-
JCEM Case Reports May 2024Malignancies may induce clinical sequelae distant from the sites of the tumor. Such paraneoplastic phenomena are known to affect many organs, including the skin....
Malignancies may induce clinical sequelae distant from the sites of the tumor. Such paraneoplastic phenomena are known to affect many organs, including the skin. Vitiligo is a disorder of patchy depigmentation, appearing as white macules with distinct margins. Rarely, vitiligo has been reported as a paraneoplastic occurrence, in the settings of pituitary adenoma, thymoma, gastric carcinoma, and lymphoma. We now describe a man presenting with the abrupt onset of vitiligo on the hands coinciding with recurrence of adrenocortical carcinoma (ACC) in the abdomen. The vitiligo rapidly dissipated following resection of his cancer. We believe this to be the first report of paraneoplastic vitiligo associated with ACC. Endocrinologists typically manage ACC and should be aware of this link, as the de novo observation of vitiligo may signal the onset or recurrence of underlying tumor. Other practitioners that encounter patients with new vitiligo should add ACC to their differential diagnosis of potential underlying conditions.
PubMed: 38707658
DOI: 10.1210/jcemcr/luae070 -
EJNMMI Research May 2024Thymic cysts are a rare benign disease that needs to be distinguished from low-risk thymoma. [F]fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed...
BACKGROUND
Thymic cysts are a rare benign disease that needs to be distinguished from low-risk thymoma. [F]fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) is a non-invasive imaging technique used in the differential diagnosis of thymic epithelial tumours, but its usefulness for thymic cysts remains unclear. Our study evaluated the utility of visual findings and quantitative parameters of [F]FDG PET/CT for differentiating between thymic cysts and low-risk thymomas.
METHODS
Patients who underwent preoperative [F]FDG PET/CT followed by thymectomy for a thymic mass were retrospectively analyzed. The visual [F]FDG PET/CT findings evaluated were PET visual grade, PET central metabolic defect, and CT shape. The quantitative [F]FDG PET/CT parameters evaluated were PET maximum standardized uptake value (SUVmax), CT diameter (cm), and CT attenuation in Hounsfield units (HU). Findings and parameters for differentiating thymic cysts from low-risk thymomas were assessed using Pearson's chi-square test, the Mann-Whitney U-test, and receiver operating characteristics (ROC) curve analysis.
RESULTS
Seventy patients (18 thymic cysts and 52 low-risk thymomas) were finally included. Visual findings of PET visual grade (P < 0.001) and PET central metabolic defect (P < 0.001) showed significant differences between thymic cysts and low-risk thymomas, but CT shape did not. Among the quantitative parameters, PET SUVmax (P < 0.001), CT diameter (P < 0.001), and CT HU (P = 0.004) showed significant differences. In ROC analysis, PET SUVmax demonstrated the highest area under the curve (AUC) of 0.996 (P < 0.001), with a cut-off of equal to or less than 2.1 having a sensitivity of 100.0% and specificity of 94.2%. The AUC of PET SUVmax was significantly larger than that of CT diameter (P = 0.009) and CT HU (P = 0.004).
CONCLUSIONS
Among the [F]FDG PET/CT parameters examined, low FDG uptake (SUVmax ≤ 2.1, equal to or less than the mediastinum) is a strong diagnostic marker for a thymic cyst. PET visual grade and central metabolic defect are easily accessible findings.
PubMed: 38702532
DOI: 10.1186/s13550-024-01108-3 -
Rozhledy V Chirurgii : Mesicnik... 2024Thoracic surgery is increasingly influenced by the development of minimally invasive approaches which have also influenced surgery in the area of the anterior...
Thoracic surgery is increasingly influenced by the development of minimally invasive approaches which have also influenced surgery in the area of the anterior mediastinum. The previously standard approach to the thymus via partial sternotomy was gradually replaced by the videothoracoscopic approach in most cases. In recent years, robotically assisted surgery has been gaining ground worldwide in this area, as well. The aim of our paper is to provide a comprehensive overview of procedures in the field of the thymus, including their indications, and to share our first experience with robot-assisted thymus surgery. At the 3rd Department of Surgery, since the start of the robot-assisted thymus surgery program, 23 thymectomies have been performed using this approach, of which 17 were performed for thymoma, 3 for myasthenia gravis, and 3 for parathyroid adenoma localized in thymus tissue. From our experience and the available data, it follows that the length of hospitalization, the rate of complications and the resulting effect of robot-assisted procedures is comparable to VTS procedures; however, the robot-assisted surgery also allows for mini-invasive treatment even in significantly obese patients and in patients with advanced thymic tumors who would otherwise be indicated for open thymectomy.
