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Updates in Surgery May 2024Thymic cysts are rare, radiological diagnosis is often incidental, and cysts seldom assume clinical relevance for symptoms of compression. Thymoma were occasionally...
Thymic cysts are rare, radiological diagnosis is often incidental, and cysts seldom assume clinical relevance for symptoms of compression. Thymoma were occasionally found inside both complex and simple thymic cysts. Given the challenges in accurately clinical diagnosing and since the occasionally discovering of thymoma inside both complex and simple thymic cysts, the management of thymic cysts remains controversial. Advancements in surgical tools such as robotics, applied to thymic conditions, could potentially transform the approach to thymic cysts. We report one the largest multicentric series of thymic cysts surgically treated with robotic approach, focusing on preoperative findings and surgical results. Cases were gathered from three Italian thoracic surgery centers with homogeneous clinical practice, significant experience in thymic neoplasms, and thoracic robotic skilled. Surgical intervention was indicated for patients with radiological diagnosis of thymic cysts under the following circumstances: the presence of symptoms, concurrent myasthenia gravis, cysts growing in follow-up, and the complexity of the cyst with suspicion of neoplasm. Data were collected and matched according to postoperative and pathological features to identify potential prognostic factors. Population include 57 patients, 29/28 male/female ratio with mean age of 59.46 ± 11.67 years. The average size of the thymic cysts was 29.14 ± 24.53 ranged between 3 and 150 mm. All patients undergone CT scan and mean of values of density was 25.82 ± 11-82 Hounsfield. Surgical procedures were robotic approach in all case including total/extended thymectomy 35 (61.4%) and cyst resection/partial thymectomy 22 (38.6%). There were no mortality or recurrence. Major complications rate was 5.3%. No correlations were observed between preoperative features and complication. Pathological examination revealed microfoci of thymic tumor in four cases. Robot-assisted surgery for thymic cysts showed excellent early clinical outcomes with low rate of postoperative complications also in case of large lesion. Thymic cysts should not be underestimated due to the risk of coexistent thymic neoplasm.
PubMed: 38816604
DOI: 10.1007/s13304-024-01895-3 -
Pathology, Research and Practice Jul 2024Four cases of a distinct carcinoma of the thymic gland are presented. The patients were 4 adult males with an age range from 40 to 47 years (mean, 43.5 years)....
Four cases of a distinct carcinoma of the thymic gland are presented. The patients were 4 adult males with an age range from 40 to 47 years (mean, 43.5 years). Clinically, all patients presented with non-specific respiratory symptoms. None of the patients had any prior history of head and neck neoplasm or surgery in that anatomic area. Large anterior mediastinal masses were found on diagnostic imaging with concurrent metastatic disease to pleura, lungs, regional lymph nodes and bones. Microscopically, all tumors were composed of a solid proliferation of hyperchromatic, monomorphic small cells with focal cytoplasmic clearing embedded in a fibromyxoid stroma. In one case, occasional duct-like structures were identified. Immunohistochemically, the tumors were positive for pancytokeratin, CD117 and MYB and negative for myoepithelial markers. Systemic chemotherapy was initiated in all patients. Despite therapy, clinical follow-up revealed that all 4 patients died of their disease 11-23 months after their initial diagnosis. The cases in this series highlight a tumor that is different from conventional thymic carcinoma and that has the morphological and immunohistochemical features commonly seen in adenoid cystic carcinomas with high-grade transformation. Correct diagnosis is essential for patient management.
Topics: Humans; Carcinoma, Adenoid Cystic; Male; Middle Aged; Adult; Thymus Neoplasms; Immunohistochemistry; Biomarkers, Tumor; Cell Transformation, Neoplastic; Fatal Outcome
PubMed: 38810375
DOI: 10.1016/j.prp.2024.155356 -
BMJ Case Reports May 2024Postpneumonectomy changes, including ipsilateral diaphragmatic elevation and rib crowding, mediastinal shifting of the heart and reduction of respiratory functional...
Postpneumonectomy changes, including ipsilateral diaphragmatic elevation and rib crowding, mediastinal shifting of the heart and reduction of respiratory functional reserve by 45%-55%, make cardiac surgery challenging in terms of access to the heart and great vessels. There is a paucity of literature on coronary artery bypass graft (CABG) surgery in patients with a history of pneumonectomy. We report a case of a male in his 70s with a history of left pneumonectomy who underwent a successful on-pump CABG surgery using the left internal mammary artery graft and great saphenous vein graft. The patient was extubated postoperatively and discharged in a stable condition. With no consensus available for optimal preoperative, intraoperative and postoperative management for these high-risk patients, our case report adds to the rare literature regarding successful on-pump CABG after pneumonectomy.
