PURPOSE:Measurement of mediastinal lymph nodes (LNs) is an integral part of patient assessment, and is performed by manually measuring the short axis length (SAL) of the LNs on axial slices. LNs with SAL ≥10 mm are considered pathologically enlarged. We aimed to quantify the interobserver agreement and variability of SAL measurements, compare them to automatically computed SALs from manual LN delineations, and establish the mean SAL measurement error. MATERIALS AND METHODS:Two radiologists independently measured the SALs of 451 LNs in 40 contrast-enhanced chest CT (CECT) scans. One of them also manually delineated the LN contours in each CECT slice, and this served to automatically classify LN as normal/enlarged based on their SALs. Differences between SAL measurements and Bland-Altman statistics were computed. RESULTS:The normal/enlarged LN overall agreement (371 normal, 52 enlarged) between both radiologists was 93.8% (423/451). For agreement/disagreement, the SAL differences were 1.1 (1.0) mm (17%) and 3.5 (3.2) mm (40%). The disagreement differences were nearly twice as large as the agreement differences. The agreement between the manual and the computed SALs for both radiologists was 92.7% (418/451), similar to the interobserver variability. CONCLUSION:Classification of mediastinal lymph nodes based on SAL measurements demonstrates high agreement. It indicates that SAL measurements automatically computed from manual LN delineations could be a reliable and time-saving tool. In cases of disagreement, the ±2 mm error supports the use of 3 size categories: normal (<8 mm), possibly enlarged (8 to 12 mm), and definitely enlarged (>12 mm).

Background u2014Synovial sarcoma is a rare soft tissue sarcoma. Treatment of synovial sarcoma includes surgery, radiation, pazopanib, and chemotherapy. Targeted therapies, such as B-Raf proto-oncogene, serine/threonine kinase (BRAF) inhibitors, are emerging as a potential treatment option. We describe the sixth case of a BRAFV600E synovial sarcoma, the first extra-thoracic case. This case is the first to show a complete pathological response to BRAF u0026amp; mitogen-activated protein kinase kinase (MEK) inhibitors. Case description u2014We treated a 22-year-old male with a left groin BRAFV600E synovial sarcoma with doxorubicin, Ifosphamide u0026amp; Sodium 2-Mercaptoethanesulfonate. When we identified BRAFV600E in the tumor, the BRAFV600E and MEK inhibitors (dabrafenib u0026amp; trametinib) were initiated, followed by surgery, with a complete pathological response. Nine months after the surgery, a local recurrence prompted the resumption of dabrafenib u0026amp; trametinib followed by radiotherapy, resulting in complete radiological response and the development of hemophagocytic lymphohistiocytosis treated with corticosteroids with resolution of symptoms. Conclusion u2014Panel sequencing of synovial sarcoma can identify targetable mutations. Treatment of BRAFV600E synovial sarcoma with dabrafenib u0026amp; trametinib can lead to complete pathological response and prolonged radiological response, as well as the rare adverse event of hemophagocytic lymphohistiocytosis. Prospective clinical trials are needed to evaluate the efficacy and safety of BRAFV600E u0026amp; MEK inhibitors as a therapeutic approach in BRAFV600E synovial sarcoma.

