and neck nonparameningial in 12 cases [20%], parameningial region in 12 cases [20%]. The histological findings were as follows: 12 cases [72.5%] for embryonal, 6 cases [10%] for alveolar and 11 cases [17. 5%] for botryoid type. With respect to the IRS III [15%] were group II, 32 [52.5%] were group III and 24 cases [40%] were group IV. The 5-year survival rate was 47.9%. Primary tumor site [P=0.0003], and histology [P=0.05] were associated significantly with survival after recurrence. Among the variables, age, gender, regional lymph node involvement, and IRS group did not affect 5-year survival but the type and time of recurrence [P=0.0002], and its relation with therapy [P=0.0001] were associated with survival. This study showed that overall survival for rhabdomyosarcoma is dependent on histological subtype, primary site, disease group, duration of disease before treatment. The outcome for infant with RMS is less satisfactory than older children and the patients aged 1-9 years had the best 5 year survival

[Prevalence of factor VIII inhibitor in patients with hemophilia A in Sanandaj]

Factor VIII inhibitor is one of the most problems in the treatment of hemophilia. Patients with hemophilia who have inhibitors are capable of severe hemorrhage and treatments of these patients are more expensive and more difficult than those patients without inhibitor. The aim of present study was to determine the prevalence of inhibitor and to assess the necessity of screening of the factor VIII inhibitors in hemophilia patients. 104 patients with hemophilia A referred to Besat Hospital of Sanandaj during 2006-2009 were evaluated for inhibitors against factor VIII using convenient sampling method. Their venous blood samples were mixed in a proportion of 9 to 1 with 3.2g/dl citrate sodium. After preparation of plasma APTT mix test and then Bethesda test followed by determination of inhibitor were performed. The mean age of subjects was 23.8-+/9.3 year. Twenty out of 104 patients with hemophilia A [19.2%] had inhibitor factor VIII. None of patients were assayed for inhibitor previously. Sixteen patients were low responder and four patients showed high responder with the Bethesda inhibitor level above five unit. Higher levels of inhibitor were existed in 11-25 years old age groups. There was no significant relationship between factor VIII inhibitor and patient's age. Regarding to high prevalence of the factor VIII inhibitor in these patients, screening all hemophilia A patients every 6 months and doing Bethesda test for new identified cases with this inhibitor is highly recommended