Topics: Humans; Male; Pneumonectomy; Coronary Artery Bypass; Aged; Mammary Arteries; Lung Neoplasms; Treatment Outcome
PubMed: 38802260
DOI: 10.1136/bcr-2024-260815 -
European Journal of Cardio-thoracic... May 2024
Topics: Humans; Teratoma; Mediastinal Neoplasms; Rupture, Spontaneous; Female; Tomography, X-Ray Computed; Neck
PubMed: 38794921
DOI: 10.1093/ejcts/ezae167 -
Life (Basel, Switzerland) May 2024Non-Hodgkin lymphoma (NHL) is among the five most common pediatric cancer diagnoses in children and adolescents and consists of a heterogeneous group of lymphoid tissue...
Non-Hodgkin lymphoma (NHL) is among the five most common pediatric cancer diagnoses in children and adolescents and consists of a heterogeneous group of lymphoid tissue malignancies -with B-cell-derived NHL accounting for nearly 80% of cases. Novel and high-throughput diagnostic tools have significantly increased our understanding of B-NHL biology and molecular pathogenesis, leading to new NHL classifications and treatment options. This retrospective cohort study investigated 17 cases of both mature B-cell NHL (Burkitt lymphoma or BL; Diffuse large B-cell lymphoma or DLBCL; Primary mediastinal large B-cell lymphoma or PMBCL; Follicular lymphoma or FL) and immature B-cell progenitor NHL (B-lymphoblastic lymphoma or BLL) that were treated in a tertiary Pediatric Hematology-Oncology Department during the last 20 years. Modern NHL protocols for children, adolescents, and young adults, along with the addition of rituximab, are safe and efficient (100% overall survival; one relapse). Elevated ESR was more prevalent than elevated LDH. Analyses have focused on immune reconstitution (grade ≥3 infections, lymphocyte and immunoglobulin levels recovery) and body-mass-index changes post-treatment, late effects (in 53% of patients), and the presence of histology markers BCL2, BCL6, CD30, cMYC, and Ki-67%. One patient was diagnosed with a second malignant neoplasm (papillary thyroid cancer).
PubMed: 38792653
DOI: 10.3390/life14050633 -
BMJ Case Reports May 2024Tumours of adipose tissue origin are relatively rare in the head and neck. Here, we report a case of an unfamiliar lipomatous lesion that involved the neck and...
Tumours of adipose tissue origin are relatively rare in the head and neck. Here, we report a case of an unfamiliar lipomatous lesion that involved the neck and mediastinum. A nil-comorbid man in his 40s presented with a slowly progressive anterior neck swelling of 3 years, which was diagnosed as lipoma by histopathological sampling. Computed tomography demonstrated the lesion to be involving parapharyngeal and retropharyngeal spaces with mediastinal extension. The lesion was removed by the transcervical approach. The final histology of the excised specimen, with immunohistochemistry for mouse double minute 2 (MDM2) and p16, suggested an atypical lipomatous tumour (ALT). This report accentuates the occurrence of this rare neoplasm in the neck, which often mimics lipoma clinically. Although radiology can demonstrate suggestive features, histology with MDM2 and/or p16 positivity can confirm the diagnosis of ALT as against the lipoma. A successful transcervical excision, despite the deeper extension of the lesion between the critical structures of the neck and mediastinum, demonstrates the non-infiltrating nature of the tumour.
Topics: Humans; Male; Head and Neck Neoplasms; Lipoma; Tomography, X-Ray Computed; Adult; Mediastinal Neoplasms; Neck; Diagnosis, Differential; Mediastinum
PubMed: 38789269
DOI: 10.1136/bcr-2023-258292 -
Radiology Case Reports Aug 2024De-differentiated primary mediastinal liposarcomas account for less than 1% of all liposarcoma pathology. We report the case of an 82-year-old male who was suffering...
De-differentiated primary mediastinal liposarcomas account for less than 1% of all liposarcoma pathology. We report the case of an 82-year-old male who was suffering from progressive dysphagia, shortness of breath, and dysphonia for a period of 2 months. A CT scan of the chest with contrast revealed a large heterogeneously enhancing posterior mediastinal mass extending into the posterior soft tissues of the neck, abutting bilateral carotid arteries, and displacing the trachea and esophagus. Treatment chosen for our patient was surgical resection followed by adjuvant radiation therapy which resolved the patient's presenting symptoms. The insights gained through the diagnosis, management, and treatment of our patient can be utilized to approach this type of rare neoplasm.
PubMed: 38783933
DOI: 10.1016/j.radcr.2024.04.054 -
BMC Neurology May 2024Ectopic cervical thymoma (ECT) is an extremely rare tumor, especially in association with myasthenia gravis (MG).
