Neurofibromatosis in Children: Actually and Perspectives

The three types of neurofibromatosis, namely type 1, type 2, and schwannomatosis, are generally associated with various benign tumors affecting the skin and the nervous system. On rare occasions, especially in patients with neurofibromatosis type 1 (NF1), malignant neoplasms may also be present, several of them possessing a more aggressive course than in individuals without this syndrome. As such, a clear delineation between the three variants of neurofibromatosis is crucial to establish the correct diagnosis and management, as well as predict the neoplasm-related outcomes. Neurofibromin, the principal product of the NF1 gene, is a potent inhibitor of cellular proliferation, having been linked to several key signaling pathways involved in tumor growth. Therefore, it may provide a useful therapeutic target for tumor management in these patients. In this article, we want to present the association between deficiency of neurofibromin and the consequences of the lack of this protein leading to different kinds of malignant tumors. The therapy is still uncertain and most therapeutic options are in development or clinical trials.

Impact of Balance Training and Co-ordination Exercises in Post-Operative Left Cerebellopontine Angle Tumor: A Case Report

Introduction: Acoustic neuromas are most common tumors of CP angle, accounting more than 90% of all such tumors. Meningioma, primary cholesteratoma and facial nerve schwanoma are the different type of tumors. Acoustic neuroma is a benign tumor situates in CP angle which has a fibrous growth and originates from the division of vestibulochochlear nerve. Aim: Impact of Balance Training and Co-ordination Exercises in Post-Operative Left Cerebellopontine Angle Tumor Case Presentation: A 40 year old male with right hand dominance was referred to physiotherapy department. On examination he presented mild impairment in balance and co-ordination, assisted walking. Discussion: This case report is an important to the published literature on rehabilitation of a patient witha CPA tumor, as it presents the sequential management in the patients post CPA tumor. Conclusion: Acoustic neuroma is most common CPA tumor. Management of CPA tumor is important to improve quality of life. As per the reference articles and the exercises planned can progressively improve balance and co-ordination of patients.

Physiotherapy Rehabilitation in Subject with Scapular Osteochondroma: A Case Report

Introduction: The most prevalent benign tumour affecting the metaphysis of long bone is osteochondroma, a form of cartilaginous tumour. Solitary or multiple osteochondromas can prevail.Hereditary multiple exostosis (HME) or familial osteochondromatosis is the multiple variant, which is an autosomal dominant syndrome. Case Presentation: A 35-year old female with right-hand dominance was referred todepartment of physiotherapy. On palpation small nodular swelling of marble size was present in left axilla. X-rays findings showed increased scapular mass and ribs were compressed laterally. Discussion: The most widespread benign tumour is osteochondroma. In 3.0-6.4 percent of all instances, the scapula is implicated. The osteochondroma normally develops on the scapula's anterior aspect. The scapula osteochondroma induces pain and a grating sound when the scapula is moved. Mechanic irritation of muscle, tendon, or soft tissue, emergence of a pseudoaneurysm or bursa, fracture, or malignant transformation are all causes of symptoms. Conclusion: Osteochondroma is a common benign tumor. Management of osteochondroma is important to improve the quality of life. Physiotherapy plays an important role in managing Scapular osteochondroma.

P.204 Integration of patient directed approach in pituitary tumor management – The Ottawa Hospital experience

Background: Pituitary adenomas are common and often require complex multidisciplinary care with multiple specialists. This may result in a health care system that is challenging for patients to navigate. Audits of care at our institution revealed opportunities for improvement to better align care with patients’ needs. Methods: A quality improvement initiative that incorporated a patient advisory committee of patients who had received treatment for pituitary adenoma at our center and their family members was used to help identify opportunities for improvement. The patient-identified gaps in care included the need to coordinate and minimize appointments and the desire for better communication and education. Based on this information, changes were implemented to the pituitary program, including increasing access to the multidisciplinary clinic and developing a standardized and centralized triage process. Results: A pre and post-intervention analysis consisting of retrospective chart reviews revealed that these changes had an impact on wait times for first assessment, and a significant shift in location of this first visit – with a larger proportion of patients being seen in the multidisciplinary clinic after intervention. Conclusions: We demonstrate that patient involvement, beyond individual patient-physician interactions, can lead to meaningful and observable changes, and can improve the quality of care for pituitary adenoma.

American Society of Clinical Oncology 2021 Annual Meeting updates on primary brain tumors and CNS metastatic tumors

In this report, select key studies presented at the American Society of Clinical Oncology (ASCO) 2021 annual meeting are reviewed. Two major phase III randomized controlled trials were presented at the meeting: GEINO 1401 and EORTC 1709/CCTG CE.8. Both are reviewed in this report. Moreover, important phase II trials, including Alliance A0716701, and key phase I trials are included. All trials presented cover important advances in the understanding of primary brain tumor management. In addition, case series papers, trials in progress and select work on exploratory CSF biomarkers are reviewed. Altogether, research presented at ASCO 2021 highlights important advances in neuro-oncologic topics that may inform future research and practice.

