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Introduction. The association between Sjögren's syndrome (SS) and the development of lymphoma is well known. The prevalence of lymphoma in patients with SS is 5%. Mucosaassociated lymphoid tissue (MALT) lymphoma is the most common lymphoma type in patients with SS. It is common for MALT lymphoma to develop in the stomach, while it is extremely rare in the thymus. Case report. We present a 61-year-old Caucasian male patient with primary SS, cutaneous vasculitis, and thymic MALT lymphoma. The patient had a two-year history of diffuse cutaneous palpable purpura on legs, intermittently enlarged left parotid gland, and dry mouth. The results of Schirmer's test were positive, labial salivary glands biopsy revealed a focus score = 1, serology testing showed positive anti-Ro/SS-A and anti-La/SS-B antibodies, while skin biopsy findings revealed leukocytoclastic vasculitis. Diagnosis of primary SS with extraglandular cutaneous manifestations was confirmed. Cryoglobulinemia (Cg) and monoclonal gammopathy (MG) were detected, which increased the suspicion of hematological malignancy, and additional diagnostic procedures were performed. Computed tomography of the chest revealed an enlarged, multicystically altered anterior mediastinal mass. A thymectomy was performed through video-assisted thoracic surgery. Histological findings of the tissue confirmed the presence of tumor tissue consistent with MALT lymphoma in the thymus. Induction therapy with pulse doses of glucocorticoids was applied for three days, which was continued with medium doses of the drug. The doses were gradually reduced, and hydroxychloroquine was introduced. This has shown to be an effective therapy against features of SS. Postoperative local radiotherapy was performed. Conclusion. In SS patients with CV and in the presence of Cg and MG, attention should also be paid to the eventual development of MALT lymphoma, including the rare localization in the thymus. [ABSTRACT FROM AUTHOR]

Background: Thymic cysts are a rare benign disease that needs to be distinguished from low-risk thymoma. [18F]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 [18F]FDG PET/CT for differentiating between thymic cysts and low-risk thymomas. Methods: Patients who underwent preoperative [18F]FDG PET/CT followed by thymectomy for a thymic mass were retrospectively analyzed. The visual [18F]FDG PET/CT findings evaluated were PET visual grade, PET central metabolic defect, and CT shape. The quantitative [18F]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 [18F]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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Metastasis from one neoplasm to another is referred to as tumor‐to‐tumor metastasis (TTM). TTM is rarely observed. Here, we present a patient with TTM from a thymic carcinoma to an ovarian mature teratoma. A 25‐year‐old woman, diagnosed with unresectable thymic carcinoma, presented with a cyst with a solid tumor component in her right ovary. Laparoscopic cystectomy of the right ovary revealed that the solid tumor was a distant metastasis of the thymic carcinoma in an ovarian mature teratoma. The possibility of malignant transformation of the ovarian mature teratoma was ruled out, enabling accurate staging of the thymic carcinoma. This case emphasizes the need for clinicians to consider TTM and the importance of pathological confirmation of TTM when investigating potential distant metastases. [ABSTRACT FROM AUTHOR]