Topics: Humans; Robotic Surgical Procedures; Thymectomy; Thymus Neoplasms; Thymoma; Myasthenia Gravis; Parathyroid Neoplasms; Thymus Gland; Male
PubMed: 38697812
DOI: 10.33699/PIS.2024.103.2.40-47 -
Clinical Immunology (Orlando, Fla.) Jun 2024Ataxia-telangiectasia (A-T) is a rare disorder caused by genetic defects of A-T mutated (ATM) kinase, a key regulator of stress response, and characterized by...
Ataxia-telangiectasia (A-T) is a rare disorder caused by genetic defects of A-T mutated (ATM) kinase, a key regulator of stress response, and characterized by neurodegeneration, immunodeficiency, and high incidence of cancer. Here we investigated NK cells in a mouse model of A-T (Atm) showing that they are strongly impaired at killing tumor cells due to a block of early signaling events. On the other hand, in Atm littermates with thymic lymphoma NK cell cytotoxicity is enhanced as compared with ATM-proficient mice, possibly via tumor-produced TNF-α. Results also suggest that expansion of exhausted NKG2D NK cells in Atm mice is driven by low-level expression of stress-inducible NKG2D ligands, whereas development of thymoma expressing the high-affinity MULT1 ligand is associated with NKG2D down-regulation on NK cells. These results expand our understanding of immunodeficiency in A-T and encourage exploring NK cell biology in A-T patients in the attempt to identify cancer predictive biomarkers and novel therapeutic targets.
Topics: Animals; Killer Cells, Natural; Ataxia Telangiectasia Mutated Proteins; NK Cell Lectin-Like Receptor Subfamily K; Mice; Ataxia Telangiectasia; Mice, Knockout; Mice, Inbred C57BL; Thymoma; Tumor Necrosis Factor-alpha; Cytotoxicity, Immunologic; Thymus Neoplasms; Signal Transduction; Membrane Proteins; Histocompatibility Antigens Class I
PubMed: 38697554
DOI: 10.1016/j.clim.2024.110233 -
The Journal of Thoracic and... Apr 2024Our previous study demonstrated that modified subxiphoid video-assisted thoracic surgery thymectomy with an auxiliary sternal retractor is feasible for locally invasive...
OBJECTIVE
Our previous study demonstrated that modified subxiphoid video-assisted thoracic surgery thymectomy with an auxiliary sternal retractor is feasible for locally invasive thymic malignancies. This study aimed to compare perioperative and oncological outcomes of modified subxiphoid video-assisted thoracoscopic surgery thymectomy versus median sternotomy thymectomy for locally advanced thymic malignancies.
METHODS
In total, 221 patients with T thymic malignancies who underwent modified subxiphoid video-assisted thoracoscopic surgery thymectomy or median sternotomy thymectomy between 2015 and 2020 were enrolled in our prospectively maintained database. A 1:1 propensity score-matching analysis was performed to balance the bias. Surgical difficulty was evaluated with a modified resection index. Perioperative and oncological results were compared between the modified subxiphoid video-assisted thoracoscopic surgery thymectomy group and the median sternotomy thymectomy group.
RESULTS
There were 72 patients in each group in the final analysis. Our results showed that the modified subxiphoid video-assisted thoracoscopic surgery thymectomy group had a shorter operative duration (98 vs 129 minutes, P < .001), less blood loss (40 vs 100 mL, P < .001), shorter drainage duration (3 vs 5 days, P < .001), shorter length of hospital stay (5 vs 6 days, P < .001), and fewer postoperative complications (5.6% vs 23.6%; P = .005). No significant difference was detected in complete resection (98.6% vs 98.6%, P = 1.000) between the 2 groups. Conversion occurred in 5 of 106 patients (4.7%). Survival analyses indicated similar recurrence-free survival (hazard ratio, 0.94; 95% CI, 0.40-2.20; P = .883) and overall survival (hazard ratio, 0.52; 95% CI, 0.05-5.02; P = .590) between the 2 groups.
CONCLUSIONS
Modified subxiphoid video-assisted thoracoscopic surgery thymectomy was safe and effective for T thymic malignancies and could be an alternative for selected patients with locally advanced thymic diseases. Further prospective studies are needed to evaluate the long-term survival of those undergoing modified subxiphoid approach thoracoscopic thymectomy.
PubMed: 38692477
DOI: 10.1016/j.jtcvs.2024.04.023 -
Biomedicines Mar 2024Programmed death-ligand 1 (PD-L1) expression in neoplastic and immune cells of the tumor microenvironment determines the efficacy of antitumor immunity, while it can be...
BACKGROUND
Programmed death-ligand 1 (PD-L1) expression in neoplastic and immune cells of the tumor microenvironment determines the efficacy of antitumor immunity, while it can be regulated at the epigenetic level by various factors, including HDACs. In this study, we aim to evaluate the expression patterns of PD-L1 in thymic epithelial tumors (TETs), while we attempt the first correlation analysis between PD-L1 and histone deacetylases (HDACs) expression.