Background:-Synovial sarcoma is a rare soft tissue sarcoma. Treatment of synovial sarcoma includes surgery, radiation, pazopanib, and chemotherapy. Targeted therapies, such as B-Raf proto-oncogene, serine/threonine kinase (BRAF) inhibitors, are emerging as a potential treatment option. We describe the sixth case of a BRAFV600E synovial sarcoma, the first extra-thoracic case. This case is the first to show a complete pathological response to BRAF & mitogen-activated protein kinase kinase (MEK) inhibitors. Case description:-We treated a 22-year-old male with a left groin BRAFV600E synovial sarcoma with doxorubicin, Ifosphamide & Sodium 2-Mercaptoethanesulfonate. When we identified BRAFV600E in the tumor, the BRAFV600E and MEK inhibitors (dabrafenib & trametinib) were initiated, followed by surgery, with a complete pathological response. Nine months after the surgery, a local recurrence prompted the resumption of dabrafenib & trametinib followed by radiotherapy, resulting in complete radiological response and the development of hemophagocytic lymphohistiocytosis treated with corticosteroids with resolution of symptoms. Conclusion:-Panel sequencing of synovial sarcoma can identify targetable mutations. Treatment of BRAFV600E synovial sarcoma with dabrafenib & trametinib can lead to complete pathological response and prolonged radiological response, as well as the rare adverse event of hemophagocytic lymphohistiocytosis. Prospective clinical trials are needed to evaluate the efficacy and safety of BRAFV600E & MEK inhibitors as a therapeutic approach in BRAFV600E synovial sarcoma.

Manual detection and measurement of structures in volumetric scans is routine in clinical practice but is time-consuming and subject to observer variability. Automatic deep learning-based solutions are effective but require a large dataset of manual annotations by experts. We present a novel annotation-efficient semi-supervised deep learning method for automatic detection, segmentation, and measurement of the short axis length (SAL) of mediastinal lymph nodes (LNs) in contrast-enhanced CT (ceCT) scans. Our semi-supervised method combines the precision of expert annotations with the quantity advantages of pseudolabeled data. It uses an ensemble of 3D nnU-Net models trained on a few expert-annotated scans to generate pseudolabels on a large dataset of unannotated scans. The pseudolabels are then filtered to remove false positive LNs by excluding LNs outside the mediastinum and LNs overlapping with other anatomical structures. Finally, a single 3D nnU-Net model is trained using the filtered pseudo-labels. Our method optimizes the ratio of annotated/non-annotated dataset sizes to achieve the desired performance, thus reducing manual annotation effort. Experimental studies on three chest ceCT datasets with a total of 268 annotated scans (1817 LNs), of which 134 scans were used for testing and the remaining for ensemble training in batches of 17, 34, 67, and 134 scans, as well as 710 unannotated scans, show that the semi-supervised models’ recall improvements were 11–24

Measurement of mediastinal lymph nodes (LNs) is an integral part of patient assessment, and is performed by manually measuring the short axis length (SAL) of the LNs on axial slices. LNs with SAL ≥10 mm are considered pathologically enlarged. We aimed to quantify the interobserver agreement and variability of SAL measurements, compare them to automatically computed SALs from manual LN delineations, and establish the mean SAL measurement error. Two radiologists independently measured the SALs of 451 LNs in 40 contrast-enhanced chest CT (CECT) scans. One of them also manually delineated the LN contours in each CECT slice, and this served to automatically classify LN as normal/enlarged based on their SALs. Differences between SAL measurements and Bland-Altman statistics were computed. The normal/enlarged LN overall agreement (371 normal, 52 enlarged) between both radiologists was 93.8% (423/451). For agreement/disagreement, the SAL differences were 1.1 (1.0) mm (17%) and 3.5 (3.2) mm (40%). The disagreement differences were nearly twice as large as the agreement differences. The agreement between the manual and the computed SALs for both radiologists was 92.7% (418/451), similar to the interobserver variability. Classification of mediastinal lymph nodes based on SAL measurements demonstrates high agreement. It indicates that SAL measurements automatically computed from manual LN delineations could be a reliable and time-saving tool. In cases of disagreement, the ±2 mm error supports the use of 3 size categories: normal (<8 mm), possibly enlarged (8 to 12 mm), and definitely enlarged (>12 mm).

CASES:We present 2 cases of median nerve reconstruction using distal nerve transfers after resection of unusual benign median nerve tumors. Critical sensation was restored in case 1 by transferring the fourth common digital nerve to first web digital nerves. Thumb opposition was regained by transferring the abductor digiti minimi ulnar motor nerve branch to the recurrent median motor nerve branch. Critical sensation was restored in case 2 by transferring the long finger ulnar digital nerve to the index finger radial digital nerve. CONCLUSION:Distal nerve transfers, even with short grafts, are reliable median nerve deficit treatments, sparing the need for larger autologous nerve grafts and late tendon opponensplasties.