Simple Summary: This study aimed to identify the role of radiotherapy (RT) in the treatment of thymic carcinoma as well as the optimal RT target volume. A total of 116 patients who received multimodal treatment including RT with or without surgery or chemotherapy were included. The RT target volume was defined as the tumor bed or gross tumor with margin, and selective irradiation of the nodal area was performed when involved. The 5-year local recurrence-free survival rate was 94.7%. Overall, 53 recurrences were observed, of which distant metastasis was the most common. No isolated infield or marginal failures were observed. No infield nodal failure occurred among the patients who had initial node metastases and received RT including the nodal areas. A high local control rate was achieved with the addition of RT. A target volume confined to the tumor bed or gross tumor plus margin with the involved lymph node stations seems reasonable. Introduction: We aimed to identify the role of radiotherapy (RT) in the treatment of thymic carcinoma as well as the optimal RT target volume. Materials and Methods: This single-institution retrospective study included 116 patients diagnosed with thymic carcinoma between November 2006 and December 2021 who received multimodal treatment including RT with or without surgery or chemotherapy. Seventy-nine patients (68.1%) were treated with postoperative RT, 17 patients (14.7%) with preoperative RT, 11 patients (9.5%) with definitive RT, and nine patients (7.8%) with palliative RT. The target volume was defined as the tumor bed or gross tumor with margin, and selective irradiation of the regional nodal area was performed when involved. Results: With a median follow-up of 37.0 (range, 6.7–174.3) months, the 5-year overall survival (OS), progression-free survival, and local recurrence-free survival rates were 75.2%, 47.7% and 94.7%, respectively. The 5-year OS was 51.9% in patients with unresectable disease. Overall, 53 recurrences were observed, of which distant metastasis was the most common pattern of failure (n = 32, 60.4%) after RT. No isolated infield or marginal failures were observed. Thirty patients (25.8%) who had lymph node metastases at the initial diagnosis had regional nodal areas irradiated. There was no lymph node failure inside the RT field. A tumor dimension of ≥5.7 cm (hazard ratio [HR] 3.01; 95% confidence interval [CI] 1.25–7.26; p = 0.030) and postoperative RT (HR 0.20; 95% CI 0.08–0.52; p = 0.001) were independently associated with OS. Intensity-modulated-RT-treated patients developed less overall toxicity (p < 0.001) and esophagitis (p < 0.021) than three-dimensional-conformal-RT-treated patients. Conclusions: A high local control rate was achieved with RT in the primary tumor sites and involved lymph node area in the treatment of thymic carcinoma. A target volume confined to the tumor bed or gross tumor plus margin with the involved lymph node stations seems reasonable. The advanced RT techniques with intensity-modulated RT have led to reduced RT-related toxicity. [ABSTRACT FROM AUTHOR]

BACKGROUND: Lipofibroadenoma of the thymus is a rare tumor, with 12 cases reported in the literature. It is composed of normotypic thymic elements, mature adipose tissue, and fibrosis. It may be associated with thymoma. CLINICAL CASE: Male patient, 77 years old, with an incidental rounded tumor of 5.1 cm in the anterior superior mediastinum, composed of adipose tissue and fibrous septa. Histology consists of anastomosing and cystic epithelial structures with loose fibroconnective stroma and mature adipose tissue. The epithelium is made up of ovoid epithelial cells, pale cytoplasm, and a vesicular nucleus with up to two nucleoli; without mitosis neither necrosis. Immunohistochemistry: Cytokeratins AE1/AE3 and 5/6 positive with CD5, CD117 and TTF1 negative in epithelial component. The patient evolved favorably, without complications. CONCLUSION: This case is the first reported in Latin America and the oldest worldwide. Its clinicopathological characteristics are like those reported. Good evolution supports its benign behavior. [ABSTRACT FROM AUTHOR]

A 42‐year‐old female patient with intellectual disability was presented to us as a newly diagnosed case of thymoma. She was identified as a case of multiple primary cancers, including adenocarcinoma of the rectum, carcinoma of the breast, and mediastinal thymoma, in a 15‐year period, who underwent chemotherapy, radiotherapy, and surgical resection. [ABSTRACT FROM AUTHOR]

