BACKGROUND: Marginal zone lymphoma (MZL) is an indolent non-Hodgkin B cell lymphoma with various architectural pattern including perifollicular, follicular colonization, nodular, micronodular, and diffuse patterns. A sclerotic variant has not been previously reported and represents a diagnostic pitfall.
METHODS: A 66-year-old male developed left upper extremity swelling. Chest computed tomography (CT) in September 2020 showed 14 cm mass in left axilla. Needle core biopsy of axillary lymph node showed sclerotic tissue with atypical B lymphoid infiltrate but was non-diagnostic. Excisional biopsy was performed for diagnosis and showed extensive fibrosis and minor component of infiltrating B cells. Flow cytometry showed a small population of CD5-, CD10-, kappa restricted B cells. Monoclonal immunoglobulin heavy chain and light chain gene rearrangement were identified. Upon being diagnosed with MZL, patient was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone and achieved complete remission by positron emission tomography/CT.
CONCLUSIONS: This is an important case report because by morphology this case could have easily been overlooked as non-specific fibrosis with chronic inflammation representing a significant diagnostic pitfall. Moreover, this constitutes a new architectural pattern. While sclerotic lymphomas have rarely been described (often misdiagnosed as retroperitoneal fibrosis), we do not know of any cases describing this architectural presentation of MZL.

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BACKGROUND: UVA1 phototherapy is a treatment used for multiple dermatological conditions. The optimal therapeutic regimens and dosing of UVA1 are a matter of debate. The dosages used vary widely between published studies and there are no evidence-based protocols that provide data on dosage, duration, or the role of maintenance therapy. The purpose of this study is to evaluate the experience in our medical center regarding treatment with UVA1, as well as the degree of patient satisfaction with the treatment according to their pathology.
METHODS: We present a retrospective evaluation of outcomes, treatment tolerability, and satisfaction in adult patients using a low dose of UVA1 phototherapy, administered in our dermatologic service between 2019 and 2022.
RESULTS: A total of 78 patients were treated with UVA1, of whom 46 patients (59%) were over 18 years old, completed treatment, and gave their consent. The overall objective clinical response rate was 91.30% (42/46), achieving a complete response in 17 (36.96%) patients, partial response in 25 (54.34%), and no response in 4 (8.70%). The complete response rates recorded were high in morphea, scleredema, or chronic hand eczema. In terms of the level of satisfaction objectively measured by TSQM-9 version 1.4, highlighting high scores obtained in mastocytosis, systemic sclerosis, morphea, scleredema, chronic hand eczema, or prurigo nodularis (over 65 points).
CONCLUSIONS: We present a review of treatment with UVA1 phototherapy at low doses with good response in a wide variety of dermatological pathologies.

BACKGROUND: Ultraviolet (UV) A1 phototherapy is considered a beneficial treatment for various inflammatory, sclerotic, malignant, and other skin conditions. However, the available data regarding its efficacy for different indications, the potential side effects, and the recommended treatment protocols are sparse.
OBJECTIVE: To assess the efficacy of UVA1 phototherapy and identify correlation between different indications and treatment protocols to response rates.
METHODS: We performed a retrospective study of a cohort of 335 patients treated with UVA1 phototherapy at the Department of Dermatology at Hadassah Medical Center, Jerusalem, Israel, between 2008 and 2018.
RESULTS: The study population included 163 patients with inflammatory diseases (mainly atopic dermatitis and other types of eczema), 67 patients with sclerotic diseases (morphea and graft versus host disease), nine patients with neoplastic diseases (cutaneous T cell lymphoma), and 188 patients with other cutaneous disorders. Response rates ranged between 85% and 89% across indications, without differences in response rates among the indication groups (p = .941). In a multivariant logistic regression model, increased number of treatments and higher maximal dosages were associated with response to treatment (p < .001). Using ROC analysis, a cut-off of 8 UVA1 phototherapy treatments was chosen as predictive for beneficial response (86.4% sensitivity, 78% specificity). A cut-off of 40 J/cm2 was chosen as an optimal maximal dosage for differentiating between responders and non-responders (51.1% sensitivity, 83.1% specificity).
CONCLUSIONS: UVA1 phototherapy is an effective treatment for a variety of skin conditions. In most patients, at least eight treatments of a medium-high dosage are required for clinical response.

Bone metastases are of high clinical relevance because they are a frequent complication of most types of common cancers, such as breast and prostate. The metastatic process is complex, requiring the completion of several different steps to allow successful dissemination and homing. In addition, preparation of the metastatic niche changes the constant cycle of bone matrix formation and degradation, leading to the clinical phenotypes of lytic and sclerotic lesions. We review our current knowledge on this topic and briefly explain the current treatment landscape of bone metastasis.
These include PubMed, international guidelines, and clinician experience.
Bone metastases remain a clinical challenge that negatively impacts patients prognosis and quality of life. A comprehensive understanding of the complex molecular mechanisms that results in bone metastasis is the basis for successful treatment of affected patients. The disruption of bone matrix metabolism is already recognized as the prerequisite for metastasis formation, but many open questions remain that need to be addressed in future research to establish individually tailored treatment approaches.
Patient-centered therapy of bone metastases requires suitable pharmacological options, and importantly a holistic approach in care delivery across the multidisciplinary team. Nurses provide the cornerstone of the multidisciplinary team and provide the closest and the most frequent contact to the patient and their families to provide timely intervention. Nurses require a basic understanding of the complex physiology of metastasis to inform practice.

