Thymoma-associated multiorgan autoimmunity

Hung, Chien-Tzu; Tsai, Tsen-Fang; Chen, Jin‐Shing; Hsieh, Min‐Shu

doi:10.1136/bcr-2018-229163

Cited by 22 publications

(16 citation statements)

References 9 publications

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“…This T cell mediated disorder is histopathologically akin to a graftversus-host disease pattern (158). Literature provides some rather complex case reports (159)(160)(161).…”

Section: Thymoma-associated Multiorgan Autoimmunitymentioning

confidence: 99%

Autoimmune disorders and paraneoplastic syndromes in thymoma

Blum¹,

Misch²,

Kollmeier³

et al. 2020

J Thorac Dis

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Thymomas are counted among the rare tumour entities which are associated with autoimmune disorders (AIDs) and paraneoplastic syndromes (PNS) far more often than other malignancies. Through its complex immunological function in the context of the selection and maturation of T cells, the thymus is at the same time highly susceptible to disruptive factors caused by the development and growth of thymic tumours. These T cells, which are thought to develop to competent immune cells in the thymus, can instead adopt autoreactive behaviour due to the uncontrolled interplay of thymomas and become the trigger for AID or PNS affecting numerous organs and tissues within the human body. While myasthenia gravis is the most prevalent PNS in thymoma, numerous others have been described, be they related to neurological, cardiovascular, gastrointestinal, haematological, dermatological, endocrine or systemic disorders. This review article sheds light on the pathophysiology, epidemiology, specific clinical features and therapeutic options of the various forms as well as courses and outcomes of AID/PNS in association with thymomas.Whenever suitable and backed by the limited available evidence, the perspectives from both the thymoma and the affected organ/tissue will be highlighted. Specific issues addressed are the prognostic significance of thymectomy on myasthenia gravis and other thymoma-associated AID/PND and further the impact and safety of immunotherapies on AID and PND relating to thymomas.

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“…This T cell mediated disorder is histopathologically akin to a graftversus-host disease pattern (158). Literature provides some rather complex case reports (159)(160)(161).…”

Section: Thymoma-associated Multiorgan Autoimmunitymentioning

confidence: 99%

Autoimmune disorders and paraneoplastic syndromes in thymoma

Blum¹,

Misch²,

Kollmeier³

et al. 2020

J Thorac Dis

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show abstract

“…Another possible rare presentation of thymoma is a type of paraneoplastic syndrome that resembles Graft-versus-host disease (GvHD). This syndrome has also been described as "thymoma associated multiorgan autoimmunity" (TAMA) and involves the skin, liver and intestines (20,21). Typical clinical manifestations of TAMA include confluent keratotic papules, scaly plaques, morbilliform eruptions, erythroderma and mucosal erosions, diarrhea and abnormal liver enzyme levels, especially alkaline phosphatase.…”

Section: Paraneoplastic Disordersmentioning

confidence: 99%

When to suspect a thymoma: clinical point of view

Minervini¹,

Kocher²

2020

J Thorac Dis

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The thymus plays a crucial role in the development of immune system, regulating the maturation, selection and migration of T lymphocytes. Alterations in lymphatic content and structure of the thymus are observed in many autoimmune diseases. Moreover, changes of the epithelial component may cause the development of thymic tumours. Thymoma is a rare epithelial tumor of the anterior mediastinal compartment with a wide spectrum of clinical presentations. The causes of thymoma are still unknown and several hypotheses have been formulated. Thymomas show a variable course causing, frequently, a prolonged clinical history. The presence of metastasis at the time of diagnosis is very uncommon. Even if about 30% of the patients with thymoma are asymptomatic, they may have local symptoms (such as cough, pain, hoarseness, and dyspnea) or paraneoplastic disorders. The role of immune system in the pathogenesis of these tumors and related paraneoplastic syndromes is not completely clear. A clinical diagnosis, especially if the first manifestation is a thymoma-associated paraneoplastic disease, is not always easy and should be supported by an appropriate imaging in order to guide the proper management for each patient. In this article, we would like to give an overview of the local and systemic clinical manifestations, which can be found in patients affected by thymoma.

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“…Thymus is a primary lymphoid organ that is only functional in the early stages of life. Thymomas, however, can potentially activate a previously dormant thymus and may be responsible for the appearance of a great variety of paraneoplastic autoimmune manifestations 1 . Thymoma‐associated autoimmune disturbances caused by a predominantly cellular immune mechanism may simulate lesion patterns observed in cutaneous, hepatic, and intestinal graft versus host disease (GVHD) 1 .…”

Section: Figurementioning

confidence: 99%

“…Thymomas, however, can potentially activate a previously dormant thymus and may be responsible for the appearance of a great variety of paraneoplastic autoimmune manifestations 1 . Thymoma‐associated autoimmune disturbances caused by a predominantly cellular immune mechanism may simulate lesion patterns observed in cutaneous, hepatic, and intestinal graft versus host disease (GVHD) 1 . The broad spectrum of cellular autoimmunity phenomena associated with thymoma has been encompassed under the name “thymoma‐associated multiorgan autoimmunity” (TAMA) 2 …”

Section: Figurementioning

confidence: 99%

“…1 Thymoma-associated autoimmune disturbances caused by a predominantly cellular immune mechanism may simulate lesion patterns observed in cutaneous, hepatic, and intestinal graft versus host disease (GVHD). 1 The broad spectrum of cellular autoimmunity phenomena associated with thymoma has been encompassed under the name "thymoma-associated multiorgan autoimmunity" (TAMA). 2 A 50-year-old male with history of a thymoma Masaoka stage B2 and WHO stage IV, and bone marrow aplasia, presented with multiple pruritic papulosquamous lichenoid lesions affecting large areas of the trunk and superior extremities ( Fig.…”

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confidence: 99%

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