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Journal of Cardiothoracic Surgery Mar 2024To demonstrate the effectiveness and feasibility of robotic portal resection (RPR) for mediastinal tumour using a prospectively collected database. (Observational Study)
Observational Study
BACKGROUND
To demonstrate the effectiveness and feasibility of robotic portal resection (RPR) for mediastinal tumour using a prospectively collected database.
METHODS
Data from 73 consecutive patients with mediastinal tumours who underwent RPRs were prospectively collected from August 2018 to April 2023. All patients underwent chest and abdominal enhanced computed tomography (CT) and preoperative multidisciplinary team (MDT) discussion. The patients were stratified into two groups based on tumour size: Group A (tumour size < 4 cm) and Group B (tumour size ≥ 4 cm). General clinical characteristics, surgical procedures, and short outcomes were promptly recorded.
RESULTS
All of the cases were scheduled for RPRs. One patient (1/73, 1.4%) was switched to a small utility incision approach because of extensive pleural adhesion. Two patients (2.8%) converted to sternotomy, however, no perioperative deaths occurred. Most of the tumours were located in the anterior mediastinum (51/73, 69.9%). Thymoma (27/73, 37.0%) and thymic cyst (16/73, 21.9%) were the most common diagnoses. The median diameter of tumours was 3.2 cm (IQR, 2.4-4.5 cm). The median total operative time was 61.0 min (IQR, 50.0-90.0 min). The median intraoperative blood loss was 20 mL (IQR, 5.0-30.0 ml), and only one patient (1.4%) experienced an intraoperative complication. The median length of hospital stay was 3 days (IQR, 2-4 days). Compared with Group A, the median total operative time and console time of Group B were significantly longer (P = 0.006 and P = 0.003, respectively). The volume of drainage on the first postoperative day was greater in group B than in group A (P = 0.013).
CONCLUSION
RPR is a safe and effective technique for mediastinal tumour treatment, which can expand the application of minimally invasive surgery for the removal of complicated mediastinal tumours.
Topics: Humans; Mediastinal Neoplasms; Robotic Surgical Procedures; Robotics; Thymus Neoplasms; Thymoma; Treatment Outcome; Retrospective Studies
PubMed: 38532497
DOI: 10.1186/s13019-024-02660-8 -
Annals of Surgical Oncology Jul 2024The role of the number of involved structures (NIS) in thymic epithelial tumors (TETs) has been investigated for inclusion in future staging systems, but large cohort...
BACKGROUND
The role of the number of involved structures (NIS) in thymic epithelial tumors (TETs) has been investigated for inclusion in future staging systems, but large cohort results still are missing. This study aimed to analyze the prognostic role of NIS for patients included in the European Society of Thoracic Surgeons (ESTS) thymic database who underwent surgical resection.
METHODS
Clinical and pathologic data of patients from the ESTS thymic database who underwent surgery for TET from January 2000 to July 2019 with infiltration of surrounding structures were reviewed and analyzed. Patients' clinical data, tumor characteristics, and NIS were collected and correlated with CSS using Kaplan-Meier curves. The log-rank test was used to assess differences between subgroups. A multivariable model was built using logistic regression analysis.
RESULTS
The final analysis was performed on 303 patients. Histology showed thymoma for 216 patients (71.3%) and NET/thymic carcinoma [TC]) for 87 patients (28.7%). The most frequently infiltrated structures were the pleura (198 cases, 65.3%) and the pericardium in (185 cases, 61.1%), whereas lung was involved in 96 cases (31.7%), great vessels in 74 cases (24.4%), and the phrenic nerve in 31 cases (10.2%). Multiple structures (range, 2-7) were involved in 183 cases (60.4%). Recurrence resulted in the death of 46 patients. The CSS mortality rate was 89% at 5 years and 82% at 10 years. In the univariable analysis, the favorable prognostic factors were neoadjuvant therapy, Masaoka stage 3, absence of metastases, absence of myasthenia gravis, complete resection, thymoma histology, and no more than two NIS. Patients with more than two NIS presented with a significantly worse CSS than patients with no more than two NIS (CSS 5- and 10-year rates: 9.5% and 83.5% vs 93.2% and 91.2%, respectively; p = 0.04). The negative independent prognostic factors confirmed by the multivariable analysis were incomplete resection (hazard ratio [HR] 2.543; 95% confidence interval [CI] 1.010-6.407; p = 0.048) and more than two NIS (HR 1.395; 95% CI 1.021-1.905; p = 0.036).
