Robotic portal resection for mediastinal tumours: a prospective 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.

Clinical evaluation of the impact of mediastinal tumour size on the subxiphoid approach video-assisted thoracoscopic surgery.

OBJECTIVES: The application of video-assisted thoracoscopic surgery (VATS) for relatively large mediastinal tumours (≥5.0 cm) has been a subject of debate, and few studies have investigated the subxiphoid approach VATS in different tumour size categories. The study aims to compare the efficacy of the subxiphoid approach VATS for achieving curative outcomes based on tumour size categories (<3.0, 3.0-4.9 and 5.0-10.0 cm). METHODS: A total of 165 patients with anterior mediastinal tumours who underwent surgery at our hospital between January 2018 and July 2022 were consecutively enrolled, categorized according to tumour size-group A (<3.0 cm): 58, group B (3.0-4.9 cm): 70 and group C (5.0-10.0 cm): 37. Clinical baseline data, intraoperative and postoperative outcomes, and postoperative complications were analysed. RESULTS: The study revealed significant differences in operation time among the 3 groups (group A: 103.4 ± 36.1, group B: 106.4 ± 35.2, group C: 127.4 ± 44.8; P < 0.05) as well as in the volume of drainage (group A: 273.3 ± 162.0, group B: 411.9 ± 342.6, group C: 509.7 ± 543.7; P < 0.05). However, no differences were seen in blood loss, drainage duration, postoperative hospital stay and duration of postoperative oral analgesics. Additionally, the incidence of postoperative complications did not exhibit significant differences across these groups. CONCLUSIONS: Subxiphoid approach VATS is considered a feasible and safe surgical method for large-sized anterior mediastinal tumours (5.0-10.0 cm) with no invasion to the surrounding tissues and organs.

Mediastinal epithelioid inflammatory myofibroblastic sarcoma with the EML4-ALK fusion: A case report and literature review.

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is an aggressive subtype of inflammatory myofibroblastic tumour which rarely affects the chest cavity. We, for the first time, report a case of mediastinal EIMS with the EML4-ALK fusion. A young woman presented to our hospital with cough, chest tightness and shortness of breath. Computed tomography (CT) showed a mixed attenuation soft-tissue mass in the right middle and upper mediastinum. Negative results were obtained from bronchoscopy forceps biopsy and endobronchial ultrasound-guided transbronchial fine needle aspiration. CT-guided percutaneous biopsy was finally performed. However, due to the rapidly progressed EIMS that compressed the trachea and right main bronchus, the patient died of respiratory failure 1 day before diagnosis. EIMS progresses rapidly, and an early diagnosis is important. For mediastinal EIMS, CT-guided percutaneous biopsy may be useful. Next-generation sequencing of blood may be instructive to EIMS patients who are intolerant to invasive biopsy.

Robot-assisted thoracoscopic surgery for mediastinal tumours in children: a single-centre retrospective study of 149 patients.

OBJECTIVES: The purpose of this retrospective study was to summarize our experience in performing robot-assisted thoracoscopic surgery (RATS) for mediastinal tumours in children to investigate its safety and feasibility. METHODS: This retrospective study involved 149 patients with mediastinal tumours who were hospitalized in the Department of Thoracic Surgery of Beijing Children's Hospital, Capital Medical University, and underwent RATS for tumour resection from March 2021 to November 2022. Information on patient age, weight, tumour size, surgical incision selection, operative time, intraoperative bleeding, intraoperative complications, length of hospital stay, rate of conversion to thoracotomy and follow-up conditions were summarized. RESULTS: All 149 surgeries were successfully completed with no cases of mortality. There were 77 male and 72 female patients, with a mean age of 5.9 years (range: 6 months-16 years, 8 months) and a mean weight of 23.6 kg (8.0-72.0 kg). The mean maximum tumour diameter was 5.5 cm (2.0-12.0 cm), the mean operative time was 106.7 min (25.0-260.0 min), the mean intraoperative bleeding volume was 11.3 ml (1.0-400.0 ml) and the mean hospital stay was 7.2 days (4.0-14.0 days). All patients recovered well with no cases of tumour recurrence or mortality during the postoperative follow-up period (3-23 months). CONCLUSIONS: RATS is safe and feasible to apply in children with mediastinal tumours who are >6 months of age and weigh more than 8 kg in terms of short-term outcomes, but longer-term follow-up is needed to fully evaluate the benefits. For cases that are associated with greater surgical difficulty and risk, a comprehensive surgical plan should be fully prepared in advance of surgery.

