Idiopathic multicentric Castleman disease and connective tissue disorder successfully treated by siltuximab: a pediatric case report.

Background: Castleman disease (CD) is a rare lymphoproliferative disease. Idiopathic multicentric CD (iMCD), representing a distinct entity in CD, is partly attributed to autoimmune abnormalities and the hyperplastic process in iMCD involving the immune system. Consequently, iMCD presents a range of overlapping manifestations with connective tissue disorder (CTD), resulting in an inability to tell whether they coexist or imitate each other. Reports of CD combined with CTD are rare, more cases are needed to be summarized and analyzed to improve the efficiency of diagnosis and accelerate the development of novel treatments. Case Description: A male pediatric patient was diagnosed with CTD in October 2019 and had been receiving regular treatment with tocilizumab and glucocorticoid or methotrexate since April 2020. He was further diagnosed with iMCD of the hyaline vascular subtype according to biopsy-proven histopathological features and imaging-proven multiple involvement in August 2021. He received 4 doses of rituximab and then a combination of thalidomide and dexamethasone for about 1 year. His clinical symptoms were well controlled throughout the disease for a long period, but inflammatory markers were repeatedly elevated, which eventually turned normal after switching to siltuximab from July 2023, although a significant elevation of interleukin-6 occurred. Conclusions: We reported a pediatric case diagnosed as CTD and iMCD, whose inflammation finally be well controlled by siltuximab. Hopefully, our work will add insight into such rare situations and it is undoubtedly that the pathophysiological mechanism of CD and CTD coexistence and prediction models of treatment response remains to be explored to facilitate the clinical management and optimal treatment.

Extracapsular extension of transitional zone prostate cancer miss-detected by multiparametric magnetic resonance imaging.

OBJECTIVES: To demonstrate the importance of extracapsular extension (ECE) of transitional zone (TZ) prostate cancer (PCa), examine the causes of its missed detection by Mp-MRI, and develop a new predictive model by integrating multi-level clinical variables. MATERIALS AND METHODS: This retrospective study included 304 patients who underwent laparoscopic radical prostatectomy after 12 + X needle transperineal transrectal ultrasound (TRUS)-MRI-guided targeted prostate biopsy from 2018 to 2021 in our center was performed. RESULTS: In this study, the incidence rates of ECE were similar in patients with MRI lesions in the peripheral zone (PZ) and TZ (P = 0.66). However, the missed detection rate was higher in patients with TZ lesions than in those with PZ lesions (P < 0.05). These missed detections result in a higher positive surgical margin rate (P < 0.05). In patients with TZ lesions, detected MP-MRI ECE may have grey areas: the longest diameters of the MRI lesions were 16.5-23.5 mm; MRI lesion volumes were 0.63-2.51 ml; MRI lesion volume ratios were 2.75-8.86%; PSA were 13.85-23.05 ng/ml. LASSO regression was used to construct a clinical prediction model for predicting the risk of ECE in TZ lesions from the perspective of MRI and clinical features, including four variables: the longest diameter of MRI lesions, TZ pseudocapsule invasion, ISUP grading of biopsy pathology, and number of positive biopsy needles. CONCLUSIONS: Patients with MRI lesions in the TZ have the same incidence of ECE as those with lesions in the PZ, but a higher missed detection rate.

Analysis on diagnosis and treatments of 16 cases of extracranial malignant rhabdoid tumor in children.

Background: To explore the clinical features, treatment and early prognosis of malignant rhabdoid tumor (MRT) of kidney and extrarenal extracranial soft tissue in children. Methods: From January 2011 to July 2021, a total of 16 patients, who were diagnosed with MRTs of the kidney and extrarenal soft tissue were retrospectively analyzed and were divided into MRT of the kidney (MRTK) and extrarenal extracranial MRT (EERT). Results: Sixteen patients were followed up for at least 15 months. The overall average age of onset was (26.6±20.1 months), the 1-year overall survival (OS) of 71.4% at the age of ≥24 months was significantly higher than the 1-year OS (11.1%) <24 months of age [hazard ratio (HR) =0.21 (0.06-0.77), log-rank P=0.019<0.05]. The distant metastasis rate of children in the MRTK group (83.3%) is significantly higher than EERT group (20%) (P=0.017<0.05), and the proportion of children with lung metastasis in the MRTK group was as much as 83.3%, which was significantly higher than that in the EERT group (10%) (P<0.05), but the related metastases and primary tumors did not regress after radiotherapy and chemotherapy in all cases. In the MRTK group, 5 cases were completely excised, but only 1 survived; while 5 cases in the EERT group that were completely excised and all alive. Children with tumor total excision in EERT group had a 1-year OS of 100% as compared to children with tumor total excision in MRTK group had a 1-year OS of 20% [HR =0.07 (0.01-0.57), log-rank P=0.013<0.05]. The average survival time of dead cases in MRTK group and EERT group were 6.60±3.28 months and 1.40±0.65 months respectively (P=0.008<0.05). Conclusions: Children with MRT under 2 years of age have significantly worse prognosis than children exceeding 2 years of age. MRTK is more prone to lung metastasis but has longer survival time, which is related to the R0 resection of the primary tumor. Children with EERT may have a better prognosis if the tumor can be completely resected with the impact of age. MRTK and EERT are both insensitive to radiotherapy and chemotherapy.

Prognostic significance of sarcopenia and systemic inflammation for patients with renal cell carcinoma following nephrectomy.

Background: To clarify the prognostic effect of preoperative sarcopenia and systemic inflammation, and to develop a nomogram for predicting overall survival (OS) of patients with renal cell carcinoma (RCC) following partial or radical nephrectomy. Methods: Patients with RCC following nephrectomy from the First Affiliated Hospital of Soochow University during January 2018 to September 2020 were included in this study. The relationship between sarcopenia and inflammatory markers was identified by logistic regression analysis. Then univariable Cox regression analysis, LASSO regression analysis and multivariable Cox regression analysis were analyzed sequentially to select the independent prognostic factors. Kaplan-Meier survival curves were applied to ascertain the prognostic value. Finally, the identified independent predictors were incorporated in a nomogram, which was internally validated and compared with other methods. Results: A total of 276 patients were enrolled, and 96 (34.8%) were diagnosed with sarcopenia, which was significantly associated with neutrophil-to-lymphocyte ratio (NLR). Sarcopenia and elevated inflammation markers, i.e., NLR, platelet-to-lymphocyte ratio (PLR) and the modified Glasgow Prognostic Score (mGPS), were independent factors for determining the OS. The model had good discrimination with Concordance index of 0.907 (95% CI: 0.882-0.931), and the calibration plots performed well. Both net reclassification index (NRI) and integrated discriminant improvement (IDI) exhibited better performance of the nomogram compared with clinical stage-based, sarcopenia-based and integrated "NLR+PLR+mGPS" methods. Moreover, decision curve analysis showed a net benefit of the nomogram at a threshold probability greater than 20%. Conclusions: Preoperative sarcopenia was significantly associated with NLR. A novel nomogram with well validation was developed for risk stratification, prognosis tracking and personalized therapeutics of RCC patients.