A 25 yearold male patient presented with a 2week history of inflammatory back pain and right heel ache. He was known to suffer from undifferentiated spondyloarthritis, with lumbar spondylitis and peripheral enthesitis. He was off therapy, but had been previously treated with biologics. Physical examination revealed a negative Patrick's test, tenderness at the insertion of the right Achilles tendon to the calcaneus, and tenderness upon palpation of the lumbar spinous processes. Ultrasound showed signs of peripheral enthesitis, and magnetic resonance imaging (MRI) scan displayed active lumbar interspinous bursitis (Figure 1A, arrows), with no signs of sacroiliitis. No close approximation of the spinous processes was present, ruling out Baastrup disease. Known erosions were also seen at 2 lumbar vertebral bodies, L1 and L5 (Figure 1B, arrows). The concomitant interspinous bursitis and peripheral enthesitis were considered signs of active spondyloarthritis. Treatment with etoricoxib was started, with a remarkable clinical improvement. The renewal of targeted therapy was discussed with the patient. Active spondyloarthritis may present as bursitis.1 In fact, bursal tissue has been suggested to contribute to the enthesis complex,2

To compare liver metastases changes in CT assessed by radiologists using RECIST 1.1 and with aided simultaneous deep learning–based volumetric lesion changes analysis. A total of 86 abdominal CT studies from 43 patients (prior and current scans) of abdominal CT scans of patients with 1041 liver metastases (mean = 12.1, std = 11.9, range 1–49) were analyzed. Two radiologists performed readings of all pairs; conventional with RECIST 1.1 and with computer-aided assessment. For computer-aided reading, we used a novel simultaneous multi-channel 3D R2U-Net classifier trained and validated on other scans. The reference was established by having an expert radiologist validate the computed lesion detection and segmentation. The results were then verified and modified as needed by another independent radiologist. The primary outcome measure was the disease status assessment with the conventional and the computer-aided readings with respect to the reference. For conventional and computer-aided reading, there was a difference in disease status classification in 40 out of 86 (46.51 • Classification of liver metastasis changes improved significantly in one-third of the cases with an automatically generated comprehensive lesion and lesion changes report. • Simultaneous deep learning changes detection and volumetric assessment may improve the evaluation of liver metastases temporal changes potentially improving disease management.

Sarcoma classification is challenging and can lead to treatment delays. Previous studies used DNA aberrations and machine-learning classifiers based on methylation profiles for diagnosis. We aimed to classify sarcomas by analyzing methylation signatures obtained from low-coverage whole-genome sequencing, which also identifies copy-number alterations. DNA was extracted from 23 suspected sarcoma samples and sequenced on an Oxford Nanopore sequencer. The methylation-based classifier, applied in the nanoDx pipeline, was customized using a reference set based on processed Illumina-based methylation data. Classification analysis utilized the Random Forest algorithm and t-distributed stochastic neighbor embedding, while copy-number alterations were detected using a designated R package. Out of the 23 samples encompassing a restricted range of sarcoma types, 20 were successfully sequenced, but two did not contain tumor tissue, according to the pathologist. Among the 18 tumor samples, 14 were classified as reported in the pathology results. Four classifications were discordant with the pathological report, with one compatible and three showing discrepancies. Improving tissue handling, DNA extraction methods, and detecting point mutations and translocations could enhance accuracy. We envision that rapid, accurate, point-of-care sarcoma classification using nanopore sequencing could be achieved through additional validation in a diverse tumor cohort and the integration of methylation-based classification and other DNA aberrations.