CONCLUSIONS
The study showed that more than two involved structures are a negative independent prognostic factor in infiltrative thymic epithelial tumors that could be used for prognostic stratification.
Topics: Humans; Thymus Neoplasms; Male; Female; Middle Aged; Neoplasms, Glandular and Epithelial; Prognosis; Survival Rate; Databases, Factual; Follow-Up Studies; Aged; Retrospective Studies; Adult; Neoplasm Staging; Thymoma; Pleura; Neoplasm Invasiveness
PubMed: 38530530
DOI: 10.1245/s10434-024-15194-z -
JCEM Case Reports Mar 2024The thymus gland aids in the maturation of the immune system. An overactive or malfunctioning thymus gland, as seen in thymomas, can lead to disrupted immune systems....
The thymus gland aids in the maturation of the immune system. An overactive or malfunctioning thymus gland, as seen in thymomas, can lead to disrupted immune systems. Thymectomy, the usual treatment, can paradoxically lead to further derangements in the immune system, leading to new autoimmune disorders. Most of these reported disorders are rheumatological. Except preclinical studies, there are no reported cases of autoimmune diabetes post-thymectomy. A 25-year-old woman who had malignant thymoma underwent chemotherapy, followed by thymectomy and radiotherapy. She developed autoimmune diabetes mellitus (AID) approximately 1 year post-thymectomy, evident from raised glycated hemoglobin, anti-glutamic acid decarboxylase (GAD) antibodies, ineffectiveness of oral glucose-lowering agents, and positive response to insulin. AID can occur after thymectomy, as evidenced by animal studies and this case report. Whether these patients would have long-term outcomes and control of diabetes differently than classic type 1 diabetes mellitus (T1D) is uncertain. Further research is needed to prove causality between thymectomy and diabetes.
PubMed: 38524389
DOI: 10.1210/jcemcr/luae039 -
The Oncologist Jun 2024Thymic epithelial tumors (TETs) are rare tumors for which treatment options are limited. The ongoing need for improved systemic therapies reflects a limited... (Review)
Review
Thymic epithelial tumors (TETs) are rare tumors for which treatment options are limited. The ongoing need for improved systemic therapies reflects a limited understanding of tumor biology as well as the normal thymus. The essential role of the thymus in adaptive immunity is largely effected by its epithelial compartment, which directs thymocyte (T-cell) differentiation and immunologic self-tolerance. With aging, the thymus undergoes involution whereby epithelial tissue is replaced by adipose and other connective tissue, decreasing immature T-cell production. Against this natural drive toward involution, a fraction of thymuses will instead undergo oncologic transformation, leading to the formation of TETs, including thymoma and thymic carcinoma. The rarity of these tumors restricts investigation of the mechanisms of tumorigenesis and development of rational treatment options. To this end, the development of technologies which allow deep molecular profiling of individual tumor cells permits a new window through which to view normal thymic development and contrast the malignant changes that result in oncogenic transformation. In this review, we describe the findings of recent illuminating studies on the diversity of cell types within the epithelial compartment through thymic differentiation and aging. We contextualize these findings around important unanswered questions regarding the spectrum of known somatic tumor alterations, cell of origin, and tumor heterogeneity. The perspectives informed by single-cell molecular profiling offer new approaches to clinical and basic investigation of thymic epithelial tumors, with the potential to accelerate development of improved therapeutic strategies to address ongoing unmet needs in these rare tumors.
Topics: Humans; Thymus Neoplasms; Thymus Gland; Neoplasms, Glandular and Epithelial; Single-Cell Analysis; Cell Differentiation
PubMed: 38520743
DOI: 10.1093/oncolo/oyae046 -
Diagnostic Cytopathology Jun 2024Spindle epithelial tumor with thymus-like elements (SETTLE) is a rare biphasic thyroid tumor with low malignant potential that has a distinct morphology. Despite fine...