METHODS
Immunohistochemistry was used to evaluate the expression of PD-L1 in tumor and immune cells of 91 TETs with SP263 and SP142 antibody clones, as well as the expressions of HDCA1, -2, -3, -4, -5, and -6.
RESULTS
The PD-L1 tumor proportion score (TPS) was higher, while the immune cell score (IC-score) was lower in the more aggressive TET subtypes and in more advanced Masaoka-Koga stages. A positive correlation between PD-L1 and HDAC-3, -4, and -5 cytoplasmic expression was identified.
CONCLUSIONS
Higher PD-L1 expression in neoplastic cells and lower PD-L1 expression in immune cells of TETs characterizes more aggressive and advanced neoplasms. Correlations between PD-L1 and HDAC expression unravel the impact of epigenetic regulation on the expression of immune checkpoint molecules in TETs, with possible future applications in combined therapeutic targeting.
PubMed: 38672128
DOI: 10.3390/biomedicines12040772 -
BMC Neurology Apr 2024Non-motor symptoms in myasthenia gravis (MG) are rarely confirmed. Although there are some small cohort studies, a large-systemic survey has not yet been performed.
BACKGROUND
Non-motor symptoms in myasthenia gravis (MG) are rarely confirmed. Although there are some small cohort studies, a large-systemic survey has not yet been performed.
METHODS
We investigated the incidence and clinical characteristics of patients with MG who had taste disorders and alopecia using data of 1710 patients with MG enrolled in the Japan MG Registry 2021.
RESULTS
Among them, 104 (6.1%) out of 1692 patients and 138 (8.2%) out of 1688 patients had histories of taste disorders and alopecia, respectively. Among the patients with MG, taste disorders were significantly more common in women, those with severe symptoms, refractory MG, or thymoma-associated MG, and were less common in those with ocular MG. The taste disorders often occurred after the onset of MG and often responded to MG treatments. Alopecia was more common in MG patients with a history of bulbar palsy and thymoma, and it often occurred before the onset of MG and sometimes responded to MG treatments. Multivariate logistic regression analysis revealed taste disturbance was associated with worst quantitative MG score and thymoma-associated MG; and alopecia was associated with thymoma-associated MG.
CONCLUSION
Clinicians should be aware of the non-motor symptoms in MG, especially in patients with severe myasthenic symptoms and thymoma-associated MG.
Topics: Humans; Myasthenia Gravis; Alopecia; Female; Male; Taste Disorders; Middle Aged; Adult; Aged; Japan; Registries; Thymoma; Incidence
PubMed: 38664714
DOI: 10.1186/s12883-024-03644-w -
European Journal of Cardio-thoracic... May 2024Robotic thymectomy has been suggested and considered technically feasible for thymic tumours. However, because of small-sample series and the lack of data on long-term...
OBJECTIVES
Robotic thymectomy has been suggested and considered technically feasible for thymic tumours. However, because of small-sample series and the lack of data on long-term results, controversies still exist on surgical and oncological results with this approach. We performed a large national multicentre study sought to evaluate the early and long-term outcomes after robot-assisted thoracoscopic thymectomy in thymic epithelial tumours.
METHODS
All patients with thymic epithelial tumours operated through a robotic thoracoscopic approach between 2002 and 2022 from 15 Italian centres were enrolled. Demographic characteristics, clinical, intraoperative, postoperative, pathological and follow-up data were retrospectively collected and reviewed.
RESULTS
There were 669 patients (307 men and 362 women), 312 (46.6%) of whom had associated myasthenia gravis. Complete thymectomy was performed in 657 (98%) cases and in 57 (8.5%) patients resection of other structures was necessary, with a R0 resection in all but 9 patients (98.6%). Twenty-three patients (3.4%) needed open conversion, but no perioperative mortality occurred. Fifty-one patients (7.7%) had postoperative complications. The median diameter of tumour resected was 4 cm (interquartile range 3-5.5 cm), and Masaoka stage was stage I in 39.8% of patients, stage II in 56.1%, stage III in 3.5% and stage IV in 0.6%. Thymoma was observed in 90.2% of patients while thymic carcinoma occurred in 2.8% of cases. At the end of the follow-up, only 2 patients died for tumour-related causes. Five- and ten-year recurrence rates were 7.4% and 8.3%, respectively.
CONCLUSIONS
Through the largest collection of robotic thymectomy for thymic epithelial tumours we demonstrated that robot-enhanced thoracoscopic thymectomy is a technically sound and safe procedure with a low complication rate and optimal oncological outcomes.