A successful case of robot-assisted thoracic surgery for apical thoracic schwannoma.

The thoracic apex is a narrow and complicated area with nerves and vessels, making it difficult to secure a visual field and perform surgical operations. Robot-assisted surgery enabled good visibility and highly flexible forceps manipulation, we were able to perform minimally invasive and safe thoracic apical tumour resection.

[Feasibility of cardiac surgical techniques for intrathoracic tumors]. / O tselesoobraznosti ispol'zovaniya kardiokhirurgicheskikh tekhnologii pri vnutrigrudnykh opukholyakh.

OBJECTIVE: To analyse safety and expediency of cardiac surgical technologies including cardiopulmonary bypass (CPB) in patients with locally advanced lung cancer and invasive tumors of the mediastinum. MATERIAL AND METHODS: Cardiac surgical techniques and CPB were used in 23 patients (group 1) with locally advanced thoracic tumors between 2005 and 2015. For the same period, there were 22 patients (group 2) who underwent combined surgeries and could have had similar techniques. However, these techniques were not used for various reasons. Mediastinal malignancies and non-small cell lung cancer were diagnosed in 26 (57.8%) and 19 (42.2%) patients, respectively. Invasion of superior vena cava (n=15), aorta (n=13) and pulmonary artery (n=12) was the most common. Lesion of innominate vein (n=8), left atrium (n=6) and innominate artery (n=4) was less common. A total of 21 pneumonectomies were performed (14 in the first group and 7 in the second group). Lobectomy was less common (one patient in each group). Sublobar lung resection was performed in 10 patients (2 patients in the first group and 8 ones in the second group). All resections were total in the first group (R0) that was confirmed by routine morphological examination of resection margins of different organs and vessels. The situation was worse in the second group (R1 in 19 (86.4%) patients, R2 in 3 (13.6%) patients). RESULTS: Total postoperative morbidity was 53.3%, mortality - 8.2%. These values are higher compared to patients undergoing surgical treatment for thoracic malignancies. Incidence of postoperative complications was higher in the first group (16 (69.6%) and 8 (36.4%), respectively). Four patients died in the first group. Sepsis (n=2), acute right ventricular failure (n=1) and acute myocardial infarction (n=1) caused death. There were no lethal outcomes in the second group. Various postoperative complications were diagnosed only in 8 (36.4%) patients. The long-term results were followed-up in 80% of patients. In the first group, 3- and 5-year survival rates were 30.5% and 25%, respectively (median 43.8 months). In the second group, these values were 25% and 2%, respectively (median 24.9 months). Long-term mortality in the second group was caused by progression of malignant process, including local recurrence, after palliative surgery (R1, R2 resection). CONCLUSION: Higher risk of postoperative complications and mortality in patients undergoing on-pump surgery is compensated by significantly better long-term results. Further progress is associated with higher safety of CPB, as well as solving some organizational and educational problems.

Robotic excision of posterior mediastinal neurogenic tumours: Technique and surgical outcomes.

Introduction: Neurogenic tumours are the most common tumours of the posterior mediastinum and account for 75% of the tumours in this region. Till recently, open transthoracic approach has been the standard of care for their excision. Thoracoscopic excision of these tumours is being commonly employed because of lesser morbidity and shorter hospital stay. The robotic surgical system offers a potential advantage over conventional thoracoscopy. We herein report our technique and surgical outcomes of excision of posterior mediastinal tumours using the Da Vinci Robotic Surgical System. Materials and Methods: We retrospectively reviewed 20 patients who underwent Robotic Portal-Posterior Mediastinal Tumour (RP-PMT) Excision at our centre. The demographic data, clinical presentation, characteristics of the tumour, operative and post-operative variables including, total operative time, blood loss, conversion rate, duration of the chest tube, hospital stay and complications were noted. Results: Twenty patients underwent RP-PMT Excision and were included in the study. The median age was 41.2 years. The most frequent presentation was chest pain. Schwannoma was the most common histopathological diagnosis. There were two conversions. The total operative time was 110 min with an average blood loss of 30 mL. Two patients developed complications. The postoperative hospital stay was 2.4 days. With a median follow-up of 36 months (6-48 months), all except patients are recurrence-free, except the patient with malignant nerve sheath tumour who developed local recurrence. Conclusion: Our study demonstrates the feasibility and safety of robotic surgery for posterior mediastinal neurogenic tumours with good surgical outcomes.

