Citations

The locally aggressive nature and high recurrence rate of dermatofibrosarcoma protuberans (DFSP) make it challenging to manage, especially when present on the scalp, owing to its ability to invade the underlying periosteum, bone, dura, or brain. Preoperative imaging is used to plan resection, but the true correlation between periosteal or osseous changes on imaging and the presence of tumor within the periosteum is unclear. We present our institutional experience of managing DFSP of the scalp with emphasis on the imaging used. A retrospective review of 33 patients with DFSP of the scalp treated at a tertiary center was conducted. Data on demographics, tumor characteristics, preoperative imaging (magnetic resonance imaging or computed tomography), and surgical outcomes were analyzed. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of each imaging modality were calculated. Patients underwent surgical resection at a mean age of 37.11 years and had a median follow-up of 28.34 months. Preoperative imaging was available for 26 patients (CT only: 5, MRI only: 12, CT and MRI: 9). Calvarial abnormalities were seen in 3 patients. Treatment included Mohs surgery (66.7%) and wide local excision (33.3%). In 10 patients with positive periosteal margins, bone involvement was managed by burring (7) or craniectomy (3). The sensitivity and PPV of CT for periosteal margin positivity were 25% and 100%, and for MRI were 33% and 100%, respectively. The specificity and NPV were 100% and 50% for CT, and 100% and 66% for MRI. Preoperative CT and MRI have low sensitivity and NPV but high specificity and PPV in determining depth of involvement and are essential to guiding reconstruction in DFSP of the scalp.

Abstract Senescent cells secrete proinflammatory factors known as the senescence-associated secretory phenotype (SASP), contributing to tissue dysfunction and aging. Mitochondrial dysfunction is a key feature of senescence, influencing SASP via mitochondrial DNA (mtDNA) release and cGAS/STING pathway activation. Here, we demonstrate that mitochondrial RNA (mtRNA) also accumulates in the cytosol of senescent cells, activating RNA sensors RIG-I and MDA5, leading to MAVS aggregation and SASP induction. Inhibition of these RNA sensors significantly reduces SASP factors. Furthermore, BAX and BAK plays a key role in mtRNA leakage during senescence, and their deletion diminishes SASP expression in vitro and in a mouse model of Metabolic Dysfunction Associated Steatohepatitis (MASH). These findings highlight mtRNA's role in SASP regulation and its potential as a therapeutic target for mitigating age-related inflammation.

Objective Recalcitrant palatal fistulas in patients with cleft palate history sometimes require free flap reconstruction. This study reviews the literature on described flaps and outcomes. Design A systematic review was conducted per the Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Setting All study designs were included. Non-English articles were excluded. Patients and Participants Patients with a history of cleft palate who underwent free flap reconstruction for a oronasal fistula. Interventions Free tissue transfer for a palatal fistula repair. Main outcomee measures Information regarding defect and flap characteristics were reviewed. Surgical outcomes such as flap loss rates, rates of recurrent fistula formation, and speech outcomes were also obtained. Results Our search returned 894 articles, of which 23 were included. All studies were retrospective case series and reports. A total of 65 patients were described with an average age of 19.3 (range 3–55) years and a median fistula size of 8.00 cm 2 (range 2.54 cm 2 – 24 cm 2 ). The most common flap was the radial forearm flap (n = 37). Nine patients (13.8%) had recurrent fistula formation with surgical revision successful in all cases in which the patient returned to the operating room. There were two partial flap losses and no total flap losses. Speech outcomes showed improvement in 27 patients across 10 studies. Conclusions Palatal fistula repair with free tissue transfer is safe with an acceptable risk profile and low flap loss rate. Early recurrence due to partial flap necrosis and dehiscence are successfully managed with flap readvancement.

Background: Regional analgesia is a critical component of enhanced recovery after surgery pathways for cystectomy, but there is little research comparing different modalities. Transversus abdominis plane block is a well-established procedure used for postoperative regional analgesia, whereas erector spinae plane block is a recently described technique that some contend improves pain control. Objective: We sought to compare these modalities in patients undergoing cystectomy, with primary end points of pain scores and total narcotic utilization measured in morphine milliequivalents. Methods: We retrospectively reviewed 77 consecutive patients who underwent open radical cystectomy. Patients who did not undergo erector spinae plane block or transversus abdominis plane block or who used chronic narcotics were excluded. Pain intensity scores were measured immediately postoperatively and in 24-hour intervals until 72 hours postoperatively. Cumulative narcotic utilization at 72 hours, return to bowel function, and length of stay were measured. Results: Fifty-one patients met inclusion criteria. Seventeen patients underwent erector spinae plane block and 34 transversus abdominis plane block. Erector spinae plane block patients reported lower pain scores immediately postoperatively (P = .046), at 48 hours (P = .019), and 72 hours (P = .019). Erector spinae plane block patients used less narcotics after 72 hours than transversus abdominis plane block patients (median: 62.50 morphine milliequivalents vs 146.25 morphine milliequivalents, P = .008). Conclusions: Compared with transversus abdominis plane block, erector spinae plane block reduced total narcotic utilization by 234% while also reducing subjective patient pain scores. Increased adoption of erector spinae plane block could have major impacts on both clinical outcomes and patient satisfaction in patients undergoing radical cystectomy.

