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Thymoma is a rare tumor with a largely indolent growth pattern. It does, however, have malignant potential as a result of its ability to invade locally and metastasize regionally. Often associated with a number of immune- and nonimmune-mediated paraneoplastic syndromes, patient outcomes are directly related to stage of disease and the ability to ac...
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Thymomas are the most common tumours of the anterior mediastinum; their clinical course is often complicated by accompanying autoimmune and paraneoplastic syndromes. Advanced malignant thymoma is particularly challenging to manage owing to the lack of evidence from randomised trials to guide treatment. Combination first-line chemotherapy has been t...
Citations
... 1 Riedel et al. previously reported several cases of indolent thymoma, corroborating our observation that thymoma can exhibit a largely indolent growth pattern. 6 In this case, the decision to opt for a high-complexity fluoroscopy-guided pericardiocentesis was influenced by several key factors, each carrying significant implications for clinical practice. The patient's initial presentation included a series of concerning symptoms, most notably exertional dyspnea and a significant decline in functional status, necessitating immediate symptomatic relief. ...
... Thymomas are epithelial tumors originating from the thymus gland and the most common tumors of the anterior mediastinum (1). It is known that approximately 2-5% of patients with thymoma develop pure red cell aplasia (PRCA) (2,3), but the concurrent combination of acquired amegakaryocytic thrombocytopenia (AAMT) is rarely reported. ...
Thymoma combined with pure red cell aplasia (PRCA) and acquired amegakaryocytic thrombocytopenia (AAMT) has been rarely reported, often occurring in the initial stage of treatment and after chemotherapy or thymectomy, while PRCA and AAMT occurring after radiotherapy for thymoma has not been reported. The present study describes the case of a 42-year-old female patient with thymoma complicated by radiation-induced PRCA and AAMT after a rapid response to radiotherapy, who was in complete remission without recurrence after adjustment of initial symptomatic therapy to cyclosporine combined with prednisone. After 1 month, the patient underwent complete resection of mediastinal tumor. Next-generation sequencing revealed that the DNA damage repair pathway-related gene MSH3 was mutated, with p.A57P in abundance of 9.21%. To the best of our knowledge, the present study is the first to report that PRCA and AAMT secondary to thymoma after radiotherapy may be associated with increased sensitivity to radiotherapy caused by a mutation in the MSH3 gene.
... Thymoma is a slowly-growing epithelial tumor of thymus gland and also the most common tumor of the anterior mediastinum [1]. According to the RARE-CARE project definition [2], thymoma is a rare tumor accounting for 0.2% to 1.5% of all malignant tumors [3]. ...
Objective:
Thymoma is a slow-growing epithelial tumor of thymus gland. Its size is associated with its prognosis. The aim of this study was to analyze the prognostic correlation of tumor volume and complete resection of thymoma at different Masaoka-Koga stages.
Methods:
A retrospective study was carried out, using the data of 502 patients who underwent complete resection of thymectomy at Zhongshan Hospital, Fudan University, in Shanghai, China, from February 2009 to February 2016. The characteristics of the patients were collected. Using Masaoka-Koga staging system, patients were divided into four different subcohorts: Stage I, stage II, stage III and stage IVa/IVb. The relationship between tumor volume and postoperative recurrence was analyzed for each subcohort, using receiver operating curves, cutoff values were obtained. and patients were grouped according to the cutoff values. Survival analysis was performed with the help of Kaplan-Meier method, and the difference between the two survival curves was compared using log-rank test. Whether tumor volume could be used as an independent risk factor for thymoma prognosis was analyzed, using a univariate Cox proportional hazards model.
Results:
The area under the curve was 0.718, 0.740, 0.798, and 0.804 for the stage I, II, III, and IVa/IVb subcohorts, respectively, and the cutoff values of tumor volume for predicting recurrence were 47.90 cm3, 53.70 cm3, 76.35 cm3, and 89.05 cm3, respectively. Patients with tumor volumes greater than the cutoff values had significantly shorter recurrence-free survival than those with tumor volumes less than the cutoff values (p < 0.001). The results of the univariate Cox proportional hazards model indicated that tumor volume was an independent risk factor for thymoma prognosis and for postoperative prognosis of thymoma in Masaoka-Koga stage I (p < 0.001).
