Rhabdomyosarcoma in children: Retrospective analysis from a single tertiary care center in Saudi Arabia.

BACKGROUND: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children occurring most commonly in the head and neck region. The treatment involves using a multimodality approach including chemotherapy, surgery, and radiation therapy. Survival for patients with localized disease has improved markedly, but the treatment of advanced disease remains a challenge. We report the clinical characteristics and outcome for patients treated at a tertiary care center in Saudi Arabia. METHODS: Patients aged 0-14 years diagnosed with RMS between 2005 and 2018 were included. Statistical analysis was performed using SPSS software. Kaplan-Meier method was used to calculate overall and event free survival. Cox proportional hazards model was used for multivariate analysis. RESULTS: One hundred and twenty-four patients were analyzed. The median age was 5.7 years with male predominance (2.4:1). The most common primary sites were head/neck (30%) and the genitourinary tract (25%). Embryonal RMS was present in 81%; alveolar in 19%. Most patients had intermediate risk disease (60%). The 5-year overall and event free survivals were 64.3% and 53.3%, respectively. Survival was influenced by primary tumor site, histology, and clinical risk group. Unfavorable primary site, high risk stratification, and poor initial response to therapy predicted a poor outcome. CONCLUSION: This study provides an insight on the current management outcomes for our patients with RMS. Cytogenetics and molecular diagnostics need to be incorporated as standard of care in the therapeutic approach of our patients. In addition, there is a need for national collaborative efforts to improve the outcome of RMS in children and adolescents.

Assessment of the Effective Management of Patients With Severe Primary Hypercholesterolemia Under Care in Family Medicine Clinics at King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.

Background Atherosclerotic cardiovascular disease (ASCVD) is the primary cause of death in Saudi Arabia. Hypercholesterolemia is a prevalent risk factor that can lead to ASCVD. The American College of Cardiology/American Heart Association (ACC/AHA) guidelines have provided recommendations for managing severe primary hypercholesterolemia, defined as medically well adults 21-75 years of age with low-density lipoprotein cholesterol (LDL-C) ≥190 mg/dL (≥4.9 mmol/L). Underutilization of the guideline recommendations has led to concern and the need for further review. This study aims to review the management of severe primary hypercholesterolemia in the Family Medicine and Polyclinics at King Faisal Specialist Hospital and Research Centre (KFSH&RC) in Riyadh, Saudi Arabia. Methodology In this retrospective cohort study, data were obtained from electronic medical records of patients aged 21-75 years who received care in the Family Medicine and Polyclinics at KFSH&RC in Riyadh with LDL-C ≥190 (≥4.9 mmol/L). The data collected included demographics, body mass index (BMI), LDL-C blood level, and lipid-lowering medications prescribed. We measured the prevalence of hypercholesterolemia, reviewed if appropriate statin therapy was prescribed as per the ACC/AHA guidelines, and determined if treated patients with severe primary hypercholesterolemia achieved LDL-C ≤100 mg/dL (≤2.6 mmol/L) from January 1, 2015, until June 30, 2020. Results The prevalence of hypercholesterolemia was 7.4%. The sample size studied included 195 patients. The majority of patients were aged 40-59 years and were either overweight or obese. Treatment with a moderate-intensity statin was observed in 46.4% of patients, and 45.4% of patients were not prescribed a statin. The LDL-C ≤100 mg/dL (≤2.6 mmol/L) was not achieved in 88.3% of patients. Conclusions Despite guidelines, the majority of patients with severe primary hypercholesterolemia are inadequately managed. High-risk patients need to be diagnosed appropriately so that they receive proper treatment to prevent ASCVD. We encourage adherence to established guidelines in the management of severe primary hypercholesterolemia to prevent premature ASCVD.

A case of spontaneous resolution of a scrotal ventriculoperitoneal shunt migration.

Ventriculoperitoneal (VP) shunt catheter migration remains a rare but documented complication seen in one in 1000 patients who receive a VP shunt. Migration of the VP shunt into the scrotum is even more uncommon and requires surgical treatment. We report a unique case of a 6-month-old preterm male who developed right scrotal migration of his VP shunt. However, the tip of the VP shunt spontaneously reduced to its normal position, and repeated imaging months later showed no recurrence.