Distribution of Mediastinal Lesions Across Multi-Institutional, International, Radiology Databases

Roden, Anja C.; Fang, Wentao; Shen, Yan; Carter, Brett W.; White, Darin; Jenkins, Sarah M.; Spears, Grant M.; Molina, Julian R.; Klang, Eyal; Segni, Mattia Di; Ackman, Jeanne B.; Sánchez, Elsa Yolanda; Girard, Nicolas; Shumeri, Engjellush; Revel, Marie; Chassagnon, Guillaume; Rubinowitz, Ami N.; Dicks, Demetrius; Detterbeck, Frank C.; Ko, Jane P.; Falkson, Conrad; Sigurdson, Samantha; Segreto, Sabrina; Vecchio, Silvana Del; Palmieri, G.; Ottaviano, Margaret; Marino, Mirella; Korst, Robert J.; Marom, Edith M.

doi:10.1016/j.jtho.2019.12.108

Cited by 64 publications

(64 citation statements)

References 22 publications

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“…Less common recurrent alterations concern gain-of-function mutations of HRAS (mainly in type A and AB thymomas) and NRAS (in type A and B thymomas), and loss-of-function mutations of TP53 (in type B thymomas and TCs). The enrichment of C>T mutations within CpG di-nucleotides is an age-related signature [41] that fits well with the age of thymoma patients [42]. KIT mutations and oncogenic driver mutations or translocations that are characteristic of lung and other cancers have not been observed.…”

Section: Genetic Alterations In Treatment Naïve Common Thymoma Typessupporting

confidence: 54%

Molecular pathology of thymomas: implications for diagnosis and therapy

et al. 2021

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Thymomas exhibit a unique genomic landscape, comprising the lowest on average total mutational burden among adult human cancers; a unique point mutation in the GTF2I gene in WHO type A and AB thymomas (and rarely others); almost unique KMT2A-MAML2 translocations in rare WHO type B2 and B3 thymomas; a unique YAP1-MAML2 translocation in almost all metaplastic thymomas; and unique miRNA profiles in relation to GTF2I mutational status and WHO histotypes. While most thymomas can be diagnosed solely on the basis of morphological features, mutational analyses can solve challenging differential diagnostic problems. No molecular biomarkers have been identified that predict the response of unresectable thymomas to chemotherapy or agents with known molecular targets. Despite the common and strong expression of PDL1 in thymomas, immune checkpoint inhibitors are rarely applicable due to the poor predictability of common, life-threatening autoimmune side effects that are related to the unrivaled propensity of thymomas towards autoimmunity.

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Section: Genetic Alterations In Treatment Naïve Common Thymoma Typessupporting

confidence: 54%

Molecular pathology of thymomas: implications for diagnosis and therapy

et al. 2021

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“…TCs are rare neoplasms with an incidence of 0.07-0.38/100,000/year [27]. They mainly occur in the prevascular mediastinum comprising 7.5% of the lesions at this site [3], and mainly affect males in the fifth-sixth decade [28,29]. The neoplastic mass can cause symptoms (such as dyspnea, chest pain, or superior vena cava syndrome) related to its growth and the compression of the surrounding anatomical structures.…”

Section: Thymic Carcinomamentioning

confidence: 99%

“…Indeed, the surgical pathology of the thymus encompasses a heterogeneous group of rare entities [2] which almost exclusively arise in the prevascular mediastinum with the exception of ectopic thymic tissue lesions. The most frequent thymus tumors in the adult population are represented by epithelial tumors, the thymomas [3], with an incidence of about 2 cases per 1,000,000 per year [4,5]. Since the first description of the association between myasthenia gravis (MG) and thymomas in the early 1900s [6], the study of thymic pathology has generated great scientific interest by several groups both in understanding the development of these rare neoplasms and especially in improving lesion classification.…”