Conclusions:
Tumor volume is significantly correlated with the postoperative prognosis of thymoma in Masaoka-Koga stage I and can serve as an independent risk factor for predicting postoperative tumor recurrence.
... This female patient was diagnosed with thymoma with metastasis to both kidneys and lungs when she was Although her history of thymoma put her at an increased risk of myasthenia gravis [40], a test for muscle-specific receptor tyrosine kinase antibodies and for acetylcholine receptor binding antibodies performed later at age 46 years was negative. The onset of her symptoms began at age 45 years with lower back muscle weakness progressing to weakness of the leg muscles. ...
Background
Valosin containing protein (VCP) is an important protein with many vital functions mostly related to the ubiquitin–proteasome system that provides protein quality control. VCP-associated inclusion body myopathy with Paget disease of bone and frontotemporal dementia, also termed VCP disease and multisystem proteinopathy (MSP 1), is an autosomal dominant disorder caused by monoallelic variants in the VCP gene on human chromosome 9. VCP has also been strongly involved in cancer, with over-activity of VCP found in several cancers such as prostate, pancreatic, endometrial, esophageal cancers and osteosarcoma. Since MSP1 is caused by gain of function variants in the VCP gene, we hypothesized our patients would show increased risk for developing malignancies. We describe cases of 3 rare malignancies and 4 common cancers from a retrospective dataset.
Results
Upon surveying 106 families with confirmed VCP variants, we found a higher rate of rare tumors including malignant peripheral nerve sheath tumor, anaplastic pleomorphic xanthoastrocytoma and thymoma. Some of these subjects developed cancer before displaying other classic VCP disease manifestations. We also present cases of common cancers; however, we did not find an increased rate compared to the general population. This could be related to the early mortality associated with this disease, since most patients die in their 50–60 s due to respiratory failure or cardiomyopathy which is earlier than the age at which most cancers appear.
Conclusion
This is the first study that expands the phenotype of VCP disease to potentially include rare cancers and highlights the importance of further investigation of the role of VCP in cancer development. The results of this study in VCP disease patients suggest that patients may be at an increased risk for rare tumors. A larger study will determine if patients with VCP disease develop cancer at a higher rate than the general population. If that is the case, they should be followed up more frequently and screened for recurrence and metastasis of their cancer.
... Approximately 50% of patients with thymoma develop a paraneoplastic syndrome. 2,3 Myasthenia gravis is the most common thymoma-associated disorder, occurring in 24.5 -40 % of all patients with thymoma. 4 Thymoma-associated paraneoplastic encephalitis (TAPE) is a rare entity that can present with variable clinical presentations, antibody findings and MRI features. ...
A 59-year-old man first presented for an episode of left arm numbness. During workup, a thymoma was incidentally discovered and resected. The symptoms in his left arm were attributed to a cardiac pathology. One month later, he began to experience fatigue, weight loss, and anorexia, followed by one generalized tonic-clonic seizure. Workup including toxic and metabolic screening and MRI Brain were unremarkable. He was started on an anti-seizure medication and did well for two years, when his symptoms recurred. Repeat MRI Brain showed multiple cortical T2/FLAIR hyperintense lesions without enhancement or diffusion restriction. Further workup included spinal MRI, CT chest/abdomen/pelvis, CSF studies, autoimmune/paraneoplastic panels in CSF and serum, all of which were unremarkable. Serum testing was positive for striational antibodies, acetylcholine receptor (AChR) binding antibodies, and AChR modulating antibodies. He received high dose steroids and plasma exchange with resolution of his symptoms, and has since been stable on mycophenolate mofetil. This presentation highlights the rare association between thymoma and encephalitis. Prompt identification and treatment is critical. This article discusses the diagnostic approach to this rare presentation including essential features of the clinical presentation, appropriate workup, pertinent differential diagnoses, and key points for the treatment of these patients.
... The concern about secondary cancer from radiotherapy has been gradually gaining momentum inasmuch as cure rates and life expectancy after treatment have been increasing, owing to early cancer detection capability, major improvements in treatment technology and multidisciplinary cancer care. The concern is of particular relevance in the case of radiotherapy treatment of thymoma, the most common tumor of the anterior mediastinum (Riedel and Burfeind 2006), where cure rates and life expectancy are relatively high, and patient age at presentation relatively low (Strobel et al 2004). ...
