Sellar Atypical Teratoid/Rhabdoid Tumors (AT/RT): A Systematic Review and Case Illustration.

INTRODUCTION: Atypical Teratoid/Rhabdoid tumors are rare, highly malignant tumors in adults, with a median survival of 20 months. We report a case of a sellar atypical teratoid/rhabdoid tumor in a 70-year-old female treated with intraventricular chemotherapy, followed by a systematic review of the current management of sellar AT/RTs. METHODS: A comprehensive systematic literature search was conducted on Web of Science, Scopus, and PubMed Central using the key terms "sellar" and "atypical teratoid/rhabdoid tumors", following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Data, including patient demographics, histology, treatments, and overall survival were extracted and analyzed. Kaplan-Meier survival curves and log-rank analysis were used to compare survival outcomes between different treatment regimens. RESULTS: Our literature search disclosed 123 publications. After prespecified exclusions, 41 patients with sellar AT/RT from 30 manuscripts were identified, and 38 were included in the final analysis. Including our patient, the median age was 44 (range: 20-70) with a substantial female predominance (94.7%). Collectively, patients who received combined chemoradiation therapy had a significantly increased overall survival compared to those who received single modality or no adjuvant therapies (median OS 27 vs. 1.25 months; p=0.0052). CONCLUSION: Atypical teratoid/rhabdoid tumor in the sellar region carries a poor prognosis. Adjuvant chemotherapy and radiation therapy were associated with significantly increased overall survival. Early consideration of neuro-oncology and radiation-oncology referral and management is likely beneficial in this patient population. Intrathecal chemotherapy is a treatment modality that requires further exploration given the limited options and current dismal prognosis of adult sellar AT/RT.

Safe Placement of Ommaya Reservoirs in Thrombocytopenic Patients: One Institutions Experience.

Objective The purpose of this study was to assess the risk of hemorrhagic complications in thrombocytopenic patients after Ommaya reservoir placement. Methods Between 2009 and 2017, 192 patients were identified on the National Neoplastic Meningitis Registry and had undergone Ommaya reservoir placement for intrathecal chemotherapy. A retrospective chart review was performed to collect the preoperative and postoperative platelet levels, whether or not the patient received any transfusion of platelets, neurological exams, and whether a postoperative head CT was obtained. Using generally accepted recommendations, a platelet level less than 100,000/µL was considered clinically significant and used as our threshold for thrombocytopenia. Results Seven patients (3.6%) were identified as thrombocytopenic in our patient population with platelet counts ranging from 54,000 to 99,000/µL. Primary diagnoses for the seven patients included leukemia, prostate cancer, primary brain cancer (four patients), and lung cancer (non-small-cell lung carcinoma). One patient received platelet transfusions preoperatively. Three patients had a routine head CT obtained postoperatively with no abnormal findings noted. There were no changes in the neurological exam noted in all of the patients included in this study. No clinically significant hemorrhages were identified in our patients. Conclusions From our single institutional experience, we found that thrombocytopenia is fairly uncommon, found in only 3.6% of our patients undergoing placement of Ommaya reservoirs. We did not encounter any increased risks of postoperative hemorrhage in studied thrombocytopenic patients.

Development of a sickle cell disease readiness for transition assessment.

BACKGROUND: Instrumentation with established reliability and validity is not yet routinely utilized to assess readiness for transition from pediatric to adult care for youth and young adults with chronic conditions, including sickle cell disease (SCD). OBJECTIVE: The aim of this study was to develop a SCD specific readiness for transition assessment tool. SUBJECTS: Fifty-seven youths with SCD, ages 15-21 years, completed the initial version of the Transition Intervention Program - Readiness for Transition (TIP-RFT) assessment; 113 youths/young adults with SCD, ages 14-26 years, at two distinct sites of care completed a refined version of the TIP-RFT. METHODS: The TIP-RFT was constructed based on a literature review, provider and patient consensus and assessed domains including knowledge and skills in medical self-care, social support, health benefits and independent living and educational/vocational skills. We used principal components factor analysis to evaluate TIP-RFT responses and assessed differences in TIP-RFT scores in relation to age, gender, sickle cell diagnosis and site of care. RESULTS: The original TIP-RFT, which had demonstrated face validity, was reduced from 56 to 22 items. The revised instrument consisting of four subscales demonstrated good internal consistency reliability and construct validity. CONCLUSION: Our results support that the TIP-RFT is a valid and reliable tool for the assessment of transition readiness for youths with SCD. The TIP-RFT assessment can guide interventions to improve transition readiness and can provide a foundation for future research on other variables that might be associated with transition readiness.

Self-efficacy and readiness for transition from pediatric to adult care in sickle cell disease.

BACKGROUND: Theories of self-care management, particularly the development of self-efficacy or confidence in one's ability to manage health-related goals, tasks, and challenges may provide a useful framework for developing programs to improve transition from pediatric to adult care for youth and young adults with sickle cell disease (SCD). OBJECTIVE: The aim of this study was to evaluate the hypothesis stating that ratings of self-efficacy is positively associated with self-ratings of transition readiness. SUBJECTS: A total of 113 individuals with SCD aged 14-26 years at two distinct sites of care were recruited for the study. MATERIALS AND METHODS: Participants completed the Transition Intervention Program Readiness for Transition (TIP-RFT) assessment, the Sickle Cell Self-Efficacy Scale and the Sickle Cell Stress -Adolescent scale. RESULTS: In multivariate regression models, self-efficacy was positively associated with scores on the total TIP-RFT and on the Education/Vocation Planning and Independent Living Skills scales. Older age was independently associated with higher scores on the Independent Living Skills scale and higher stress levels were independently associated with lower scores on Education/Vocation Planning scale. CONCLUSION: The TIP-RFT assessment, along with measures of self-efficacy and stress, appear to be useful measures of overall transition readiness for youth and young adults with SCD. Future studies should evaluate whether self-management skill development and health outcomes are indeed affected by programs to improve readiness for transition from pediatric to adult care.