Thymic carcinoma associated with cerebellar degeneration

Cuartero, Irene García; Galdeano, Mónica Rodríguez; Pinar, Montserrat Pérez; Pozo, Julián Solís García del

doi:10.12890/2018_000780

Cited by 2 publications

(2 citation statements)

References 7 publications

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“…Lung, breast, lymphoma, gastronintestinal, ovary cancer (85) 9 patients with PCD and SCLC (65) one thymic carcinoma (86) 39 cases with lymphoma, non-SCLC and genitourinary cancers (87) Collectively, these findings suggest that defects in immune tolerance induction could be implicated in the pathogenesis of PCD. Central tolerance is only partial since lymphocytes capable of recognizing autoantigens are prevalent, even in healthy individuals (115).…”

Section: Immune Tolerance and Pcdmentioning

confidence: 97%

Immunological Bases of Paraneoplastic Cerebellar Degeneration and Therapeutic Implications

2020

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Paraneoplastic cerebellar degeneration (PCD) is a rare immune-mediated disease that develops mostly in the setting of neoplasia and offers a unique prospect to explore the interplay between tumor immunity and autoimmunity. In PCD, the deleterious adaptive immune response targets self-antigens aberrantly expressed by tumor cells, mostly gynecological cancers, and physiologically expressed by the Purkinje neurons of the cerebellum. Highly specific anti-neuronal antibodies in the serum and cerebrospinal fluid represent key diagnostic biomarkers of PCD. Some anti-neuronal antibodies such as anti-Yo autoantibodies (recognizing the CDR2/CDR2L proteins) are only associated with PCD. Other anti-neuronal antibodies, such as anti-Hu, anti-Ri, and anti-Ma2, are detected in patients with PCD or other types of paraneoplastic neurological manifestations. Importantly, these autoantibodies cannot transfer disease and evidence for a pathogenic role of autoreactive T cells is accumulating. However, the precise mechanisms responsible for disruption of self-tolerance to neuronal self-antigens in the cancer setting and the pathways involved in pathogenesis within the cerebellum remain to be fully deciphered. Although the occurrence of PCD is rare, the risk for such severe complication may increase with wider use of cancer immunotherapy, notably immune checkpoint blockade. Here, we review recent literature pertaining to the pathophysiology of PCD and propose an immune scheme underlying this disabling disease. Additionally, based on observations from patients' samples and on the pre-clinical model we recently developed, we discuss potential therapeutic strategies that could blunt this cerebellum-specific autoimmune disease.

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Section: Immune Tolerance and Pcdmentioning

confidence: 97%

Immunological Bases of Paraneoplastic Cerebellar Degeneration and Therapeutic Implications

2020

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“…dermatomyositis ( 6 , 7 ), skleromyxedema ( 8 ), and Leser-Trélat sign ( 9 )] and neurological diseases [e.g. cerebellar degeneration ( 10 ) and myasthenia gravis ( 11 )]. Although dermatologic paraneoplastic syndromes, especially dermatomyositis, seem to occur, no cases of paraneoplastic psoriasis have been reported in patients with thymic carcinoma.…”

Section: Introductionmentioning

confidence: 99%

Case Report: Paraneoplastic psoriasis in thymic carcinoma

Mix,

Knoll,

Häring

et al. 2023

Front. Oncol.

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Thymic carcinomas are exceedingly rare and very aggressive malignancies of the anterior mediastinum. While thymomas exhibit a high association with paraneoplastic syndromes, these phenomena are a rarity in thymic carcinomas. In general, acanthotic syndromes such as acroceratosis neoplastica and acanthosis nigricans maligna are commonly observed as paraneoplastic phenomena in patients with carcinomas. In contrast, psoriasis vulgaris, another acanthotic disease, rarely occurs as a paraneoplasia. We report the case of a 36-year-old patient with progressive thymic carcinoma (undifferentiated carcinoma, T3N2M1a) and paraneoplastic psoriasis occurring ten months before the initial diagnosis of the carcinoma. Over the course of the disease, new psoriatic flares heralded relapse or progression of the carcinoma. To our knowledge, this is the first reported case of paraneoplastic psoriasis in thymic carcinoma.

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Thymic carcinoma associated with cerebellar degeneration

Cited by 2 publications

References 7 publications

Immunological Bases of Paraneoplastic Cerebellar Degeneration and Therapeutic Implications

Immunological Bases of Paraneoplastic Cerebellar Degeneration and Therapeutic Implications

Case Report: Paraneoplastic psoriasis in thymic carcinoma

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