Objective. This study aims at quantifying the lifetime attributable risk of secondary fatal cancer (LARFAC) to patients receiving adjuvant radiotherapy treatment for thymoma, a neoplasm where cure rates and life expectancy are relatively high, patient age at presentation relatively low and indications for radiotherapy controversial depending on the disease stage. Approach. An anthropomorphic phantom was scanned, organs were contoured and a standard 6 MV 3DCRT treatment plan was produced for thymoma treatment. The phantom was loaded with thermoluminescent dosimeters (TLDs) and treated by linear accelerator per plan. The TLDs were subsequently read for out-of-field dose distribution while in-field dose distribution was obtained from the planning system. Sex and age-specific lifetime radiogenic cancer risk was calculated as the sum of in-field risk and out-of-field risk. The latter risk was estimated using hybrid ICRP 2007 103-BEIR VII tables of organ-specific risks based on the linear-no threshold (LNT) model and applicable at low doses, while the former using mathematical risk models applicable at high doses. Main results. The LARFAC associated with a prescribed dose of 50 Gy to target volume in 25 fractions was in the approximate range of 1%–3%. The risk was higher for young and female patients. The largest contributing organ to this risk were the lungs by far. Using the LNT model inappropriately to calculate risk at therapeutic doses (in-field) would overestimate the risk up to tenfold. Significance. The LARFAC to patient from thymoma radiotherapy was quantified taking into consideration the inapplicability of the LNT model at therapeutic doses. The risk is not negligible; the information may be relevant to patients and clinicians.
... ymomas are rare epithelial tumors that commonly present with paraneoplastic syndromes, with up to 50% of thymoma patients presenting with associated immunodeficiencies and autoimmune phenomena [15]. A recent systematic review reports estimates of common paraneoplastic syndromes in thymoma patients including myasthenia gravis (63%), pure red cell aplasia (7.7%) and hypogammaglobulinemia referred to as Good's syndrome (6%) [16]. ...
We report a case of pure white cell aplasia (PWCA) postthymoma resection in a 74-year-old male presenting with a 2-week history of fevers, night sweats, and severe febrile neutropenia. His pure white cell aplasia was treated with intravenous immunoglobulin (IVIg), granulocyte colony-stimulating factor (G-CSF), prednisone, and cyclosporine with a mixed response. He also developed immune thrombocytopenia, which responded well to a short course of eltrombopag. With continued cyclosporine treatment, his platelet counts were stable after stopping eltrombopag. The patient's cyclosporine treatment was complicated by renal failure, resulting in cessation of cyclosporine. His PWCA and immune thrombocytopenia significantly worsened after stopping cyclosporine, and unfortunately, he died from multiorgan failure and sepsis.
... Thymic epithelial tumours (TETs) arise from the thymus and represent approximately 0.2-1.5% of all malignancies [1][2][3]. The World Health Organization's histological classification is based on morphology, atypia grade and lymphocytic presence [4,5], and represents an independent prognostic factor for survival in these patients [6,7]. ...
Background:
The use of 18F FDG PET/CT scan in thymic epithelial tumours (TET) has been reported in the last two decades, but its application in different clinical settings has not been clearly defined.
Methods:
We performed a pictorial review of pertinent literature to describe different roles and applications of this imaging tool to manage TET patients. Finally, we summarized future prospects and potential innovative applications of PET in these neoplasms.
Results:
18FFDG PET/CT scan may be of help to distinguish thymic hyperplasia from thymic epithelial tumours but evidences are almost weak. On the contrary, this imaging tool seems to be very performant to predict the grade of malignancy, to a lesser extent pathological response after induction therapy, Masaoka Koga stage of disease and long-term prognosis. Several other radiotracers have some application in TETs but results are limited and almost controversial. Finally, the future of PET/CT and theranostics in TETs is still to be defined but more detailed analysis of metabolic data (such as texture analysis applied on thymic neoplasms), along with promising preclinical and clinical results from new "stromal PET tracers", leave us an increasingly optimistic outlook.