Clinico-Pathological and Radiological Spectrum of Mediastinal Masses in a Tertiary Care Center: A Cross-Sectional Study.

Introduction The phrase "mediastinal mass" refers to a mass within the mediastinum. About 50% of all mediastinal masses, including teratoma, thymoma, lymphoma, and thyroid illness, are anterior mediastinal tumors. Data on the mediastinal mass in India are relatively sparse, especially in this region, compared to those from other countries. Mediastinal masses are very infrequent lesions that might occasionally present a diagnostic and therapeutic challenge to the doctor. The current study describes the socio-demographic characteristics, symptoms, diagnosis, and location of mediastinal mass among the study participants. Methodology We carried out a retrospective, cross-sectional study in a tertiary care center in Chennai for three years. We included patients with an age above 16 years who visited the tertiary care center in Chennai during the study period. We included all patients with a mediastinal mass diagnosed by CT scan, with or without signs and symptoms of mediastinal compression. Patients under the age of 16 and those with insufficient data were both excluded from the study. As per the universal sampling technique, we included all the patients who met the eligibility criteria during the study period (three years) as study subjects. By using the hospital records, we collected all data about the patients like socio-demographic data, presenting complaints, past history, x-ray findings, and co-morbidities. Similarly, we recorded blood parameters, pleural fluid parameters, and histopathological reports from the laboratory register. Results The mean age of the study participants was 41.11 years, with a high proportion of patients belonging to the age group of 21 to 30 years. Over 70% of the study participants were male. Only about 54.5% of the study participants had symptoms because of a mediastinal mass. The most common local symptom felt by the patients was dyspnea, followed by a dry cough. Weight loss was the most common symptom for the patients. Most study participants (47.7%) had seen a doctor within one month of the onset of symptoms. About 4.5% of the patients had pleural effusion, as diagnosed by x-ray. Most of the study participants had a mass in the anterior mediastinum, followed by the posterior mediastinum. Most of the participants (15.9%) had non-caseating granulomatous inflammation suggestive of sarcoidosis.  Conclusion The most common tumor found in our study was lymphoma, which was followed by non-caseating granulomatous disease and thymoma. Anterior compartments are most commonly involved. We observed the most common presentation in the third decade of life with a male to female ratio of 2:1, with dyspnea being the most common symptom, followed by a dry cough. Our study found 4.5% of the patients had pleural effusion as a complication.

Lambert-Eaton myasthenic syndrome with primary thymic marginal zone B-cell lymphoma: A case report.

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune paraneoplastic syndrome with proximal muscle weakness, that often complicates small cell lung cancer. It is known that neurological symptoms do not improve with malignancy treatment alone in many LEMS patients, therefore treatment is often difficult. Since Lambert-Eaton myasthenic syndrome is a rare disease with a frequency of about 1/100 that of myasthenia gravis, there are only a few case reports on malignancy complications other than small cell lung cancer. We report a LEMS patient in his 40s who was found to have an anterior mediastinal mass. We performed surgical resection and confirmed the diagnosis of primary thymic marginal zone B-cell lymphoma by pathological diagnosis using immunostaining. Thymectomy and malignant lymphoma treatment with rituximab had no effect on neurological symptoms. The neurological symptoms improved only after we provided comprehensive care with the haematology, neurology, and rehabilitation department.

EPIPERICARDIAL FAT NECROSIS.

