Operative management and outcomes in children with pheochromocytoma.

OBJECTIVE: This study aimed to evaluate management and prognosis in children with pheochromocytoma who were treated at an Egyptian tertiary center. METHODS: The authors conducted an 8-year retrospective analysis for 17 patients who were presented from January 2013 to January 2021. Clinical criteria, operative details, and follow-up data were assessed. Overall (OS) and event-free survival (EFS) were estimated by the Kaplan-Meier method. An event was assigned with the occurrence of recurrence or metachronous disease, or death. RESULTS: Median age at diagnosis was 14 years (range: 6-17.5 years). Ten patients (58.8%) were males and seven (41.2%) were females. Hypertension-related symptoms were the main presentations in 15 patients (88%). None of the included children underwent genetic testing. Sixteen patients (94%) had unilateral tumors (right side: 12), whereas only one was presented with bilateral masses. The median tumor size was 7 cm (range: 4-9 cm). Metastatic workup did not reveal any metastatic lesions. All patients underwent open adrenalectomy, and clinical manifestations were completely resolved after surgery. Adjuvant therapy was not administered to any patient. There were no deaths or relapses at a median follow-up time of 40 months, whilst two children had metachronous disease after primary resection. Both were managed by adrenal-sparing surgery, and they achieved a second complete remission thereafter. Five-year OS and EFS were 100% and 88%, respectively. CONCLUSIONS: Complete surgical resection achieves excellent clinical and survival outcomes for pheochromocytoma in children. Meticulous, long-term follow-up is imperative for early detection of metachronous disease to facilitate adrenal-sparing surgery. Genetic assessment for patients and their families is essential; however, it was not available at our institution.

Surgical Resection of Hepatoblastoma: Factors Affecting Local Recurrence.

INTRODUCTION: This study aimed to investigate potential factors contributing to local recurrence after surgical resection of hepatoblastoma (HB). MATERIALS AND METHODS: This retrospective study involving all patients with HB who underwent nontransplant surgery at our tertiary center between July 2007 and July 2018. Data were analyzed regarding microscopic surgical resection margin, tumor multifocality and extracapsular tumor extension in correlation with local recurrence. These relations were assessed by logistic regression. RESULTS: The study included 133 patients with a median age of 1.3 years (range: 0.5-12.8 years). They were classified into 99 cases (74.44%) standard risk and 34 cases (25.56%) high risk. Delayed surgical resection was adopted in all patients. Follow-up to July 2019 revealed that 23 patients (23/133, 17.3%) developed local recurrence, whereas the remaining 110 were locally disease free. Microscopic positive margin (R1) was detected in 29 patients, 8 of them had local recurrence (p = 0.097). Regarding tumor multifocality, there were 12 patients who had multifocal lesions, 3 of them developed local recurrence (p = 0.459). Forty-four patients had extracapsular tumor extension in their pathological reports, 12 of them had local recurrence (p = 0.032). CONCLUSION: Extracapsular tumor extension was a significant prognostic factor of local recurrence after surgical resection of HB. R1 margin does not necessarily require a second resection, and it could achieve accepted results when combined with adjuvant platinum-based chemotherapy. However, patients who are not eligible for surgical resection must be transferred for primary transplantation to obtain favorable outcome.

Diagnostic, prognostic and predictive values of miR-100 and miR-210 in pediatric acute lymphoblastic Leukemia.

BACKGROUND: : microRNAs are playing important roles in the diagnosis and prognosis of pediatric acute lymphoblastic leukemia (ALL). METHODS: Expression levels of miR-100 and miR-210 were assessed in bone marrow aspirate of 85 pediatric ALL patients compared to 12 healthy control using quantitative real-time polymerase chain reaction. Data were correlated with relevant clinico-pathological features of the patients, response to treatment, disease-free survival (DFS), and overall survival (OS). RESULTS: miR-100 was significantly downregulated in ALL patients [median: 1.21, range: 0-434.3] compared to the control group [median: 8.41, range; 0-840.3, P = 0.035]. miR-210 was significantly upregulated in ALL patients [median: 6.34, range: 1.16-1088.7] compared to the control group [median: 2.57, range: 0.11-709.2, P = 0.025]. The sensitivity, specificity, and area under curve of miR-100 were (64.7%, 62.5%, and 0.642; respectively, P = 0.035) at a cut-off 2.6 and that of miR-210 were (60%, 58.3% and 0.650; respectively, P = 0.025) at a cut-off 3.5. miR-100 overexpression associated with shorter DFS and OS (P = 0.033 and 0.046; respectively). Patients with miR-100 lowexpression showed a significant incidence of late death ( P = 0.024). There was no significant association between miR-210 expression and DFS, OS, incidence of early or late death. CONCLUSION: : miR-100 and miR-210 could be used as potential diagnostic markers for pediatric ALL. miR-100 is a useful prognostic and predictive biomarker for childhood ALL.

