ABSTRACT
AIMS: The aim of this study was to evaluate quality of life among adolescents diagnosed with malignancies and currently undergoing treatment in the age group of 13-18 years old. METHOD: The study used a descriptive cross-sectional design. Participants were recruited from the pediatric oncology department at King Hussein Cancer Center in Jordan. The Arabic version of self-report (adolescent 13-18) pediatric quality of life inventory (PedsQLTM ) 4.0 Generic Core Scale was used. RESULTS: Eighty patients were enrolled in the study. The total score of PedsQL 4.0 generic core was 62.0 (SD=16.5). The highest score was for social functioning (mean=85.4, SD=18.4) and the lowest score was school functioning (mean=39.5, SD=28.4). Females had significantly higher scores for health-related quality of life (HRQOL) in school functioning. Type of tumor did not affect HRQOL. Outpatient participants had significantly higher scores for HRQOL in all domains, except in school functioning where inpatients had a significantly higher score. CONCLUSION: To the best of our knowledge, this is the first study to reveal the HRQOL scores for Jordanian adolescents with cancer. Addressing the special needs in such a unique age group is essential when planning a comprehensive care plan for a better quality of life.
Subject(s)
Neoplasms/therapy , Quality of Life , Adolescent , Combined Modality Therapy , Cross-Sectional Studies , Female , Follow-Up Studies , Humans , Jordan/epidemiology , Male , Neoplasms/epidemiology , Neoplasms/pathology , Prognosis , Self ReportABSTRACT
Pleomorphic xanthoastrocytoma is a rare brain tumor with unique high frequency of BRAF V600E mutation which is plausible for targeted therapy. The anaplastic variant has generally worse prognosis. We present an adolescent patient with a disseminated relapse of anaplastic pleomorphic xanthoastrocytoma following surgery, radiotherapy, and chemotherapy. She had a dramatic and prolonged response to a BRAF inhibitor (Dabrafinib) and later to addition of a MEK inhibitor (Trametinib) on tumor progression. With minimal side effects and a good quality of life, the patient is alive more than 2 years after initiation of targeted therapy. This experience confirms the potential role of targeted treatments in high-grade BRAF-mutated brain tumors.
Subject(s)
Astrocytoma , Brain Neoplasms , Imidazoles/administration & dosage , Mutation, Missense , Oximes/administration & dosage , Proto-Oncogene Proteins B-raf/genetics , Pyridones/administration & dosage , Pyrimidinones/administration & dosage , Adolescent , Amino Acid Substitution , Astrocytoma/genetics , Astrocytoma/therapy , Brain Neoplasms/genetics , Brain Neoplasms/therapy , Female , HumansABSTRACT
Malignant rhabdoid tumor (MRT) of the liver is a rare malignancy with grave prognosis. This entity should be considered in the differential diagnosis of any aggressive liver tumor with low levels of alpha fetoprotein. We report 2 cases of hepatic MRT presenting in infancy. In these 2 cases, we show that loss of INI1 facilitates making the correct diagnosis of primary hepatic MRT utilizing BAF 47 (INI1 gene product) immunostains. Difficulty encountered in making this rare diagnosis, including the need for repeated biopsies, can be avoided if MRT is considered in the differential diagnosis early on and BAF 47 immunohistochemistry is ordered.