CASE:A 7-year-old boy presented with osteosarcoma of the ulna that required a transhumeral amputation. The patient completed neoadjuvant chemotherapy before surgery. To prevent bone overgrowth and improved prosthetic fitting, a modified amputation was performed with acute shortening and distal epiphysis preservation. CONCLUSION:In this modified amputation, bone overgrowth was not seen and repeated surgeries for stump shaping were prevented. The modified technique enabled length for the prosthetic elbow and a wide stump for better fitting. In cases of transhumeral amputation in children, the modified technique should be considered.

The patient, then a 35-year-old female, presented with an 18-month history of bilateral hip and groin pain, limiting her ability to ambulate. The pain appeared upon rest and exertion, and responded poorly to simple analgesics. No other systemic complaints were present.

A 19-year-old male patient presented with a 2-week history of right hip and buttock aches. His past medical record was unremarkable, except for severe diffuse acne, for which he was recently started on isotretinoin. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) scans displayed changes consistent with active sacroiliitis. While therapy with non-steroidal anti-inflammatory drugs achieved no benefit, prednisone led to remarkable recovery. The acute presentation, the severe acne, the temporal association with the use of isotretinoin, and the response to corticosteroids are compatible with the diagnosis of acne fulminans. Abbreviations: CT: Computed Tomography; MRI: Magnetic Resonance Imaging.

Arthritis & RheumatologyVolume 73, Issue 5 p. 902-902 Clinical Image An Unusual Presentation of Erosive Gout Fadi Kharouf, Corresponding Author Fadi Kharouf fadikharouf@hotmail.com orcid.org/0000-0002-3540-0341 Hebrew University, Hadassah Medical CenterSearch for more papers by this authorYusef Azraq, Yusef Azraq Hebrew University, Hadassah Medical CenterSearch for more papers by this authorYaakov Applbaum, Yaakov Applbaum Hebrew University and Shaare Zedek Medical CenterSearch for more papers by this authorHagit Peleg, Hagit Peleg Hebrew University, Hadassah Medical Center, Jerusalem, IsraelSearch for more papers by this author Fadi Kharouf, Corresponding Author Fadi Kharouf fadikharouf@hotmail.com orcid.org/0000-0002-3540-0341 Hebrew University, Hadassah Medical CenterSearch for more papers by this authorYusef Azraq, Yusef Azraq Hebrew University, Hadassah Medical CenterSearch for more papers by this authorYaakov Applbaum, Yaakov Applbaum Hebrew University and Shaare Zedek Medical CenterSearch for more papers by this authorHagit Peleg, Hagit Peleg Hebrew University, Hadassah Medical Center, Jerusalem, IsraelSearch for more papers by this author First published: 10 January 2021 https://doi.org/10.1002/art.41641Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume73, Issue5May 2021Pages 902-902 RelatedInformation

Aneurysmal bone cyst (ABC) is a benign locally destructive lesion that usually developed in the bone cavity of bones, less commonly on the surface of cortical bones and very rarely develop outside the bone. There are only 35 reports of extraskeletal aneurysmal bone cyst (ESABC) in the English literature. We report a case of a 12-year-old female with no history of trauma who presented with knee pain. Imaging studies revealed an infrapatellar mass that was fast to calcify during a period of 3 months. MRI showed high T2 center, low T1 signal, and heterogenic enhancement with a rim of low intensity consistent with calcified boarders surrounded by severe soft tissue edema. The lesion was surgically excised, and a histological examination revealed an aneurysmal bone cyst possibly arising within myositis ossificans or heterotopic ossification. In her last follow-up 1.5 years after the surgical excision, the patient was symptom free and without signs of recurrence. To the best of our knowledge, this is the first reported case of an intra-articular ESABC located in the knee.