Objectives: We aimed to evaluate the diagnostic ability for the prediction of histologic grades and prognostic values on recurrence and death of pretreatment 2-[18F]FDG PET/CT in patients with resectable thymic epithelial tumours (TETs). Methods: One hundred and fourteen patients with TETs who underwent pretreatment 2-[18F]FDG PET/CT between 2012 and 2018 were retrospectively evaluated. TETs were classified into three histologic subtypes: low-risk thymoma (LRT, WHO classification A/AB/B1), high-risk thymoma (HRT, B2/B3), and thymic carcinoma (TC). Area under the receiver operating characteristics curve (AUC) was used to assess the diagnostic performance of PET/CT variables (maximum standardised uptake value [SUVmax], metabolic tumour volume [MTV], total lesion glycolysis [TLG], maximum diameter). Cox proportional hazards models were built using PET/CT and clinical variables. Results: The tumours included 52 LRT, 33 HRT, and 29 TC. SUVmax showed good diagnostic ability for differentiating HRT/TC from LRT (AUC 0.84, 95% confidence interval [CI] 0.76 − 0.92) and excellent ability for differentiating TC from LRT/HRT (AUC 0.94, 95% CI 0.90 − 0.98), with significantly higher values than MTV, TLG, and maximum diameter. With an optimal cut-off value of 6.4, the sensitivity, specificity, and accuracy for differentiating TC from LRT/HRT were 69%, 96%, and 89%, respectively. In the multivariable Cox proportional hazards analyses for freedom-from-recurrence, SUVmax was an independent prognostic factor (p < 0.001), whereas MTV and TLG were not. SUVmax was a significant predictor for overall survival in conjunction with clinical stage and resection margin. Conclusion: SUVmax showed excellent diagnostic performance for prediction of TC and significant prognostic value in terms of recurrence and survival. Key Points: • Maximum standardised uptake value (SUVmax) shows excellent performance in the differentiation of thymic carcinoma from low- and high-risk thymoma. • SUVmax is an independent prognostic factor for freedom-from-recurrence in the multivariable Cox proportional hazard model and a significant predictor for overall survival. • 2-[18F]FDG PET/CT can provide a useful diagnostic and prognostic imaging biomarker in conjunction with histologic classification and stage and help choose appropriate management for thymic epithelial tumours. [ABSTRACT FROM AUTHOR]

Objectives: The amount and distribution of intratumoural collagen fibre vary among different thymic tumours, which can be clearly detected with T2- and diffusion-weighted MR images. To explore the incidences of collagen fibre patterns (CFPs) among thymomas, thymic carcinomas and lymphomas on imaging, and to evaluate the efficacy and reproducibility of CFPs in differential diagnosis of thymic tumours. Materials and methods: Three hundred and ninety-eight patients with pathologically diagnosed thymoma, thymic carcinoma and lymphoma who underwent T2- and diffusion-weighted MR imaging were retrospectively enrolled. CFPs were classified into four categories: septum sign, patchy pattern, mixed pattern and no septum sign. The incidences of CFPs were compared among different thymic tumours, and the efficacy and reproducibility in differentiating the defined tumour types were analysed. Results: There were significant differences in CFPs among thymomas, thymic squamous cell carcinomas (TSCCs), other thymic carcinomas and neuroendocrine tumours (OTC&NTs) and thymic lymphomas. Septum signs were found in 209 (86%) thymomas, which differed between thymomas and any other thymic neoplasms (all p < 0.005). The patchy, mixed patterns and no septum sign were mainly seen in TSCCs (80.3%), OTC&NTs (78.9%) and thymic lymphomas (56.9%), respectively. The consistency of different CFP evaluation between two readers was either good or excellent. CFPs achieved high efficacy in identifying the thymic tumours. Conclusion: The CFPs based on T2- and diffusion-weighted MR imaging were of great value in the differential diagnosis of thymic tumours. Key Points: • Significant differences are found in intratumoural collagen fibre patterns among thymomas, thymic squamous cell carcinomas, other thymic carcinomas and neuroendocrine tumours and thymic lymphomas. • The septum sign, patchy pattern, mixed pattern and no septum sign are mainly seen in thymomas (86%), thymic squamous cell carcinomas (80.3%), other thymic carcinomas and neuroendocrine tumours (79%) and thymic lymphomas (57%), respectively. • The collagen fibre patterns have high efficacy and reproducibility in differentiating thymomas, thymic squamous cell carcinomas and thymic lymphomas. [ABSTRACT FROM AUTHOR]

