Alexandra E. Richards

Background/Introduction: Standardized staging imaging for Langerhans Cell Histiocytosis (LCH) is lacking. This study aims to identify patient and disease factors that influence imaging selection and propose a clinical decision algorithm that supports more deliberate, radiation- and cost-conscious use of imaging. Methods: A retrospective review of patients with biopsy-proven LCH, evaluated at a single institution, was conducted from 2001 to 2025. Age, sex, and presenting location at diagnosis were compared across imaging groups (Positron emission tomography/computed tomography [PET/CT], bone scintigraphy, and skeletal survey). Disease extent (unifocal, multifocal, multisystem) was summarized according to the imaging modality each patient received. Results: The cohort included 78 patients: 30 PET/CT, 11 bone scans, and 37 skeletal surveys. Median age differed significantly by modality: PET/CT 34 years (interquartile range [IQR] 8–56) vs. bone scan 13 years (3–37) vs. skeletal survey 7 years (2–14) (p < 0.001). Imaging selection varied significantly by anatomic location (p = 0.016): bone lesions were assessed with skeletal survey (52.8%), PET/CT (28.3%), and bone scan (18.9%). All pulmonary cases received PET/CT imaging. Six of the 78 patients had multisystem disease, and all six had been staged with PET/CT (Fisher’s exact p = 0.006, PET/CT vs. skeletal survey; Bonferroni-adjusted p = 0.018). Conclusions: In addition to age, the initial presentation site influences the choice of imaging modality. Given the higher radiation burden and cost of PET/CT, further research is needed to determine whether this observed practice pattern translates to improved clinical outcomes.

Background: Epstein-Barr virus (EBV)-associated lymphoproliferative disorders (LPDs) present unique challenges in immunocompromised patients. Neurotoxic complications typically arise after prolonged exposure and tend to manifest as peripheral neuropathy. This case describes acute encephalopathy shortly after a brief course, an uncommon and severe adverse effect. Case Report: We describe a 17-year-old Hispanic male with EBV-associated LPD in the setting of complex autoimmune vasculitis and immunosuppression. Disease was unresponsive to multiple therapies, but eventually responded to a course of SMILE (steroid-dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide). Brentuximab vedotin maintenance was initiated to secure remission. However, 7 days post-initiation, he developed culture-negative sepsis which progressed to acute encephalopathy and multi-organ failure. Infectious work-up along with neurological evaluation, including imaging and cerebrospinal fluid analysis, did not identify an alternative etiology. Conclusion: This case highlights a rare instance of early brentuximab vedotin-induced encephalopathy and underscores the need for close neurological monitoring in high-risk patients.

Background: Langerhans cell histiocytosis (LCH) is a rare malignancy driven by MAPK pathway activation and often involves BRAF V600E mutations. Targeted therapy with trametinib, a MEK inhibitor, is a promising alternative to conventional chemotherapy.Procedure: We retrospectively analyzed the records of patients treated with trametinib either at relapse or as front‐line therapy between 2020 and 2024.Results: Fifteen pediatric patients received trametinib either at diagnosis (n = 6), relapse (n = 7), or due to chemotherapy intolerance (n = 2). Molecular testing identified MAPK pathway mutations in 11 patients. The median age at treatment initiation was 5 years (range: 0.1–16.5). Notably, five of these patients started trametinib before the age of 1. The median treatment duration was 2.2 years (range: 0.1–4.7 years). All 15 patients achieved favorable responses without concerns regarding growth and development. The adverse effects included rash (40%) and diarrhea (13%), which were all mild and were managed with temporary dose adjustments. A self‐weaning dosing strategy minimized long‐term exposure while maintaining disease control.Conclusion: Our data suggest that trametinib is a safe and effective therapy for pediatric LCH with broad efficacy and tolerability. However, prospective studies are needed to confirm these findings and refine targeted treatment protocols.

Background After surgery for degenerative cervical spine problems, most patients hope to return to noncompetitive sports and other leisure activities. Limited data are available to counsel patients about return to play (RTP) in non-competitive sports after cervical surgery. Methods Participants had cervical surgery for degenerative diagnoses from April 1, 2007, to April 1, 2018. Demographic data were collected, and participants were asked to complete a survey regarding sports participation before and after cervical surgery. Results Of the 73 participants who responded to the study, the majority (81.1%) were able to return to one or multiple hobby sports after elective spine surgery. RTP rates at 12 months for golf, tennis, and swimming were 67.6%, 31.2%, and 81.6%, respectively. Younger age and lack of preoperative motor deficit were significant predictors of return to swimming after surgery. After surgery, 54.3% of golfers reported similar or improved levels of play. Conclusions After elective cervical spine surgery, the majority of hobby athletes can expect to return to athletics. The majority of golfers returned to play with similar or improved frequency and quality of play compared to preoperative levels. Future prospective studies will further elucidate factors predicting RTP after different types of elective cervical surgeries.

Cerebrovascular neurosurgery is a field where the highs are high and the lows are low. The successful cerebrovascular neurosurgeon gets to save lives and restore neurological function but must also to attend families and patients who are facing stroke and death. Patients generally fall into 2 categories: those who have had hemorrhagic or ischemic strokes, and those who are at risk for stroke but are so far unscathed. Patients in the first group have experienced a catastrophe. The neurosurgeon typically meets the patient and their family in the hospital. Morbidity and mortality within this group is common and can be devastating. The able neurosurgeon must be able to assess the situation and act rapidly to prevent worsening of neurological damage and decide how best to keep the patient from further harm. Those in the second group have often received a diagnosis after medical imaging for an unrelated complaint. While the vascular lesion may be asymptomatic, these patients are fearful and anxious about the possibility of experiencing a stroke. For these patients, neurosurgeons must be able to summarize the available evidence, provide comfort, and recommend the safest treatment option. Sometimes the safest course is not surgery, but instead, reassurance and medical management.

Cognard type V dural fistulas represent a rare and unique entity, and present clinically with myelopathy. They are frequently located around the foramen magnum and are most commonly fed by dural branches of the vertebral and external carotid artery. Detection requires meticulous angiography to define the angioarchitecture of the fistula, which in turn defines the treatment modality. We present the case of a 63 yr old woman, after informed consent was obtained, with prolonged, severe and progressive lower extremity myelopathy for 6 yr, with T2-weighted signal changes in the thoracic spinal cord on magnetic resonance imaging (MRI). Cervical MRI was unremarkable. Following computed tomography (CT), angiography of the spinal canal that revealed tortuous and dilated veins on the thoracic spinal cord surface, catheter angiography was performed. This demonstrated a fistula in relation to a dural branch of left vertebral artery with a characteristic single draining vein coursing caudally to the thoracic level, with delayed outflow suggestive of venous hypertension. Given the fact that the dural feeder was tortuous and relatively small, thus, precluding distal microcatheter access, and with presence of a single accessible draining vein, microsurgical treatment was preferred. Following a midline suboccipital craniotomy in prone position, a limited vertical dural opening was performed. Careful microsurgical arachnoid dissection revealed the arterialized draining vein with the aid of Indocyanine Green angiography. The draining vein was clipped, coagulated, and disconnected. Postoperative recovery was uneventful and the patient is undergoing rehabilitation therapy. Follow-up angiography showed complete elimination of the fistula. Salient teaching points are narrated at conclusion.