Early diagnosis of sacroiliitis may lead to preventive treatment which can significantly improve the patient's quality of life in the long run. Oftentimes, a CT scan of the lower back or abdomen is acquired for suspected back pain. However, since the differences between a healthy and an inflamed sacroiliac joint in the early stages are subtle, the condition may be missed. We have developed a new automatic algorithm for the diagnosis and grading of sacroiliitis CT scans as incidental findings, for patients who underwent CT scanning as part of their lower back pain workout. The method is based on supervised machine and deep learning techniques. The input is a CT scan that includes the patient's pelvis. The output is a diagnosis for each sacroiliac joint. The algorithm consists of four steps: (1) computation of an initial region of interest (ROI) that includes the pelvic joints region using heuristics and a U-Net classifier; (2) refinement of the ROI to detect both sacroiliiac joints using a four-tree random forest; (3) individual sacroiliitis grading of each sacroiliiac joint in each CT slice with a custom slice CNN classifier, and; (4) sacroiliitis diagnosis and grading by combining the individual slice grades using a random forest. Experimental results on 484 sacroiliiac joints yield a binary and a 3-class case classification accuracy of 91.9% and 86%, a sensitivity of 95% and 82%, and an Area-Under-the-Curve of 0.97 and 0.57, respectively. Automatic computer-based analysis of CT scans has the potential of being a useful method for the diagnosis and grading of sacroiliitis as an incidental finding.

Background The presence of structural sacroiliitis is strong evidence for the diagnosis of spondyloarthritis (SpA). Purpose To assess the performance of abdominal computed tomography (CT) and pelvic plain radiography for the diagnosis of structural sacroiliitis compared with sacroiliac CT (SI joint CT) considered the reference technique in patients with SpA. Material and Methods All SpA patients eligible for biologic treatment were selected from 2005 to 2012. An assessment of sacroiliitis was based on radiography according to the modified New York criteria and on abdominal CT and SI joint CT scans depending on the presence of erosion on at least two consecutive slices. A senior rheumatologist and radiologist independently scored the grade and diagnosis of structural sacroiliitis for the three imaging modalities. After a consensus reading of conflicting examinations (radiography and CT), a final diagnosis of structural sacroiliitis was attained. Results Of the 72 patients selected, sacroiliitis was diagnosed on radiography, abdominal CT, and SI joint CT in 40, 31, and 44 patients, respectively. Inter-reader agreements for the grade of sacroiliitis were substantial for the three imaging modalities, with a weighted kappa range of 0.63–0.75 (95% confidence interval [CI], 0.52–0.83), and they were moderate for the diagnosis of sacroiliitis, with a kappa range of 0.50–0.55 (95% CI, 0.32–0.74). The sensitivity and specificity were 79.1% and 70.5%, respectively, for radiography and 82.1% and 100%, respectively, for abdominal CT. Conclusion This study demonstrates the relevance of abdominal CT for the diagnosis of structural sacroiliitis, with good sensitivity and excellent specificity. These imaging techniques avoid unnecessary examinations.

e22538 Background: Pazopanib, (P) a multikinase inhibitor targeting the VEGF and PDGF has been the first targeted therapy to supplement the arsenal of chemotherapies in soft tissue sarcoma (STS). With a manageable toxicity profile comprised mainly of fatigue, diarrhea, HTN P is capable of controlling STS for several months before resistance develops and chemotherapy is resumed. Preclinical data showed that mTOR inhibition indirectly affects angiogenesis. It affects endothelial cell proliferation and tumor cell production of VEGF via its downstream effect on the hypoxia inducible factor 1. Sirolimus (S) is an oral mTOR inhibitor. We report on a series of 8 metastatic STS patients for whom P treatment was supplemented by S, after escape from P alone. Methods: Second line and beyond metastatic STS patients, who progressed on P after a response duration of at least 4 months were re-challenged with combination of P 600mg and S 3-4mg, in two medical centers; Hadassah Medical Center and Tel Aviv Medical Center. Patients were evaluated for response after 6 w of treatment; patients with response or SD continued treatment until DP. Results: Eight patients (5 females) (1 ULMS, 2 SFT, 2 UPS, 1 LMS, 1 DSRCT, 1 undifferentiated uterine sarcoma (UUS)) between the ages of 36-74 received the combination (P+S) in 2nd lines and beyond, following 4-28m of P alone. Four patients, 3 and 1 had PD (2 SFT+1 UPS+UUS), SD and PR (UPS) as best response, respectively. Treatment duration on the combination ranges between 5m to not-reached yet. One patient ULMS with SD underwent lung metastasectomy after 8m on the combination her treatment is on-going after 17 m. Except of one case of small bowel perforation, probably related to the efficacy of the combination, surgically treated with complete recovery and one case of hyperglycemia managed with metformin, treatment was very well tolerated without added side effects. Conclusions: Supplementing P with S, has reversed resistance to P in 4 of 8 patients. The combination delays reintroduction of chemotherapy extending the chemo-free window with good quality of life. A study designed to prove this concept is underway.