Outcome of Children Treated for Infantile Hepatic Hemangioendothelioma.

PURPOSE: Infantile hepatic hemangioendothelioma (IHHE) is the most common hepatic vascular tumor in children. We report on the treatment outcome of our large single-center experience of patients with IHHE over a 9-year period. MATERIALS AND METHODS: A retrospective analysis of all IHHE patients treated at the Children Cancer Hospital Egypt from April 2008 through April 2017. RESULTS: In total, 28 patients (18 females, 10 males) were diagnosed with IHHE with a median age at diagnosis of 3 months. The lesions were multifocal (n=12), focal (n=10), and diffuse (n=6). Six (21.4%) patients initially had low T3 and T4. Eleven patients did not receive any treatment, whereas 1 patient underwent resectional surgery. Sixteen patients received drug treatment, 9 of whom responded well to first-line propranolol/prednisolone, whereas 7 patients needed salvage treatment. Twenty-five patients are alive, whereas 3 patients have died. CONCLUSIONS: Overall, patients with IHHE do well, a significant percentage of whom do not require drug therapy, particularly for those with small focal lesions. In patients with multifocal/diffuse disease, there is a high incidence of low T3 and T4 and while some of these patients did well without additional therapy, those with rapidly progressive lesions during treatment may do poorly.

In vitro knock-out of miR-155 suppresses leukemic and HCV virus loads in pediatric HCV-4-associated acute lymphoid leukemia: A promising target therapy.

Hepatitis C virus (HCV) infection is a major public health problem, having a high prevalence in Egypt. Leukemia and lymphoma have been associated with HCV infection. MicroRNA-155 (miR-155) has been reported to play a regulatory role in cancer, inflammation, and immune response to infection. The expression level of miR-155 in HCV viremic patients is controversial; although high miR-155 levels were demonstrated in HCV genotypes 1,2, and 3, low levels of miR-155 were detected in Egyptian patients with HCV genotype 4. Several studies have investigated the correlation between the levels of miRNA-155 and the replication of HCV, others have evaluated miRNA-155 as a prognostic biomarker in different types of cancer. No studies have investigated the impact of miRNA-155 knockdown on HCV pediatric patients associated with childhood acute lymphoblastic leukemia (ALL). We knocked-out the miR_155a in cultured polymorphonuclear cells (PBMCs) obtained from 60 children with ALL; 30 were associated with HCV-4 infection and 30 were HCV negative. The miR_155a, HCV viral load, and cell proliferation werre assessed in treated and untreated cells using TaqMan assay quantitative polymerase chain reaction. We found that miRNA-155 was significantly upregulated by seven folds in the HCV-4 associated ALL group; while being linked to high HCV viral load and leukemic burden, miR_155a knock-out can improve the disease outcome. We conclude that miR-155 is a critical miRNA that is considered a therapeutic target in pediatric HCV leukemic patients.

Surgical approaches, anaesthetic management and outcome in pediatric superior mediastinal tumors.

BACKGROUND: Pediatric superior mediastinal tumors are a heterogeneous group of tumors with marked variation in pathology and extension. We reviewed our experience with different surgical approaches to tumors originating from or extending to superior mediastinum in pediatrics. PATIENTS AND METHODS: The medical records of all patients who had undergone resection for superior mediastinal tumors in Children's Cancer Hospital - Egypt, between January 2008 to December 2015, were reviewed for demographic data, clinico-pathological features, radiologic findings, operative techniques and outcome. RESULTS: The study included 20 patients. Diagnosis included: germ cell tumors (n=8), neuroblastoma (n=4), soft tissue sarcoma (n=3), thymolipoma (n=2), infantile fibromatosis (n=1), calcifying fibrous tumor (n=1), and thymic carcinoma (n=1). Tumor extension was divided into tumors extending unilaterally to one hemithorax (n=9), tumors extending bilaterally to both hemithoraces (n=4), and cervico thoracic junction tumors (n=7). Extended lateral thoracotomy was used in 8 patients. Other approaches included trapdoor (n=5), clamshell (n=4), cervical approach (n=2) and double level lateral thoracotomy (n=1). There was no perioperative mortality, and postoperative morbidity was 20%. At the end of December 2016, 15 patients were alive free of disease, 5 patients developed local and/ or distant relapse. CONCLUSION: Pediatric superior mediastinal tumors could be divided into 3 groups according to tumor extension. Each group has an optimum surgical approach that achieves the best exposure for adequate resection. However, further research is needed to confirm the conclusion as this was a descriptive study and the sample size was too small for valid statistical analysis.