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Objectives To compare two questionnaires that measure health-related quality of life (HRQL) in idiopathic pulmonary fibrosis (IPF) patients. Background IPF is a disease with a progressive course as it is associated with high rates of mortality and a median survival rate of 3–4 years, so it severely affects the patient’s quality of life. General and disease-specific questionnaires are available to assess HRQL. Patients and methods HRQL was measured in 30 previously diagnosed IPF patients using a general questionnaire [short form 36 and specific questionnaires − St George’s Respiratory Questionnaire (SGRQ)]. Pulmonary function tests and 6-min walking distance test (6MWDT) were used to assess the patients’ functional status. Dyspnea at rest was evaluated by baseline dyspnea index and after exercise by the modified Borg scale. Results Pulmonary function tests showed a restrictive pattern, a decline in the exercise capacity of the patient (6MWDT), and higher scores of dyspnea at rest and following exercise. Both questionnaires showed a good correlation with some of patients’ functional parameters but all of the SGRQ domains showed a strong correlation with all functional status parameters. Also, a good correlation with HRQL was presented by 6MWDT and baseline dyspnea index. Conclusion Specific SGRQ is a more suitable instrument for HRQL assessment in IPF patients than the general questionnaire (short form 36).

Purpose: To report our experience and management strategies during 10 years for 137 childhood craniopharyngiomas treated at a single institution., Methods: Medical records of children with craniopharyngioma treated at Children's Cancer Hospital Egypt (CCHE-57357) from July 2007 to December 2017 were retrospectively reviewed. Beta-catenin as an immunohistochemical marker was assessed also in available specimens., Results: Our registry included 137 patients. Headache (n = 122), visual failure (n = 118), and hypothyroidism(n = 78) were the most common findings on presentation. Three management protocols were identified; 65 patients were primarily followed up after surgery, 71 patients had radiotherapy after surgery, and one patient underwent surgery for Ommaya insertion with intracystic interferon injection. Overall, gross total resection/near total resection was achieved in 48 cases (35.04%), subtotal resection was achieved in 58 patients (42.33%), 29 (21.16%) had biopsy and Ommaya reservoir, and two patients with calcified lesions had no operations. Fifty-four patients showed recurrence/progression of their lesions. Allover, 5-year progression-free survival (PFS) was 52.3%, while it was 34.49% and 72.25% for the follow-up group and the radiotherapy group, respectively. Beta-catenin mutations were positive in 61/95 patients; 5-year PFS for beta-catenin negative and positive cases was 65.5% and 39.4% respectively (p = 0.087). Mortality was reported in eight patients. Intraoperative endoscopy-assisted assessment was the cornerstone of tailored decision-making., Conclusion: The concepts of conservative surgery and multimodal management should be applied to reach the perfect balance between the quality of life and the best tumor control rates. Beta-catenin mutations more than 5% are associated with statistically trending aggressive clinical behavior. The CCHE-57357 algorithm of individualized management protocol was presented.

Purpose: Our aim is to present our experience in the management of pediatric meningiomas in the largest referral center in Egypt for pediatric tumors focusing on variables including clinical picture, anatomic location, histopathology, treatment strategies, and outcome together with their possible correlation to prognosis., Methods: We retrospectively reviewed the medical records of 39 pediatric patients who were treated for CNS meningiomas in Children's Cancer Hospital-Egypt (CCHE-57357) 2007-2017., Results: The prevalence of pediatric meningioma was 1.42%. Four cases had type 2 neurofibromatosis (NFII). The mean age was 8.19 years. The presence of NFII was associated with challenging multiple lesions, older age of presentation and poorer prognosis and functional outcome. Convexity was the commonest location. Gross total resection (GTR) was achieved in 28 cases, subtotal resection (STR) in 8 cases, and biopsy was decided in 3 patients. Histopathological examination revealed WHO grade I in 16 patients and higher grades in 23 patients (59%). The 5-year overall survival (OS) rate was 87.8% while the 5-years event-free survival (EFS) rate was 85.6%. Tumor location, histopathology, and clinical presentation were not statistically correlated to prognosis., Conclusions: Pediatric CNS meningiomas are uncommon pediatric tumors but of an aggressive clinical and pathological behaviors as compared to adult meningiomas. The presence of NFII is associated with a poorer prognosis and functional outcomes. Although being challenging, the maximum and safe surgical excision should be exercised even in recurrent cases in order to achieve the best outcome. Adjuvant radiotherapy provides good tumor control for inoperable residual atypical or anaplastic meningiomas.