Introduction: Epipericardial fat necrosis (EFN) is a relatively rare benign disease that causes acute chest pain. Case Description: A woman in her 20s presented with acute left shoulder and epigastric pain. One day before presentation, she had visited a cardiologist and an acute coronary syndrome had been ruled out. The pain worsened with deep inspiration. Chest computed tomography (CT) showed a soft-tissue attenuation lesion containing a fatty centre located in the epipericardial fat at the left cardiophrenic angle. Hence, EFN was diagnosed and the pain was resolved with loxoprofen. The lesion had disappeared on a follow-up chest CT scan. Discussion: EFN is a rare benign disease that causes acute chest pain. Approximately 70-90% of EFN cases are misdiagnosed by clinicians as other diseases. Conclusion: In patients with acute chest pain, the correct diagnosis of EFN avoids unnecessary invasive investigations and reassures patients. LEARNING POINTS: Patients with epipericardial fat necrosis typically present with acute pleural chest pain without any associated symptoms.Characteristic CT findings of the encapsulated fatty pericardial lesion with a surrounding inflammatory reaction are key for the diagnosis of epipericardial fat necrosis.The correct diagnosis of epipericardial fat necrosis in patients with acute chest pain reassures them and avoids unnecessary invasive investigation.

Diffuse large B-cell lymphoma with cardiac invasion diagnosed using transesophageal ultrasound-guided bronchoscopic aspiration.

Transesophageal ultrasound-guided bronchoscopic aspiration (EUS-B-FNA) allowed for minimally invasive and simultaneous diagnosis and evaluation of the degree of invasion by echocardiography. EUS-B-FNA may be useful for the evaluation and diagnosis of tumours with cardiac invasion.

Thoracoscopic management of posterior mediastinal neurogenic tumours.

Background: This study describes the surgical technique of thoracoscopic resection of posterior mediastinal neurogenic tumours and reporting the surgical outcomes. Methods: This is a retrospective analysis of 21 patients operated over 7 years in a dedicated thoracic surgery centre. The demographic and post-operative parameters along with complications were recorded and analysed. Results: Twelve patients had right-sided tumours, while 9 had left-sided lesions, and 9 were on the left side. The most common diagnosis was schwannoma (n = 15, 71.42%), followed by neurofibroma (n = 4, 19.04%). The average surgery duration was 104 min (85-135 min), and the mean blood loss was 120 ml (25 ml-250 ml). The average lesion size was 4.8 cm (2 cm-7 cm). Conversion to open procedure was required in one patient. Two patients (14.2%) developed complications. One patient developed Horner's Syndrome and the other developed post-operative lung atelectasis. The median follow-up was 36 months (6-90 months). No recurrence was observed during the follow-up period. Conclusions: Thoracoscopic approach to posterior mediastinal neurogenic tumours is feasible and allows for low morbidity, short hospital stay and superior cosmesis.

IgG4-associated fibrosing mediastinitis requiring differentiation from posterior mediastinal tumour: A case report.

A 71-year-old man, who was found to have a posterior mediastinal tumour, was referred to our hospital. Contrast-enhanced computed tomography (CT) showed a 15-cm soft tissue shadow in the posterior mediastinum, with many affected areas and a gradually increasing pattern. We also detected oligemic areas with poor contrast-filling. There was no invasion into the adjacent vertebral body and the blood vessels penetrating the interior were intact. Positron emission tomography-CT revealed a high maximum standardized uptake level of 4.53 in the mediastinal masses. We performed thoracoscopic surgery for the biopsy. Histological findings showed lymphoplasmacytic infiltration in the fibrous stroma as well as storiform fibrosis. Immunohistochemical examination revealed abundant infiltration of immunoglobulin G4 (IgG4)-positive plasma cells and 40% IgG4/IgG-positive plasma cells. Postoperative serum examinations showed a high serum IgG4 level (570 mg/dl). Accordingly, we diagnosed the patient with IgG4-related fibrosing mediastinitis, a rare manifestation of IgG4-related disease.

Full subcostal subxiphoid robotic-assisted radical thymectomy: preclinical cadaveric study for optimizing patient positioning, table settings, and port configuration.