OBJECTIVE: Accumulating evidence supports the use of antineutrophil cytoplasmic antibody (ANCA) type to classify different clinical entities. We aimed to evaluate whether the presence and type of ANCA determine different diseases, based on clinical phenotypes, renal involvement, and response to treatment.
METHODS: Differences in terms of clinical manifestations, disease activity, laboratory parameters, and histology were recorded between patients with focal necrotizing glomerulonephritis (FNGN) due to myeloperoxidase (MPO-), proteinase 3-ANCA(+) [PR3-ANCA(+)], and ANCA(-) disease at time of diagnosis. Patients were treated with the same protocol and followed-up for 24 months, in a scheduled basis of every month for the first year and every 3 months for the second year. Primary end points were: (i) Combined end-stage renal disease (ESRD) and/or death and (ii) The presence of major or minor relapse during follow-up and secondary endpoint was the combination of ESRD and reduction of estimated glomerular filtration rate (eGFR) ≥ 50%.
RESULTS: A total of 92 patients (M/F 39/53, mean age 59.1 ± 15 years) diagnosed with FNGN due to ANCA-associated vasculitis (AAV), 36 (39.1%) patients diagnosed with PR3-ANCA, 39 (42.4%) patients diagnosed with MPO-ANCA, and 17 (18.5%) patients diagnosed with ANCA(-) were included. Number of involved systems differed significantly between PR3-, MPO-ANCA, and ANCA(-), with only renal involvement in 3, 25.5, and 29% of patients, two systems involved in 33, 31, and 59% of patients, and > 3 systems involved in 64, 43.5, and 12% of patients, respectively (p = 0.002). Histology classification revealed focal, crescentic, mixed, and sclerotic type in 14, 64, 19, and 3% of PR3-ANCA(+), 8, 28, 18, and 46% of MPO-ANCA, and 41, 29, 6, and 24% of ANCA(-), respectively (p < 0.0001). Primary end point of ESRD ± Death was reached in 11 (30.6%), 16 (41%), and 6 (35.5%) patients with PR3-ANCA(+), MPO-ANCA(+), and ANCA(-), respectively (p = NS); similarly, ESRD± > 50% eGFR reduction in 8 (22.2%), 15 (38.5%), and 5 (29.4%) patients, respectively (p = NS), meaning that patients with MPO-ANCA(+) showed a propensity to decline renal function. Rate of relapse was increased in the presence of patients with PR3-ANCA(+), 14 (38.9%), 4 (11.8%), and 2 (10.3%) of patients with PR3-ANCA(+), MPO-ANCA(+), and ANCA(-), had at least one relapse during the two-year follow-up (p = 0.006).
CONCLUSIONS: Clinical phenotype and renal histology differ significantly between PR3-ANCA(+), MPO-ANCA(+), and ANCA(-) disease and FNGN; however, renal function outcome is similar, despite the increased rate of relapses in patients with PR3-ANCA(+).

OBJECTIVE: To develop a deep convolutional neural network capable of detecting spinal sclerotic metastases on body CTs.
METHODS: Our study was IRB-approved and HIPAA-compliant. Cases of confirmed sclerotic bone metastases in chest, abdomen, and pelvis CTs were identified. Images were manually segmented for 3 classes: background, normal bone, and sclerotic lesion(s). If multiple lesions were present on a slice, all lesions were segmented. A total of 600 images were obtained, with a 90/10 training/testing split. Images were stored as 128 × 128 pixel grayscale and the training dataset underwent a processing pipeline of histogram equalization and data augmentation. We trained our model from scratch on Keras/TensorFlow using an 80/20 training/validation split and a U-Net architecture (64 batch size, 100 epochs, dropout 0.25, initial learning rate 0.0001, sigmoid activation). We also tested our model\'s true negative and false positive rate with 1104 non-pathologic images. Global sensitivity measured model detection of any lesion on a single image, local sensitivity and positive predictive value (PPV) measured model detection of each lesion on a given image, and local specificity measured the false positive rate in non-pathologic bone.
RESULTS: Dice scores were 0.83 for lesion, 0.96 for non-pathologic bone, and 0.99 for background. Global sensitivity was 95% (57/60), local sensitivity was 92% (89/97), local PPV was 97% (89/92), and local specificity was 87% (958/1104).
CONCLUSIONS: A deep convolutional neural network has the potential to assist in detecting sclerotic spinal metastases.