Identification of immune-related subtypes of colorectal cancer to improve antitumor immunotherapy

Immunotherapy involving immune checkpoint inhibitors (ICIs) for enhancing immune system activation is promising for tumor management. However, the patients’ responses to ICIs are different. Here, we applied a non-negative matrix factorization algorithm to establish a robust immune molecular classification system for colorectal cancer (CRC). We obtained data of 1503 CRC patients (training cohort: 488 from The Cancer Genome Atlas; validation cohort: 1015 from the Gene Expression Omnibus). In the training cohort, 42.8% of patients who exhibited significantly higher immunocyte infiltration and enrichment of immune response-associated signatures were subdivided into immune classes. Within the immune class, 53.1% of patients were associated with a worse overall prognosis and belonged to the immune-suppressed subclass, characterized by the activation of stroma-related signatures, genes, immune-suppressive cells, and signaling. The remaining immune class patients belonged to the immune-activated subclass, which was associated with a better prognosis and response to anti-PD-1 therapy. Immune-related subtypes were associated with different copy number alterations, tumor-infiltrating lymphocyte enrichment, PD-1/PD-L1 expression, mutation landscape, and cancer stemness. These results were validated in patients with microsatellite instable CRC. We described a novel immune-related class of CRC, which may be used for selecting candidate patients with CRC for immunotherapy and tailoring optimal immunotherapeutic treatment.

Analysis of quality control group of departments on improving effect of PICC maintenance quality in outpatient children with tumor

To explore the clinical effect of quality control group of departments on improving PICC maintenance quality in outpatient children with tumor. Methods: A total of 4100 children with tumor who were treated in our outpatient department of intravenous therapy from January to December 2020 were divided into observation group (July to December 2020: after the establishment of quality control department) and control group (January to June 2020: before the establishment of quality control group of departments). In the control group, conventional PICC treatment management and maintenance measures were used. In the observation group, quality control group was used to manage, and moreover, the PICC maintenance quality, the incidence rate of PICC-related risk events and the level of PICC maintenance knowledge and satisfaction evaluation were compared between the two groups. Results: (1) Comparing the PICC maintenance quality between the two groups, the rate of absence of PICC maintenance during the course of tumor management in the observation group (1.14%) was significantly lower than that in the control group (4.00%), and there was significant difference in the PICC maintenance quality between the two groups (P<0.05). (2) Comparing the relative indexes of children with tumor: Before intervention, there was no significant difference in the relative indexes of children with tumor (PICC maintenance knowledge level) between the two groups (P>0.05), but after intervention, the relative indexes of children with tumor (PICC maintenance knowledge level, and service evaluation recognition degree) in the observation group were significantly higher than that in the control group (P<0.05). (3) Comparing the probability of PICC-related risk events between the two groups: In the course of tumor management, PICC-related complications were observed in the observation group (3.59%, 3.18%), which were significantly lower than those in the control group (11.95%, 11.37%) (P<0.05). Conclusion: The application of quality control group of departments in the management of PICC in outpatient children with tumor has obvious clinical effect. It can effectively improve the level of maintenance knowledge and service satisfaction of family members, optimize the quality of maintenance, reduce the rate of lack of maintenance and risk related to PICC, and is worthy of being popularized in clinical practice.

Falx Cerebelli Meningioma: Case Report of a Rarely Occurring Tumor, Management Nuances, and Literature Review

The falx cerebelli is a small crescent fold of dura mater that is attached to the internal occipital crest and projects forward into the posterior cerebellar notch between the cerebellar hemispheres. We report a rare case of a 61-year-old female who presented with a 1-month history of headache and gait instability. Imaging findings were suggestive of a meningioma arising from the falx cerebelli. Complete surgical resection was achieved with a standard posterior fossa midline approach. Duraplasty was performed using animal allograft dura (Duraguard) and additional layers of oxidized cellulose preparation (Surgicel), fibrin sealant, and nonsuturable collagen matrix (Duragen) were utilized to reduce the risk of a cerebrospinal fluid leak. Pathology confirmed a World Health Organization (WHO) grade-I meningioma. Postoperatively, patient with asymptomatic thrombosis of the left transverse/sigmoid sinuses and later with a pseudomeningocele managed with a lumbar drain. To our knowledge, this is the second documented case in the literature. We discuss intraoperative nuances and unique aspects in the postoperative care and management of these patients.