Biliary tract cancers, including cholangiocarcinoma, gallbladder, and ampulla of Vater cancers, rank second among hepatobiliary cancers, known for their poor prognoses. The United States has witnessed a notable increase in intrahepatic cholangiocarcinoma incidence. This study examines the incidence and survival outcomes of biliary tract cancers in Olmsted County, Minnesota from 1976 to 2018. Using data from the Rochester Epidemiology Project (REP), residents aged 20 and above were analyzed across four eras. Incidence rates were calculated and adjusted for age and sex, and temporal trends were assessed using Poisson regression. Intrahepatic cholangiocarcinoma exhibited a significant escalation in incidence rates over time, while gallbladder cancers showed a decline among women. Median survival times for biliary tract cancers notably improved. These findings confirm the rising incidence of intrahepatic cholangiocarcinoma and suggest improving survival rates. Nevertheless, the overall prognosis for biliary tract cancers remains very poor, emphasizing the continual need for enhanced management strategies and further research.

Background: Studies that assess cognition prospectively and study in detail anxiety history in the participants’ medical records within the context of brain aging and Alzheimer’s disease are limited. Objective: To examine the associations of anxiety and unspecified emotional distress (UED) acquired throughout a person’s life with prospectively collected cognitive outcomes. Methods: Mayo Clinic Study of Aging participants who were cognitively unimpaired at baseline were included. Anxiety and UED data were abstracted from the medical record using the Rochester Epidemiology Project (REP) resources and were run separately as predictors in our models. The data were analyzed using Cox proportional hazards models for the outcomes of incident mild cognitive impairment (MCI) and dementia and using linear mixed effects models for the outcomes of global and domain specific cognitive z-scores and included key covariates. Results: The study sample ( n = 1,808) had a mean (standard deviation) age of 74.5 (7.3) years and 51.4% were male. Anxiety was associated with increased risk of MCI and dementia and was associated with lower baseline cognitive z-scores and accelerated decline over time in the global, memory, and attention domains. UED was associated with faster decline in all domains except visuospatial but did not show evidence of association with incident cognitive outcomes. These results varied by medication use and timing of anxiety. Conclusions: Anxiety and UED both showed inverse associations with cognition. Utilization of anxiety and UED data from across the life course, as available, from the REP system adds robustness to our results.

BACKGROUND Among the spectrum of adipose lesions associated with peripheral nerves, complete circumferential encasement of the nerve by the lesion is a very rare entity. The authors report the first case of a giant lipoma that completely and circumferentially encased the radial nerve. OBSERVATIONS A 57-year-old woman presented with a large left-arm mass that she had noticed over a year following significant weight loss. She had hypersensitivity and dysesthesias in the left posterior arm but no weakness or other symptoms. Magnetic resonance imaging of the left upper limb demonstrated a large lipoma completely encasing the radial nerve, originating at the level of the spiral grove proximally and extending distally to the lateral epicondyle. The lesion was completely resected at surgery with total preservation of radial nerve function. LESSONS Lipomas encasing peripheral nerves can be difficult to manage while preserving function. This case adds to the intriguing spectrum of adipose lesions associated with peripheral nerves. https://thejns.org/doi/10.3171/CASE24148

Abstract Rationale Dysanapsis refers to a mismatch between airway tree caliber and lung size arising early in life. Dysanapsis assessed by computed tomography (CT) is evident by early adulthood and associated with chronic obstructive pulmonary disease (COPD) risk later in life. Objectives By examining the genetic factors associated with CT-assessed dysanapsis, we aimed to elucidate its molecular underpinnings and physiological significance across the lifespan. Methods We performed a genome-wide association study of CT-assessed dysanapsis in 11,951 adults, including individuals from two population-based and two COPD-enriched studies. We applied colocalization analysis to integrate genome-wide association study and gene expression data from whole blood and lung. Genetic variants associated with dysanapsis were combined into a genetic risk score that was applied to examine association with lung function in children from a population-based birth cohort (n = 1,278) and adults from the UKBiobank (n = 369,157). Measurements and Main Results CT-assessed dysanapsis was associated with genetic variants from 21 independent signals in 19 gene regions, implicating HHIP (hedgehog interacting protein), DSP, and NPNT as potential molecular targets based on colocalization of their expression. A higher dysanapsis genetic risk score was associated with obstructive spirometry among 5-year-old children and among adults in the fifth, sixth, and seventh decades of life. Conclusions CT-assessed dysanapsis is associated with variation in genes previously implicated in lung development, and dysanapsis genetic risk is associated with obstructive lung function from early life through older adulthood. Dysanapsis may represent an endophenotype link between the genetic variations associated with lung function and COPD.