Background In some countries, patients with spondyloarthritis are eligible to receive a TNF blocker only if they present with radiographic spondyloarthritis (AS). This study is therefore of interest with regard to validation of the use of other imaging modalities, particularly thoracic and pelvic CT scanning, to establish the diagnosis of structural sacroiliitis with the same level of performance as radiography. Objectives To assess the performance of thoraco-abdominal and pelvic CT (TAP-CT) and pelvic plain radiography (RX) for the diagnosis of structural sacroiliitis compared to sacroiliac CT (SIJ-CT) in patients with spondyloarthritis (SpA). Methods All SpA patients eligible for biologic treatment with or without structural sacroiliitis on radiography (ASAS criteria) were selected from 2005 to 2012. The three imaging studies were performed in the same year. Sacroiliitis was assessed on RX according to New York criteria, and in TAP-CT and SIJ-CT scans depended on the presence of erosions on two consecutive slides. A senior rheumatologist and radiologist independently scored the grade and the diagnosis of structural sacroiliitis for the three imaging modalities. After a consensus reading of the conflicting exams (RX and CT), a final diagnosis of structural sacroiliitis was retained. The gold standard for the diagnosis of structural sacroiliitis was SIJ-CT. Results Of the 72 patients selected, sacroiliitis was diagnosed on RX, TAP-CT and SIJ-CT in 40, 31 and 44 respectively. Inter-reader agreements for the grade of sacroiliitis were good for the three imaging modalities with a weighted kappa that varied between 0.63 and 0.75 (IC-95%: 0.52-0.83). Inter-reader agreements for the diagnosis of sacroiliitis were moderate with a kappa that varied between 0.50 and 0.55 (CI-95%: 0.32-0.74). For the RX and the TAP-CT, the sensitivity and the specificity were respectively 79.1%, 70.5%, and 82.1%, 100%. Finally, 4 out of 5 patients were correctly classified on RX or TAP-CT Conclusions This study demonstrates the interest of TAP-CT for the diagnosis of structural sacroiliitis with a good sensitivity and an excellent specificity. This imaging technique systematically performed in various clinical situations would limit the prescription of additional exams to establish the diagnosis of structural sacroiliitis. References Devauchelle-Pensec V, D9Agostino MA, Marion J, Lapierre M, Jousse-Joulin S, Colin D et al. Computed tomography scanning facilitates the diagnosis of sacroiliitis in patients with suspected spondylarthritis: results of a prospective multicenter French cohort study. Arthritis Rheum. 2012 May; 64(5):1412-9. Geijer M, Göthlin GG, Göthlin JH. The clinical utility of computed tomography compared to conventional radiography in diagnosing sacroiliitis. A retrospective study on 910 patients and literature review. J Rheumatol. 2007 Jul; 34(7):1561-5. Slobodin G, Croitoru S, Starikov N, Younis S, Boulman N, Rimar D et al. Incidental computed tomography sacroiliitis: clinical significance and inappropriateness of the New York radiological grading criteria for the diagnosis. Clin Rheumatol. 2012 Mar;31(3):425-8. Disclosure of Interest None declared