Section: Introductionmentioning

confidence: 99%

The Rarest of Rare Thymic Lesions: A 10-Year Surgical Pathology Experience

Calabrese

Fortarezza

Pezzuto

et al. 2021

Cancers

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The thymus is a specialized primary lymphoid organ located in the midline pre-vascular mediastinum. The organ is the site of various pathological processes, neoplastic and not, whose rarity has not allowed in-depth studies on clinical or histological features of rarest and unusual variants. Herein, we report a 10-year Padova experience in the surgical pathology of the thymus, focusing on the pathological description of nonneoplastic lesions and rare epithelial and mesenchymal tumors recorded in our database, which comprises over 600 thymectomies. The extrapolated rare cases have been categorized into four groups that included 15 cysts, 18 carcinomas, 5 neuroendocrine tumors, and 2 soft tissue tumors. The cases are described from a clinical and pathological point of view and discussed in dedicated sections with a review of the most important literature. In this case, review series, we aim to update the epidemiology of these rare entities, improve diagnostic awareness, and finally, promote a collaborative network between referral centers.

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“…Thymic epithelial tumors (thymomas and thymic carcinomas) and lymphomas represent the two most frequent types of mediastinal neoplasms in adults [1]. Non-neoplastic mediastinal masses mainly consist of cysts and several types of thymic hyperplasia: (i) true thymic hyperplasia of unknown etiology that shows normal-looking histology and a thymus weight that mostly exceeds 100 g, (ii) rebound hyperplasia that follows the cessation of various "stressors" such as infection or chemotherapy and shows normal-forage or juvenile histology and organ weights usually below 100 g, (iii) diseases with the unifying histological hallmark of lymphofollicular hyperplasia.…”

Section: Introductionmentioning

confidence: 99%

Thymic Hyperplasia with Lymphoepithelial Sialadenitis (LESA)-Like Features: Strong Association with Lymphomas and Non-Myasthenic Autoimmune Diseases

Porubský

Popović

Badve

et al. 2021

Cancers

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Thymic hyperplasia (TH) with lymphoepithelial sialadenitis (LESA)-like features (LESA-like TH) has been described as a tumor-like, benign proliferation of thymic epithelial cells and lymphoid follicles. We aimed to determine the frequency of lymphoma and autoimmunity in LESA-like TH and performed retrospective analysis of cases with LESA-like TH and/or thymic MALT-lymphoma. Among 36 patients (21 males) with LESA-like TH (age 52 years, 32–80; lesion diameter 7.0 cm, 1–14.5; median, range), five (14%) showed associated lymphomas, including four (11%) thymic MALT lymphomas and one (3%) diffuse large B-cell lymphoma. One additional case showed a clonal B-cell-receptor rearrangement without evidence of lymphoma. Twelve (33%) patients (7 women) suffered from partially overlapping autoimmune diseases: systemic lupus erythematosus (n = 4, 11%), rheumatoid arthritis (n = 3, 8%), myasthenia gravis (n = 2, 6%), asthma (n = 2, 6%), scleroderma, Sjögren syndrome, pure red cell aplasia, Grave’s disease and anti-IgLON5 syndrome (each n = 1, 3%). Among 11 primary thymic MALT lymphomas, remnants of LESA-like TH were found in two cases (18%). In summary, LESA-like TH shows a striking association with autoimmunity and predisposes to lymphomas. Thus, a hematologic and rheumatologic workup should become standard in patients diagnosed with LESA-like TH. Radiologists and clinicians should be aware of LESA-like TH as a differential diagnosis for mediastinal mass lesions in patients with autoimmune diseases.

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Distribution of Mediastinal Lesions Across Multi-Institutional, International, Radiology Databases

Cited by 64 publications

References 22 publications

Molecular pathology of thymomas: implications for diagnosis and therapy

Molecular pathology of thymomas: implications for diagnosis and therapy

The Rarest of Rare Thymic Lesions: A 10-Year Surgical Pathology Experience

Thymic Hyperplasia with Lymphoepithelial Sialadenitis (LESA)-Like Features: Strong Association with Lymphomas and Non-Myasthenic Autoimmune Diseases

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