Background: Breast cancer is becoming a common disease in women. It progresses slowly and may recur after a long time. Therefore, when a tumor is found in the chest of a patient with a history of breast cancer, an immediate concern is whether it is a primary tumor or a metastatic tumor. However, mediastinal metastasis is extremely unlikely to occur before lung metastasis, and breast cancer is not likely to have a solitary mediastinal metastasis. Additionally, patients should not undergo invasive procedures unnecessarily, so careful consideration is required. Case Reports: We present 2 cases. In case 1, a 48-year-old woman with a history of breast cancer had a mediastinal tumor. Based on imaging findings, cystic thymoma was suspected. Thoracoscopic intraoperative rapid biopsy showed a lymphocyte-predominant tumor tissue; therefore, the tumor was resected via a median sternotomy. The final pathological diagnosis was breast cancer metastasis. In case 2, a 47-year-old woman who underwent breast cancer resection 15 years earlier was referred for upper limb edema. Based on imaging findings, a left medial vein occlusion due to mediastinal tumor was diagnosed. Our experience in case 1 suggested that a biopsy alone should be performed first. A tumor biopsy was performed through a small transverse neck incision in case 2, and the final diagnosis was metastatic breast cancer of the mediastinum. Conclusions: In patients with a suspected primary mediastinal tumor on imaging, the possibility of a metastatic tumor should be considered if they have a history of breast cancer, regardless of how long in the past it was. [ABSTRACT FROM AUTHOR]

Background: Thymic carcinoma is a rare malignant neoplasm. High-grade thymic carcinoma has a high recurrence rate following surgery, and a low 5-year survival rate. Approximately 30% of patients with thymic carcinoma will be asymptomatic at the time of diagnosis. Extrathoracic metastasis on presentation is uncommon. Treatment of the primary tumor includes surgery, chemotherapy, and fractionated radiation. A rare case of thymic carcinoma that presented with bone and cerebral metastases is reported in a patient who responded well to stereotactic radiosurgery and chemotherapy. Case Report: A 63-year-old woman presented to the hospital for evaluation of hip pain. She was diagnosed with a lytic bone lesion of the right femur and brain metastasis. Biopsies from the mediastinal mass and right femur showed histological features consistent with carcinoma. Immunohistochemistry showed positive immunostaining of the tumor cells for the c-kit receptor (CD117) and CD5, supporting a diagnosis of stage IVb thymic carcinoma. Treatment included stereotactic radiosurgery, which delivered multiple radiation beams to the tumor tissue from different directions to target the tumor without affecting normal tissues. She was treated as an outpatient with carboplatin and taxol after stereotactic radiosurgery. The patient recovered well following treatment. Conclusions: A case of thymic carcinoma with bone and cerebral metastases was successfully treated with stereotactic radiosurgery and chemotherapy. [ABSTRACT FROM AUTHOR]

View large Download slide View large Download slide OBJECTIVES We queried the European Society of Thoracic Surgeons (ESTS) prospective thymic database for descriptive analysis and for comparison with the ESTS retrospective thymic database (1990–2010). METHODS Data were retrieved (January 2007–November 2017) for 1122 patients from 75 ESTS institutions. RESULTS There were 484 (65%) thymomas, 207 (28%) thymic carcinomas and 49 (7%) neuroendocrine thymic tumours. Staging (Masaoka) included 483 (67%) stage I and II, 100 (14%) stage III and 70 (10%) stage IV tumours. The new International Association for the Study of Lung Cancer/International Thymic Malignancies Interest Group tumour, node and metastasis (TNM) classification was available for 224 patients and including 177 (85%) stage I–II, 37 (16%) stage IIIA and 10 (4%) stage IIIB tumours. Chemotherapy as induction and adjuvant treatment was used in 14% and 15% of the patients. Radiotherapy was almost exclusively used postoperatively (24%). A minimally invasive surgical approach (video-assisted thoracic surgery/robotic-assisted thoracic surgery) was used in 276 (33%) patients. The overall recurrence rate was 10.8% (N  = 38). Compared to the ESTS retrospective database, the increased prevalence of thymic carcinomas (from 9% to 28%) and neuroendocrine thymic tumours (from 2% to 7%), an increase in the use of minimally invasive techniques (from 6% to 34%) and a wider use of chemotherapy as induction (from 9% to 15%) and adjuvant (from 2% to 16%) treatment were observed in the prospective database. The introduction of a set of variables considered essential for the data use ('minimum dataset') resulted in an increased average completeness rate. CONCLUSIONS The reported data from the ESTS prospective thymic database confirm the recent trends in the management of thymic tumours. The ESTS prospective thymic database represents a powerful resource open to all ESTS members for the global effort to manage these rare tumours. [ABSTRACT FROM AUTHOR]