Robotic subxiphoid transthoracic thymectomy showed several surgical advantages. Nevertheless, the intercostal insertion of trocars may lead to nerve injury with enhanced post-operative pain. Moreover, the dissection of peri-diaphragmatic mediastinal fat may result challenging, in particular on left side, where the presence of the heart precludes the optimal visualization. We describe a preclinical cadaveric study of a novel full subcostal robotic-assisted technique to overcome these limitations. A total subcostal robotic-assisted radical thymectomy was evaluated on a cadaver model using the da Vinci Xi system. The exploratory procedure was divided in two steps: (a) dissection of the thymus gland, except the left mediastinal epi-diaphragmatic fat pad; (b) dissection of the left diaphragmatic mediastinal fat pad avoiding heart compression while perfectly visualizing the left phrenic nerve. Five different setups were explored based on camera and trocars insertions, patient's positioning and table's settings. Both the tasks were accomplished using the novel technique. The subxiphoid insertion of the camera and the position of two robotic arms about 8 cm distally on the subcostal made the most part of mediastinal dissection straightforward. Left peri-diaphragmatic fat pad can be better visualized and dissected positioning the camera in the left subcostal port shifting the instruments on the right side. This may permit a better control of the left phrenic nerve reducing heart compression. Full subcostal robotic-assisted thymectomy resulted feasible in cadaveric model. Clinical trial should be performed to confirm the translational use of this novel technique and the speculated advantages in living model.

Resection of a capillary haemangioma using robotic-assisted thoracic surgery: A case report.

Capillary haemangioma is a rare condition that is difficult to diagnose preoperatively because of its rarity and nonspecific imaging findings. In this report, we describe a case of capillary haemangioma diagnosed by robot-assisted thoracic surgery (RATS). A 72-year-old man was incidentally found to have an anterior mediastinal tumour on chest computed tomography. The preoperative imaging findings were indicative of thymoma, and surgical treatment by RATS was selected. The intraoperative findings suggested that the tumour was a haemangioma originating from the pericardiophrenic vein. The pathological findings revealed a well-defined tumour with capillaries in a vascular-like structure and some thrombus formation. The pathological diagnosis was capillary haemangioma. The patient was discharged unaided at 7 days postoperatively and no recurrence was observed at 16 months postoperatively.

A rare case of hepatic metastasis 20 years after surgical resection of a thymoma: A case report.

INTRODUCTION: Thymoma is the most common solid, primary mediastinal tumour, accounting for 20% of mediastinal neoplasms. Malignant thymus tumours (thymomas and thymic carcinomas) metastasize primarily locally. Distant metastases, especially to liver are very rare. PRESENTATION OF CASE: We review the case of a 59 year-old female patient, who underwent resection of the thymus with a diagnosed type B2 thymoma 20 years ago. The patient was referred to our hospital with a newly discovered space-occupying lesion in the liver, which had been detected in a routine follow-up magnetic resonance imaging scan. Since a malignant tumour of the liver could not be excluded, a conventional left hemihepatectomy was performed. Histological examination revealed a liver metastasis of the type B2 thymoma, which had been removed 20 years ago. CLINICAL DISCUSSION: The case was discussed in the interdisciplinary tumour board. Based on the very long history of the primary removal of the thymoma as well as the R0 resection of the liver metastasis, a follow-up regimen with CT scans on a regular basis was recommended. CONCLUSIONS: Newly discovered lesions of the liver in patients even with a long history of a thymoma should raise the suspicion of a liver metastasis that should be surgically resected as the therapy of choice. Further, this case indicates the importance for long-term radiographic follow-up.

Safety of subxiphoid uniportal video-assisted thoracoscopic surgery for anterior mediastinal tumour in obese patients.

INTRODUCTION: Subxiphoid uniportal video-assisted thoracoscopic surgery (VATS) has been gradually applied for the treatment of anterior mediastinal tumour (AMT). However, whether obesity is a risk factor for subxiphoid uniportal VATS for AMT is still unknown. AIM: To explore the safety and short-term outcome of subxiphoid uniportal VATS for AMT in obese patients. MATERIAL AND METHODS: The clinical data of 142 patients who received VATS via subxiphoid approach for AMT were analysed. According to body mass index (BMI), the patients were divided into an obese group (BMI ≥ 28 kg/m2) and a non-obese group (BMI < 28 kg/m2). Then, the clinical and surgical characteristics between the obese group and the non-obese group were analysed to explore the effect of obesity on VATS for AMT. The pain scores were evaluated by the Numeric Rating Scale. RESULTS: The operative time and tracheal intubation time using subxiphoid uniportal VATS for AMT in the obese group were longer than that in the non-obese group (p < 0.05). However, there was no obvious difference in intraoperative blood loss, chest tube drainage time, chest tube drainage volume, and length of hospital stay between the obese group and the non-obese group (p > 0.05). Moreover, there was also no significant difference in postoperative complications, including pulmonary complications, wound infection, arrhythmia, and pulmonary leak, between the obese group and the non-obese group. In addition, the pain scores in the obese group were similar to those in the non-obese group. CONCLUSIONS: Although obesity might prolong operative time of subxiphoid uniportal VAST for AMT, it does not increase the rate of postoperative complications. An experienced centre can properly conduct VAST via subxiphoid approach when treating AMT in obese patients.

