BackgroundJuvenile xanthogranuloma (JXG) is a non‐Langerhans cell histiocytosis characterized by yellowish papules in the skin. JXGs most often occur in infancy or early childhood and are typically solitary and asymptomatic, often regressing after several years. While JXGs predominantly occur on the skin, extracutaneous JXGs also exist.AimsIn this paper, we review the literature on single, multiple, and visceral JXGs and provide recommendations on monitoring and work‐up.Materials & MethodsA literature review was conducted with the PubMed database using selective search terms for single, multiple, ocular, and visceral lesions as well as NF1/JMML.Results / DiscussionJXG is typically a self‐limited disorder if lesions are cutaneous and singular. While rare, JXGs may manifest as multiple and extracutaneous lesions. Further screening and referral to specialists may be warranted in these cases based on age and extent of involvement.ConclusionOur review demonstrates common presentations of single, multiple, and extracutaneous lesions in addition to those that occur with NF1 and JMML. We suggest patients be evaluated on a case‐by‐case basis by a dermatologist and referred to specialists as appropriate.
9566 Background: Merkel cell carcinoma (MCC) is an aggressive skin cancer with a recurrence rate of 40%. Early detection of recurrence can improve outcomes, and effective surveillance is crucial for management of patients with MCC. While Merkel cell polyomavirus (MCPyV) oncoprotein serology is useful in surveillance for MCPyV-positive MCC tumors, patients with MCPyV-negative tumors have no available blood biomarkers and require frequent imaging. This prospective, multicenter study assessed whether circulating tumor DNA (ctDNA) can assess disease burden and detect recurrence regardless of virus status. Methods: A total of 328 blood samples were collected from 125 patients at various time points with a median follow-up of 6 months (range: 0-21 months) between April 2020 to January 2022. Whole-exome sequencing was performed on tumor tissue and matched normal blood to identify a set of somatic, clonal single nucleotide variants, which were tracked in subsequent blood (plasma) samples using a personalized and multiplex PCR-NGS based ctDNA assay (Signatera). Clinically evident disease was defined as MCC noted either by physical exam or by imaging, and molecular evidence of disease was defined as a positive ctDNA test. Surveillance phase began once there was no clinically evident or molecular evidence of disease. Results: Among 125 patients, 47 (38%) had clinically evident MCC and all were found to be ctDNA-positive at the first time point (sensitivity: 100%; 95% CI: 91-100%). Of the 47, 24 were newly diagnosed with MCC and had a median primary tumor size of 2.2 cm (range 0.5-8.5 cm) and a median ctDNA value of 26 mean tumor molecules (MTM)/mL (range: 0.08-1470 MTM/mL). Primary tumor diameter and ctDNA value were strongly correlated (Spearman’s r = 0.81, p < 0.001). Of the 125 patients, 73 (58%) patients were assessed in the surveillance setting and had a total of 152 plasma samples available for longitudinal ctDNA testing. Over this period, 7 ctDNA tests were positive while 145 were negative. After a positive test, 5/7 developed a clinically evident recurrence (4 within 60 days). Of the remaining 2 without clinical recurrence, one had < 60 days of follow-up at time of data analysis. The estimated risk of recurrence, accounting for incomplete follow-up, was 57% within 60 days of a positive ctDNA test (n = 7 tests). In contrast, after a negative ctDNA test (n = 145 tests), the risk of recurrence was 0% within 60 days and 3% between 60-90 days. Conclusions: To our knowledge, this is the largest study to explore ctDNA testing in MCC patients. This study demonstrates that ctDNA testing can detect MCC recurrence early and is a promising clinical surveillance tool regardless of tumor viral status.
AIM:To evaluate whether intra-procedural conebeam computed tomography (CBCT) performed during modified balloon-occluded retrograde transvenous obliteration (mBRTO) can accurately determine technical success of complete variceal obliteration.
METHODS:From June 2012 to December 2014, 15 patients who received CBCT during mBRTO for treatment of portal hypertensive gastric variceal bleeding were retrospectively evaluated. Three-dimensional (3D) CBCT images were performed and evaluated prior to the end of the procedure, and these were further analyzed and compared to the pre-procedure contrast-enhanced computed tomography to determine the technical success of mBRTO including: Complete occlusion/obliteration of: (1) gastrorenal shunt (GRS); (2) gastric varices; and (3) Core tip: This is a retrospective study to evaluate the feasibility, usefulness and efficacy of cone-beam computed tomography (CBCT) in modified balloonoccluded retrograde transvenous obliteration (mBRTO) procedures including coil-assisted retrograde transvenous obliteration and plug-assisted retrograde transvenous oblitearation. With an intra-procedural three-dimensional CBCT, the technical success of mBRTO was determined prior to the completion of the procedure in 100% of cases. With CBCT, a complete anatomy of gastric varices including gastrorenal shunt, gastric varices, collaterals, afferent and efferent vessels were identified easily. The CBCT may improve the technical and clinical success of mBRTO procedures and potentially reduce additional procedure time and cost.Lee EW, So N, Chapman R, McWilliams JP, Loh CT, Busuttil RW, Kee ST. Usefulness of intra-procedural cone-beam computed tomography in modified balloon-occluded retrograde transvenous obliteration of gastric varices.
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