Spindle epithelial tumor with thymus-like elements (SETTLE) is a rare biphasic thyroid tumor with low malignant potential that has a distinct morphology. Despite fine needle aspiration (FNA) being a common method for evaluating thyroid nodules and lymph nodes, there are limited cytologic descriptions of SETTLE in the literature due to its rarity. As a result, SETTLE is frequently underdiagnosed or misdiagnosed as medullary carcinoma, thymoma, teratoma, synovial sarcoma, or solitary fibrous tumor, among others. We present a case of a 28-year-old man with a history of a hemithyroidectomy diagnosed as SETTLE found to have a neck nodule along the strap muscle suspicious for recurrence 5 years post-surgery. The ultrasound-guided FNA cytology specimen of the neck nodule showed loosely cohesive, monomorphous ovoid to spindled cells with scant cytoplasm and nuclei with fine to granular chromatin. In addition, there were occasional clusters of cells with a papillary configuration. The tumor cells were associated with magenta, amorphous extracellular material. Immunocytochemical staining of the cell block material revealed that tumor cells were positive for p63, cytokeratin AE1/3, and CK8/18 and negative for TTF-1 and thyroglobulin. Overall, the morphological and immunocytochemical findings were consistent with a local recurrence of SETTLE. The subsequent left anterior strap mass excision revealed a 4 cm encapsulated tumor consistent with SETTLE. Because ofits rarity and low level of awareness, SETTLE poses a diagnostic and therapeutic challenge. We herein present the cytologic findings of monomorphic SETTLE and highlight the potential cytomorphologic and immunophenotypic pitfalls. We also highlight how tumors with high-risk features can be a therapeutic challenge.
Topics: Humans; Male; Adult; Neoplasm Recurrence, Local; Thyroid Neoplasms; Neoplasms, Glandular and Epithelial; Biopsy, Fine-Needle; Biomarkers, Tumor
PubMed: 38520309
DOI: 10.1002/dc.25301 -
The New England Journal of Medicine Mar 2024Autoantibodies against interleukin-12 (anti-interleukin-12) are often identified in patients with thymoma, but opportunistic infections develop in only some of these...
BACKGROUND
Autoantibodies against interleukin-12 (anti-interleukin-12) are often identified in patients with thymoma, but opportunistic infections develop in only some of these patients. Interleukin-12 (with subunits p40 and p35) shares a common subunit with interleukin-23 (subunits p40 and p19). In a patient with disseminated infection, the identification of both anti-interleukin-23 and anti-interleukin-12 prompted further investigation.
METHODS
Among the patients (most of whom had thymoma) who were known to have anti-interleukin-12, we screened for autoantibodies against interleukin-23 (anti-interleukin-23). To validate the potential role of anti-interleukin-23 with respect to opportunistic infection, we tested a second cohort of patients with thymoma as well as patients without either thymoma or known anti-interleukin-12 who had unusual infections.
RESULTS
Among 30 patients with anti-interleukin-12 who had severe mycobacterial, bacterial, or fungal infections, 15 (50%) also had autoantibodies that neutralized interleukin-23. The potency of such neutralization was correlated with the severity of these infections. The neutralizing activity of anti-interleukin-12 alone was not associated with infection. In the validation cohort of 91 patients with thymoma, the presence of anti-interleukin-23 was associated with infection status in 74 patients (81%). Overall, neutralizing anti-interleukin-23 was detected in 30 of 116 patients (26%) with thymoma and in 30 of 36 patients (83%) with disseminated, cerebral, or pulmonary infections. Anti-interleukin-23 was present in 6 of 32 patients (19%) with severe intracellular infections and in 2 of 16 patients (12%) with unusual intracranial infections, including and complex.
CONCLUSIONS
Among patients with a variety of mycobacterial, bacterial, or fungal infections, the presence of neutralizing anti-interleukin-23 was associated with severe, persistent opportunistic infections. (Funded by the National Institute of Allergy and Infectious Diseases and others.).
Topics: Adult; Humans; Autoantibodies; Immunologic Deficiency Syndromes; Interleukin-12; Interleukin-23; Mycoses; Opportunistic Infections; Thymoma; Thymus Neoplasms; Antibodies, Neutralizing; Bacterial Infections
PubMed: 38507753
DOI: 10.1056/NEJMoa2210665 -
Journal of Thoracic Disease Feb 2024Anti-titin antibodies have been previously associated with thymoma-associated myasthenia gravis (MG) and a more clinically severe form of MG. While currently only...
BACKGROUND
Anti-titin antibodies have been previously associated with thymoma-associated myasthenia gravis (MG) and a more clinically severe form of MG. While currently only serving as a disease biomarker, its possible utility as an indicator of underlying thymus malignancy may be of value in clinical practice.