Metaplastic thymoma is an extremely rare tumor. To date, only 17 cases of metaplastic thymoma have been reported. To the best of the authors’ knowledge, this is the second reported case of a sarcomatoid carcinoma arising in metaplastic thymoma; the carcinoma in this case is larger than that in the previous case. A 63-year-old woman with cough and asthenia for 2 weeks was admitted to the hospital. Computed tomography (CT) revealed a giant mass on the right side of the front mediastinum medium. The mediastinal tumor was excised, and additional pathological examinations, immunohistochemical tests, and electron-microscopic tests were performed. The tumor was diagnosed as a sarcomatoid carcinoma arising in metaplastic thymoma. Here, the authors discuss the clinical pathology of the sarcomatoid carcinoma arising in metaplastic thymoma and describe the biological behaviors with respect to the pathological features. [ABSTRACT FROM PUBLISHER]

Thymolipoma is a rare benign slow-growing encapsulated tumor of anterior mediastinum that accounts for 2–9% of all thymic neoplasms. About 30–50% of them are asymptomatic and found incidentally. Less than 200 cases have been published in the world literature so far. Here we report a case of thymolipoma of an adult female presented with progressive dyspnea, chest tightness and non-productive chough for 2-month duration. All blood investigations were normal. Radiological examinations suggested the presence of fat and soft tissue within the tumor without invading the neighboring structures. We concluded that the only curative treatment of thymolipoma is surgical excision. Unfortunately, the patient did not give consent despite telling her the outcomes if we do not do the surgical procedure, and a week later she passed away due to respiratory distress secondary to the mass effect of the tumor. [ABSTRACT FROM AUTHOR]

Background Despite being a rare disease entity, Thymoma and Thymic carcinomas represent the most common cause of anterior mediastinal mass. Clinical course is widely varied from an indolent course to florid metastatic disease with paraneoplastic manifestation. Treatment guidelines are based on small phase 2 studies. Data of chemotherapy in advanced thymoma is limited. Methods It is a retrospective study of advanced thymic epithelial tumors treated with chemotherapy over a period of 7 years (2011 -2017). All patients with unresectable /metastatic thymic tumours and who have received chemotherapy as were included in the study. Independent review of radiology images and histopathology slides were performed. Results There were 55 cases registered with thymic malignancy and 32 cases were analyzed for present study. Among them 27 cases were thymoma, 3 were thymic carcinoma and 2 were thymic NET. The median age was 39 years (22 65 years). Male: female ratio was 3: 1. The median duration of symptoms was 5.9 months (0-36 months). Twelve cases (38%) had features of superior vena cava obstruction at presentation. The mean size of mediastinal mass was 8.9 + 3.26 (range 2-15 cm). More than half of the cases (56%) showed calcification. Mediastinal lymph nodes were seen in 14 patients (46%). There was pleural involvement in 14 patients (73%) and pericardial involvement in 13 cases (43%). Pleural effusion was seen in 17(53%). Only 5 cases had stage III, 18 had stage Iva and 9 had stage IVb disease. The most common histological subtype as per WHO classification was B1 in 11 patients (34%) followed by B3 in 9 patients (28%) in our cohort. Out of 32 cases 3 have had upfront R2 resection. Post chemotherapy, one case attained CR, 10 had PR, 13 had SD and 3 had PD. 6 cases underwent surgery post induction therapy. 3 had R1 resection and 3 had R2 resection. Cisplatin, doxorubicin and cyclophosphamide (CAP) remains first choice for chemotherapy in 81% patients followed by paclitaxel-carboplatin in 12.5%. Radiation therapy was given to 13 patients. After a median follow up of 24.86 months, median PFS is 16.1 months and median OS was not reached. Conclusions Thymic malignancies are a heterogeneous group of disorders. Multi-modality treatment including chemotherapy is an effective and feasible treatment for advanced thymoma. Legal entity responsible for the study All authors are equally responsible. Institute ethics committee has given the clearance. Funding Has not received any funding. Disclosure All authors have declared no conflicts of interest. [ABSTRACT FROM AUTHOR]