IMPORTANCE Merkel cell carcinoma (MCC) often behaves aggressively; however, disease-recurrence data are not captured in national databases, and it is unclear what proportion of patients with MCC experience a recurrence (estimates vary from 27%-77%). Stage-specific recurrence data that includes time from diagnosis would provide more precise prognostic information and contribute to risk-appropriate clinical surveillance. OBJECTIVE To estimate risk of stage-specific MCC recurrence and mortality over time since diagnosis. DESIGN, SETTING, AND PARTICIPANTS This prospective cohort study included 618 patients with MCC who were prospectively enrolled in a Seattle-based data repository between 2003 and 2019. Of these patients, 223 experienced a recurrence of MCC. Data analysis was performed July 2019 to November 2021. MAIN OUTCOMES AND MEASURESStage-specific recurrence and survival, as well as cumulative incidence and Kaplan-Meier analyses. RESULTS Among the 618 patients included in the analysis (median [range] age, 69 years; 227 [37%] female), the 5-year recurrence rate for MCC was 40%. Risk of recurrence in the first year was high (11% for patients with pathologic stage I, 33% for pathologic stage IIA/IIB, 30% for pathologic stage IIIA, 45% for pathologic stage IIIB, and 58% for pathologic stage IV), with 95% of recurrences occurring within the first 3 years. Median follow-up among living patients was 4.3 years. Beyond stage, 4 factors were associated with increased recurrence risk in univariable analyses: immunosuppression (hazard ratio [HR], 2.4; 95% CI, 1.7-3.3; P < .001), male sex (HR, 1.9; 95% CI, 1.4-2.5; P < .001), known primary lesion among patients with clinically detectable nodal disease (HR, 2.3; 95% CI, 1.4-4.0; P = .001), and older age (HR, 1.1; 95% CI, 1.0-1.3; P = .06 for each 10-year increase). Among 187 deaths in the cohort, 121 (65%) were due to MCC. The MCC-specific survival rate was strongly stage dependent (95% at 5 years for patients with pathologic stage I vs 41% for pathologic stage IV). Among patients presenting with stage I to II MCC, a local recurrence (17 arising within/adjacent to the primary tumor scar) did not appreciably diminish survival compared with patients who had no recurrence (85% vs 88% MCC-specific survival at 5 years). CONCLUSIONS AND RELEVANCEIn this cohort study, the MCC recurrence rate (approximately 40%) was notably different than that reported for invasive melanoma (approximately 19%), squamous cell carcinoma (approximately 5%-9%), or basal cell carcinoma (approximately 1%-2%) following definitive therapy. Because more than 90% of MCC recurrences arise within 3 years, it is appropriate to adjust surveillance intensity accordingly. Stage-and time-specific recurrence data can assist in appropriately focusing surveillance resources on patients and time intervals in which recurrence risk is highest.
Background: Retinopathy of prematurity (ROP) can lead to retinal detachment and severe vision loss and is a common cause of childhood blindness. Optical coherence tomography angiography (OCTA) is a noninvasive imaging modality that can be used to detect potential abnormalities in the microvasculature in this population. The objective of this study is to assess the feasibility of a newly developed handheld swept source OCT (SS-OCT) device to successfully acquire structural vitreoretinal and retinal microvascular images in awake premature infants.Methods: OCT and OCTA images were acquired at the time of routine ROP examinations from awake, unsedated preterm infants in the Neonatal Intensive Care Unit using a clinical research prototype handheld probe integrated with an SS-OCT system working at 1,060 nm wavelength and an imaging speed of 200,000 A-scans per second (200 kHz), enabling volume OCT and OCTA scans. Each volume was acquired with approximately 36˚ field of view (~6.3×6.3 mm in infants) in 4.8 s. Quality of acquired OCT and OCTA volume images, microvascular information, and vitreoretinal features were determined by 3-masked grader consensus.Results: Twelve infants (5 females, mean gestational age 28.3 weeks, median birth weight 901 g, stages 0 to 3 ROP) underwent a total of 73 individual eye imaging sessions. High-quality OCT images of the fovea and the optic nerve were present in 69/73 (94.5%) and 56/73 (76.7%) scans, respectively. Vitreous bands were observed in 10/73 (13.7%); punctate hyperreflective vitreous opacities in 47/73 (64.4%); epiretinal membrane (ERM) in 6/73 (8.2%); and cystoid macular edema (CME) in 12/73 (16.4%) scans. Mild vessel elevation was noted in 3/73 (4.1%) images, and severe vessel elevation in 4/73 (5.5%) scans. OCTA images obtained in 8 awake infants revealed good quality images of the foveal microvasculature in 11/19 (58%) eye imaging sessions for 6/8 (75%) infants; and peripapillary microvasculature in 14/19 (74%) eye imaging sessions for 5/8 (63%) infants. Conclusions:The SS-OCTA handheld device can capture important vitreoretinal characteristics such as peripapillary and foveal microvasculature, as well as hyperreflective punctate vitreous opacities and tractional vitreous bands, which may predict ROP severity. These images were captured in awake, premature infants without the use of direct ocular contact, an eyelid speculum, or sedation.
Merkel cell carcinoma (MCC) is an aggressive skin cancer associated with a high risk of local recurrence and distant metastasis. Optimal care of this potentially life-threatening cancer is critical but challenging because: physicians are often unfamiliar with its management due to rarity, and MCC management remains controversial, in part because it is rapidly evolving across multiple specialties. While guidelines offer a broad overview of management, they are often not sufficient when making decisions for individual patients. Herein, we present a literature review as well as practical approaches adopted at our institutions for staging, surveillance and therapy of MCC. Each of these areas are discussed in light of how they can be appropriately customized for prevalent but challenging situations. We also provide representative examples of MCC patient scenarios and how they were managed by a multidisciplinary team to identify suitable evidence-based, individualized treatment plans.
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