BACKGROUND. CT attenuation thresholds that accurately distinguish enostoses from untreated osteoblastic metastases have been published. In the Mayo Clinic practices, these thresholds have been applied more broadly to distinguish benign sclerotic bone lesions other than enostoses from osteoblastic metastases. OBJECTIVE. The purpose of this article is to determine if CT attenuation thresholds allow the distinguishing of benign sclerotic bone lesions from osteoblastic metastases in patients undergoing bone biopsy. METHODS. A retrospective search was conducted to identify sclerotic lesions described on CT between October 7, 1998, and July 15, 2018, that underwent subsequent biopsy. Two musculoskeletal radiologists recorded lesions\' maximum and mean attenuation. Using previously published attenuation thresholds, sensitivity and specificity for differentiating benign sclerotic lesions from osteoblastic metastases were calculated. ROC curve analysis was performed to determine if more appropriate attenuation thresholds exist. Intraclass correlation coefficients (ICCs) were computed. RESULTS. A total of 280 patients met inclusion criteria. Of those, 162 had malignant biopsy results and 118 had benign biopsy results. Of the 162 malignant lesions, 81 had received prior treatment. Maximum and mean attenuation were not significantly different between benign and malignant lesions for either reader (all p > .05). For reader 1, to distinguish benign from malignant lesions, a maximum attenuation threshold of more than 1060 HU resulted in sensitivity of 23.7%, specificity of 87.0%, and accuracy of 60.6%. A mean attenuation threshold of greater than 885 HU resulted in sensitivity of 19.5%, specificity of 90.7%, and accuracy 60.7%. ROC curve analysis showed AUCs for mean and maximum attenuation thresholds of 51.8% and 54.6%, respectively. Subgroup analyses of benign versus malignant and treated versus untreated lesions had similar results. Similar findings were obtained for reader 2. The two readers\' ICC was 0.946 for maximum attenuation and 0.918 for mean attenuation. CONCLUSION. Published attenuation thresholds for distinguishing enostoses from osteoblastic metastases had slightly decreased specificity and markedly decreased sensitivity when applied to the differentiation of benign sclerotic lesions from osteoblastic metastases in our sample of biopsy-proven lesions. ROC analysis showed no high-performing attenuation threshold alternative. CLINICAL IMPACT. Published CT attenuation thresholds intended for distinguishing enostoses from osteoblastic metastases should not be used more broadly. More accurate alternative thresholds could not be derived.

Melorheostosis is a rare dysostosis involving cortical bone overgrowth that affects the appendicular skeleton. Patients present with pain, deformities, contractures, range of motion limitation(s), and limb swelling. It has been described in children as well as adults. We recently identified somatic mosaicism for gain-of-function mutations in MAP2K1 in patients with melorheostosis. Despite these advances in genetic understanding, there are no effective therapies or clinical guidelines to help clinicians and patients in disease management. In a study to better characterize the clinical and genetic aspects of the disease, we recruited 30 adults with a radiographic appearance of melorheostosis and corresponding increased uptake on 18F-NaF positron emission tomography (PET)/CT. Patients underwent physical exam, imaging studies, and laboratory assessment. All patients underwent nerve conduction studies and ultrasound imaging of the nerve in the anatomic distribution of melorheostosis. We found sensory deficits in approximately 77% of patients, with evidence of focal nerve entrapment in five patients. All patients reported pain; 53% of patients had changes in skin overlying the affected bone. No significant laboratory abnormalities were noted. Our findings suggest that patients with melorheostosis may benefit from a multidisciplinary team of dermatologists, neurologists, orthopedic surgeons, pain and palliative care specialists, and physical medicine and rehabilitation specialists. Future studies focused on disease management are needed. © 2019 The Authors. JBMR Plus Published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research.

OBJECTIVE: To assess the effectiveness of a CT temporal subtraction (TS) method on radiologists\' performance in sclerotic metastasis detection in the thoracolumbar spine.
METHODS: 20 pairs (current and previous CTs) of standard-dose CT and their TS images in patients with sclerotic bone metastasis and 20 pairs (current and previous CTs) of those in patients without bone metastasis were used for an observer performance study. A total of 135 lesions were identified as the reference standard of actionable lesions (sclerotic metastasis newly appeared or increased in size or in attenuation). 4 attending radiologists and 4 radiology residents participated in this observer study. Ratings and locations of \"lesions\" determined by the observers were utilized for assessing the statistical significance of differences between radiologists\' performances without and with the CT-TS images in JAFROC analysis. The statistical significance of differences in the reviewing time was determined by a two-tailed paired t-test.
RESULTS: The average figure-of-merit (FOM) values for all but one radiologist increased to a statistically significant degree, from 0.856 without the CT-TS images to 0.884 with the images (P = .037). The average sensitivity for detecting the actionable lesions was improved from 60.7 % to 72.5% at a false-positive rate of 0.15 per case by use of the CT-TS images. The average reading time with CT-TS images was significantly shorter than that without (150.6 s vs. 166.5 s, P = .004).
CONCLUSIONS: The use of CT-TS would improve the observer performance for the detection of the sclerotic bone metastasis in the thoracolumbar spine.