METHODS
Data was retrospectively collected and analyzed from 2013 to 2022 using an institutional record of MG patients. Anti-titin antibodies were assessed using Line Blot immunoassay.
RESULTS
From 130 MG cases, 32 (24.6%) were anti-titin positive. Anti-titin positive cases were associated with older age of disease onset [median (IQR): 63.0 (44.3-70.8) 35.5 (24.8-60.8) years] (P<0.01). Thymectomy was performed in 46 (35.4%) MG patients, 12 of which anti-titin positive (26.1%). Thymectomy samples from anti-titin positive patients comprised 10 (83.3%) cases of thymoma and 2 (16.7%) cases of thymus hyperplasia. There was a tendency towards anti-titin positive patients having more thymoma while anti-titin negative displayed more hyperplasia (P<0.01). Anti-titin positivity correlated with thymoma in patients with age of onset bellow 50 years (P=0.028). Anti-titin positivity was significantly associated with generalized MG in the late-onset group (P=0.005).
CONCLUSIONS
The presence of anti-titin antibodies appears to correlate with underlying thymoma in early-onset MG cases and with generalized MG in late-onset cases. Prospective studies are needed to further study this association.
PubMed: 38505076
DOI: 10.21037/jtd-23-94 -
Zhonghua Xue Ye Xue Za Zhi = Zhonghua... Dec 2023
Topics: Humans; Thymoma; Thymus Neoplasms; Red-Cell Aplasia, Pure; Prognosis
PubMed: 38503528
DOI: 10.3760/cma.j.issn.0253-2727.2023.12.011 -
Portuguese Journal of Cardiac Thoracic... Oct 2023A mediastinal cavernous hemangioma is difficult to distinguish from other types of mediastinal tumours. They are usually asymptomatic and incidentally discovered in an...
A mediastinal cavernous hemangioma is difficult to distinguish from other types of mediastinal tumours. They are usually asymptomatic and incidentally discovered in an imaging study but can present with compressive symptoms or by infiltration of adjacent structures. A 64-year-old woman with a prior history of triple negative invasive carcinoma of the breast, under surveillance was referred after a Chest CT-scan showed a soft tissue 40x20 mm mediastinal mass, suggestive of a thymoma, and as such no tissue biopsy was obtained. A right-side uniportal VATS was performed, the anterior mediastinum dissected and the mass was exposed, and several anomalous veins were identified. Histopathology showed 36x31x15 mm mass, compatible with a cavernous hemangioma of the anterior mediastinum. This case, whilst not questioning the NCCN statement suggesting not doing a tissue biopsy, points to the fact that rare differential diagnosis, like a Cavernous Hemangioma do exist, and a careful and sound judgement is needed at all times.
Topics: Female; Humans; Middle Aged; Mediastinal Neoplasms; Thymoma; Mediastinum; Hemangioma, Cavernous; Hemangioma; Thymus Neoplasms; Respiratory Tract Neoplasms
PubMed: 38499033
DOI: 10.48729/pjctvs.329 -
Handbook of Clinical Neurology 2024Thymoma is often associated with paraneoplastic neurologic diseases. Neural autoantibody testing is an important tool aiding diagnosis of thymoma and its autoimmune... (Review)
Review
Thymoma is often associated with paraneoplastic neurologic diseases. Neural autoantibody testing is an important tool aiding diagnosis of thymoma and its autoimmune neurologic complications. Autoantibodies specific for muscle striational antigens and ion channels of the ligand-gated nicotinic acetylcholine receptor superfamily are the most prevalent biomarkers. The autoimmune neurologic disorders associating most commonly with thymoma are myasthenia gravis (MG), peripheral nerve hyperexcitability (neuromyotonia and Morvan syndrome), dysautonomia, and encephalitis. Patients presenting with these neurologic disorders should be screened for thymoma at diagnosis. Although they can cause profound disability, they usually respond to immunotherapy and treatment of the thymoma. Worsening of the neurologic disorder following surgical removal of a thymoma may herald tumor recurrence. Prompt recognition of paraneoplastic neurologic disorders is critical for patient management. A multidisciplinary approach is required for optimal management of neurologic autoimmunity associated with thymoma.
Topics: Humans; Thymoma; Neoplasm Recurrence, Local; Thymus Neoplasms; Autoantibodies; Nervous System Diseases; Isaacs Syndrome
PubMed: 38494291
DOI: 10.1016/B978-0-12-823912-4.00008-6