Conclusions:
PET plays different roles in the management of thymic epithelial tumours, and its applications may be of help for physicians in different clinical settings.
... She was treated with surgical excision and responded well to chemotherapy post-operatively. Although her history of thymoma put her at an increased risk of myasthenia gravis [22], a test for muscle-speci c receptor tyrosine kinase antibodies and for acetylcholine receptor binding antibodies performed later at age 46 years was negative. ...
Background
Valosin containing protein (VCP) is an important protein with many vital functions mostly related to the ubiquitin-proteasome system that provides protein quality control. VCP-associated inclusion body myopathy with Paget disease of bone and frontotemporal dementia (IBMPFD), also termed VCP disease and multisystem proteinopathy (MSP 1), is an autosomal dominant disorder caused by variants in the VCP gene on human chromosome 9. VCP has also been strongly involved in cancer, with over-activity of VCP found in several cancers such as prostate, pancreatic, endometrial, esophageal cancers and osteosarcoma. Since MSP1 is caused by gain of function variants in the VCP gene, our hypothesis is that we would find an increased tendency of developing malignancies amongst our patients.
Results
Upon surveying 106 families with confirmed VCP variants, we found increased incidence of unusual tumors including malignant peripheral nerve sheath tumor, anaplastic pleomorphic xanthoastrocytoma and thymoma. Some of these cases developed cancer before displaying other classic VCP disease manifestations. We also present cases of common cancers; however, we did not find an increased incidence compared to the general population. This could be related to the early mortality associated with this disease.
Conclusion
This is the first study that expands the phenotype of VCP disease to potentially include unusual cancers and highlights the importance of further investigation of the role of VCP in cancer development. The results of this study in VCP disease patients suggests that patients may be at an increased risk for unusual tumors.
... As an exceptionally rare pediatric diagnosis, thymoma of the anterior mediastinum presents a challenging diagnosis in children, with only 23 pediatric cases identified by a pediatric tumor surveillance registry between 1973 and 2008 [1][2][3][4]. Though often incidentally found, children can present with non-specific upper respiratory symptoms or chest pain, dyspnea from chest pain, and dyspnea secondary to mass effect on mediastinal structures. ...
... Though often incidentally found, children can present with non-specific upper respiratory symptoms or chest pain, dyspnea from chest pain, and dyspnea secondary to mass effect on mediastinal structures. As the majority of thymic epithelial neoplasms (TENs) is present in adulthood between the fourth and fifth decades, there are limited data on the treatment and outcomes in the pediatric population [3]. The presence of symptoms, paraneoplastic or autoimmune syndromes, and age less than 10 at the time of diagnosis are associated with poor outcomes and an increased association with malignancy [3][4][5]. ...
... As the majority of thymic epithelial neoplasms (TENs) is present in adulthood between the fourth and fifth decades, there are limited data on the treatment and outcomes in the pediatric population [3]. The presence of symptoms, paraneoplastic or autoimmune syndromes, and age less than 10 at the time of diagnosis are associated with poor outcomes and an increased association with malignancy [3][4][5]. We present a rare case of an advanced pediatric TEN Masaoka stage III World Health Organization (WHO) type B3 in a 16-year-old female. ...
Thymoma is a very rare pediatric tumor, accounting for less than 1% of all childhood mediastinal tumors with scant literature, and only 23 pediatric cases were identified by a pediatric tumor surveillance registry between 1973 and 2008. In contrast to adult thymomas, pediatric thymomas have an aggressive tendency, though the majority is discovered as incidental findings. Patient presentations to the emergency department (ED) are often subtle and non-specific such as dyspnea, cough, and chest pain, requiring a broad differential on the part of the emergency clinician. Because of this presentation, diagnosis often occurs later in the disease process when compared with adults. Chest radiograph may demonstrate an enlarged thymic shadow or cardiomegaly, necessitating further cardiac workup, commonly routed through cardiology. Computed tomography and biopsy are required for definitive diagnosis, requiring a multidisciplinary approach to management. We present a case of a 16-year-old female complaining of progressive dyspnea and chest pain over the course of one to two months with radiographic cardiomegaly. She was found to have a Masaoka stage III World Health Organization (WHO) type B3 thymic endothelial neoplasm and underwent surgical resection.