Topics: Humans; Thymectomy; Thymus Neoplasms; Male; Female; Middle Aged; Robotic Surgical Procedures; Retrospective Studies; Aged; Adult; Treatment Outcome; Postoperative Complications; Italy; Neoplasms, Glandular and Epithelial; Young Adult
PubMed: 38663851
DOI: 10.1093/ejcts/ezae178 -
Neurological Sciences : Official... Jul 2024Coronavirus disease 2019 (COVID-19) has been a great concern since 2019. Patients with myasthenia gravis (MG) may be at higher risk of COVID-19 and a more severe disease...
BACKGROUND
Coronavirus disease 2019 (COVID-19) has been a great concern since 2019. Patients with myasthenia gravis (MG) may be at higher risk of COVID-19 and a more severe disease course. We examined the associations between COVID-19 and MG.
METHODS
This single-center retrospective cohort study involved 134 patients who were diagnosed with MG from June 2020 to November 2022 and followed up until April 2023. They were divided into a COVID-19 group and non-COVID-19 group. Logistic regression analysis was used to detect factors potentially associating COVID-19 with MG.
RESULTS
Of the 134 patients with MG, 108 (80.6%) had COVID-19. A higher number of comorbidities was significantly associated with an increased risk of COVID-19 (p = 0.040). A total of 103 patients (95.4%) had mild/moderate COVID-19 symptoms, and 4 patients (3.7%) were severe/critical symptoms (including 2 deaths). Higher age (p = 0.036), use of rituximab (p = 0.037), tumors other than thymoma (p = 0.031), Hashimoto's thyroiditis (p = 0.011), more comorbidities (p = 0.002), and a higher baseline MG activities of daily living (MG-ADL) score (p = 0.006) were risk factors for severe COVID-19 symptoms. The MG-ADL score increased by ≥ 2 points in 16 (15.7%) patients. Dry cough and/or expectoration (p = 0.011), use of oral corticosteroids (p = 0.033), and use of more than one kind of immunosuppressant (p = 0.017) were associated with the increase of the post-COVID-19 MG-ADL score.
CONCLUSION
Most patients with MG have a mild course of COVID-19. However, patients with older age, many comorbidities, a high MG-ADL score, and use of a variety of immunosuppressants during COVID-19 may be more prone to severe symptoms.
Topics: Humans; Myasthenia Gravis; COVID-19; Male; Female; Retrospective Studies; Middle Aged; China; Adult; Aged; Comorbidity; SARS-CoV-2; Risk Factors; Severity of Illness Index
PubMed: 38652194
DOI: 10.1007/s10072-024-07518-4 -
Journal of Clinical Neuroscience :... Jun 2024To investigate the relationship between short-term changes in quantitative myasthenia gravis score (QMGS) after thymectomy and postoperative recurrence in myasthenia... (Observational Study)
Observational Study
OBJECTIVE
To investigate the relationship between short-term changes in quantitative myasthenia gravis score (QMGS) after thymectomy and postoperative recurrence in myasthenia gravis (MG) patients without thymoma.
METHODS
A retrospective observational cohort study. The QMGS of 44 patients with non-thymomatous MG were evaluated before and 1 month after thymectomy, and the frequency and time of postoperative recurrence were recorded. The reduction rate of QMGS (rr-QMGS) was defined as (QMGS one week before thymectomy - QMGS one month after thymectomy)/ QMGS one week before thymectomy × 100 %, as an indicator of short-term symptom change after thymectomy. The receiver operating characteristic (ROC) curve was established to determine an appropriate cut-off value of rr-QMGS for distinguishing postoperative recurrence. Multivariate Cox regression analysis was applied to predict postoperative recurrence.
RESULTS
Postoperative recurrence occurred in 21 patients (30 times in total) during follow-up. The mean annual recurrence rate was 3.98 times/year preoperatively and 0.30 times/year postoperatively. ROC analysis determined the cut-off value of rr-QMGS was 36.7 % (sensitivity 90.5 %, specificity 52.2 %). Multivariate Cox regression analysis showed that rr-QMGS<36.7 % (hazard rate[HR]6.251, P = 0.014) is positive predictor of postoperative recurrence. Kaplan-Meier analysis showed that postoperative recurrence time was earlier in the low rr-QMGS group than in the high rr-QMGS group (12.62 vs. 36.60 months, p = 0.005).
CONCLUSIONS
Low rr-QMGS is associated with early postoperative recurrence. Rr-QMGS can be used to predict postoperative recurrence of non-thymomatous MG.
Topics: Humans; Myasthenia Gravis; Thymectomy; Male; Female; Middle Aged; Retrospective Studies; Adult; Recurrence; Aged; Young Adult; ROC Curve; Follow-Up Studies
PubMed: 38640804
DOI: 10.1016/j.jocn.2024.04.011