Robot-assisted intrathoracic procedure for dumbbell tumour in the prone position.

A 24-year-old man presented with a dumbbell-shaped right posterior mediastinal mass. The patient was placed in the prone position following general anaesthesia and intubation. After laminectomy and dissection of the dorsal part of the tumour using a posterior approach were performed, the tumour was completely resected using a robotic approach in the thoracic cavity without repositioning. This is the first report of robotic resection for posterior mediastinal tumour in the prone position as well as a novel combined posterior approach and robotic resection for dumbbell tumours.

Mature mediastinal teratoma with somatic type malignancy including neuroblastoma and intestinal type of adenocarcinoma: A Case Report.

INTRODUCTION AND IMPORTANCE: The majority of mediastinal tumours develop asymptomatically and are often detected incidentally on a chest X-ray performed for another reason. Mediastinal tumours, although mostly asymptomatic, may cause non-specific symptoms associated with advanced tumour growth. CASE PRESENTATION: We present a case of a 30-year-old woman who presented with exhaustion and lower back pain accompanied by severe headaches with symptoms of visual disturbances, followed by the typical Horner syndrome. Computed tomography revealed a tumour measuring 12 × 11 × 10 cm in the right cavity with features suggestive of teratoma. The patient underwent mediastinal tumour resection and thymectomy. The pathomorphological examination confirmed the primary diagnosis of mediastinal teratoma, but rare somatic type malignancy was detected. Therefore, the patient was referred for further oncological treatment. DISCUSSION: Mediastinal teratoma is an uncommon finding and usually asymptomatic. Despite its slow growth, it can grow enough to compress adjacent structures, causing symptoms similar to those presented in our patient. CONCLUSION: Radiologic imaging proves diagnostic in most cases. Despite the somatic type malignancy, surgical excision of the tumour using the en-bloc technique seems to be a sufficient option for the patient, and further oncological treatment is not always obligatory.

Clear Cell Carcinoma of the Thymus: An Improbable Enemy.

INTRODUCTION: Thymic clear cell carcinoma is the most uncommon subtype of thymic carcinoma, with 20 cases reported worldwide. CASE DESCRIPTION: We present the case of a 61-year-old female with dyspnoea and chest pain for 2 days. Computed tomography (CT) angiography showed pulmonary thromboembolism and the existence of mediastinal and bilateral hilar lymphadenopathy, the largest infracarinal with an inferior axis of 25 mm, and also, micronodules on the left pulmonary parenchyma. The patient was admitted for aetiological assessment and underwent anticoagulant therapy. After a month, she had an ischaemic stroke, the sequelae of which proved to be fatal. The autopsy showed a mass in the superior-anterior mediastinum, with dimensions of 11×8×6 cm, corresponding to a thymus signet ring cell primary carcinoma. The immunohistochemistry study revealed that this mass was positive for AE1/AE3, CK5/6 and CK7. CONCLUSION: The clinical, morphological and immunophenotypic diversity of this tumour makes its diagnosis a difficult multidisciplinary challenge, which requires a high level of clinical knowledge and accurate imaging and histological investigation. LEARNING POINTS: Thymic clear cell carcinoma is a very rare entity with an aggressive and nonspecific clinical behaviour.There are no defined diagnostic criteria, although diagnosis could be established with histologic/cytology analysis.There are no clear guidelines for treatment, which can include highly invasive surgery and chemotherapy or radiation therapy.