The neurotrophic tropomyosin receptor kinase (NTRK) genes (NTRK1, NTRK2, and NTRK3) code for three transmembrane high-affinity tyrosine-kinase receptors for nerve growth factors (TRK-A, TRK-B, and TRK-C) which are mainly involved in nervous system development. Loss of function alterations in these genes can lead to nervous system development problems; conversely, activating alterations harbor oncogenic potential, promoting cell proliferation/survival and tumorigenesis. Chromosomal rearrangements are the most clinically relevant alterations of pathological NTRK activation, leading to constitutionally active chimeric receptors. NTRK fusions have been detected with extremely variable frequencies in many pediatric and adult cancer types, including central nervous system (CNS) tumors. These alterations can be detected by different laboratory assays (e.g., immunohistochemistry, FISH, sequencing), but each of these approaches has specific advantages and limitations which must be taken into account for an appropriate use in diagnostics or research. Moreover, therapeutic targeting of this molecular marker recently showed extreme efficacy. Considering the overall lack of effective treatments for brain neoplasms, it is expected that detection of NTRK fusions will soon become a mainstay in the diagnostic assessment of CNS tumors, and thus in-depth knowledge regarding this topic is warranted.

Background/Objectives: Adrenocortical tumors (ACTs), including adrenocortical adenoma (ACA) and carcinoma (ACC), represent 0.3–0.4% of pediatric tumors. Beckwith–Wiedemann spectrum (BWSp) confer an increased risk of ACTs, but prognosis, management, and associated molecular characteristics are unclear. Methods: This paper combines a literature review of 54 published cases of BWSp-ACT with a report of one newly identified patient, totaling 55 cases with a confirmed BWSp clinical and/or molecular diagnosis. Results: Nineteen patients with ACA, 33 with ACC, and 3 with ACT of uncertain malignant potential (umACT) were included. Twenty patients had uniparental disomy of chromosome 11p15.5 (patUPD11), 11imprinting Center 2 Loss-of-methylation (IC2-LoM), and had 2 11p15 locus duplication. Eleven patients were diagnosed during cancer screening procedures, including two metastatic at diagnosis ACC. Conclusions: Almost half of ACC patients reached the minimum score for clinical BWSp diagnosis only after ACC onset, suggesting that the BWSp score has limited value for the early diagnosis in such a setting. Two patients with metastatic ACC had a histopathological Wieneke score ≤2, not correlating with clinical malignancy and confirming limitations of the current histopathological classification, as previously documented. Ultrasound screening failed identifying the ACC before metastasis in two cases, indicating an urgent need to develop new strategies for screening of ACTs in BWSp. Furthermore, some cases of metastatic ACC exhibited unexpectedly indolent behavior despite being malignant.

Purpose: Palliative care for adolescents with poor-prognosis cancer raises distinct and unresolved psychological challenges that differ from the broader adolescent and young adult (AYA) oncology population. This article aims to clarify key psychological processes of adolescents in palliative care and to support multidisciplinary teams in navigating complex behaviors and clinical decision-making. Approach: The study was grounded in developmental and psychodynamic theory and employed a structured reflective methodology to examine emotional, behavioral, and relational dynamics emerging during clinical management. Clinical observations and practices were integrated with current literature on palliative AYA oncology care. Conceptual Framework: Anticipatory grief (AG) emerges as the primary psychological construct through which adolescents’ experiences in palliative oncology care can be understood. It is associated with fragmentation of future perspectives, identity development, and bodily experiences. These processes are illustrated through clinical manifestations, with clear implications for multidisciplinary team practice. Defense mechanisms, along with transference and countertransference dynamics, are also crucial aspects for professionals to recognize and manage. Understanding these elements allows the health care team to provide better emotional containment, navigate relational complexities, and support the adolescent’s evolving needs. Conclusions: Adolescents in palliative oncology require a distinct psychological framework that goes beyond standard AYA care models. Addressing AG as an organizing process of the adolescent experience calls for greater clinical attention to developmental disruption, relational dynamics, and meaning-making processes. Multidisciplinary teams should integrate reflective and psychologically informed approaches into routine care to improve care quality and clinical decision-making.