BACKGROUND
Ectopic cervical thymoma (ECT) is an extremely rare tumor, especially in association with myasthenia gravis (MG).
CASE PRESENTATION
We report a case of myasthenia gravis with an ectopic thymoma in the neck, whose myasthenic symptoms significantly improved after complete removal of the mass. A 55-year-old woman with generalized myasthenia gravis (MG) experienced worsening neuromuscular weakness after abruptly discontinuing pyridostigmine. Testing revealed acetylcholine receptor-antibody (AChR-Ab) positivity and a cervical mass initially thought to be thyroid or parathyroid was identified as a thymoma, type A. Post-surgery and radiation therapy, her myasthenic symptoms improved significantly with less prednisone and pyridostigmine requirements over time and no need for additional immunotherapies.
CONCLUSIONS
Diagnosing ECTs is challenging due to rarity, atypical locations, and inconclusive fine needle aspiration cytology (FNAC) results, often misinterpreted as thyroid or parathyroid lesions. As proper management of patients with MG, including thymectomy, offers favorable clinical outcomes such as significant improvement in myasthenic complaints and reduced immunosuppressive medication requirements, clinicians should be vigilant of the ectopic locations of thymomas to ensure timely diagnosis and intervention.
Topics: Humans; Female; Myasthenia Gravis; Middle Aged; Thymoma; Thymus Neoplasms; Choristoma
PubMed: 38783232
DOI: 10.1186/s12883-024-03656-6 -
Journal of Experimental & Clinical... May 2024Primary mediastinal B-cell lymphoma (PMBL) and classical Hodgkin lymphoma (cHL) are distinct hematological malignancies of B-cell origin that share many biological,...
BACKGROUND
Primary mediastinal B-cell lymphoma (PMBL) and classical Hodgkin lymphoma (cHL) are distinct hematological malignancies of B-cell origin that share many biological, molecular, and clinical characteristics. In particular, the JAK/STAT signaling pathway is a driver of tumor development due to multiple recurrent mutations, particularly in STAT6. Furthermore, the XPO1 gene that encodes exportin 1 (XPO1) shows a frequent point mutation (E571K) resulting in an altered export of hundreds of cargo proteins, which may impact the success of future therapies in PMBL and cHL. Therefore, targeted therapies have been envisioned for these signaling pathways and mutations.
METHODS
To identify novel molecular targets that could overcome the treatment resistance that occurs in PMBL and cHL patients, we have explored the efficacy of a first-in-class HSP110 inhibitor (iHSP110-33) alone and in combination with selinexor, a XPO1 specific inhibitor, both in vitro and in vivo.
RESULTS
We show that iHSP110-33 decreased the survival of several PMBL and cHL cell lines and the size of tumor xenografts. We demonstrate that HSP110 is a cargo of XPO1 as well as of XPO1. Using immunoprecipitation, proximity ligation, thermophoresis and kinase assays, we showed that HSP110 directly interacts with STAT6 and favors its phosphorylation. The combination of iHSP110-33 and selinexor induces a synergistic reduction of STAT6 phosphorylation and of lymphoma cell growth in vitro and in vivo. In biopsies from PMBL patients, we show a correlation between HSP110 and STAT6 phosphorylation levels.
CONCLUSIONS
These findings suggest that HSP110 could be proposed as a novel target in PMBL and cHL therapy.
Topics: Humans; Exportin 1 Protein; Karyopherins; Receptors, Cytoplasmic and Nuclear; Animals; Mice; Hodgkin Disease; Lymphoma, B-Cell; HSP110 Heat-Shock Proteins; Cell Line, Tumor; Mediastinal Neoplasms; Xenograft Model Antitumor Assays; Triazoles; Hydrazines; Female; STAT6 Transcription Factor; Molecular Targeted Therapy
PubMed: 38773631
DOI: 10.1186/s13046-024-03068-x -
Indian Journal of Pathology &... Apr 2024Pyrexia of unknown origin can be caused due to numerous infective and noninfective causes. It poses a diagnostic dilemma to the clinicians and requires a myriad of...
Pyrexia of unknown origin can be caused due to numerous infective and noninfective causes. It poses a diagnostic dilemma to the clinicians and requires a myriad of investigations for the confirmation of diagnosis. Thymomas are rare mediastinal tumors that present as anterior mediastinal mass; however, thymomas presenting as pyrexia of unknown origin has rarely been reported in the literature. We report an interesting case of a middle-aged male who presented as pyrexia of unknown origin due to thymoma.
Topics: Humans; Thymoma; Male; Fever of Unknown Origin; Thymus Neoplasms; Middle Aged; Tomography, X-Ray Computed
PubMed: 38769812
DOI: 10.4103/ijpm.ijpm_686_21