Remission of Thymoma on Steroid Therapy in a Patient With Atypical Thymoma-Associated Multiorgan Autoimmunity: A Case Report and Literature Review
| Autor: | Sylwia Dębska-Szmich, Rafał Czyżykowski, Ewa Wrona, Marta Pastuszka, Marcin Braun, Piotr Potemski |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
medicine.medical_specialty
Thymoma Paraneoplastic Syndromes thymoma-associated multiorgan autoimmunity (TAMA) Immunology Mucocutaneous zone Autoimmunity Case Report medicine.disease_cause 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine Adrenal Cortex Hormones Atypical Thymoma hemic and lymphatic diseases Myasthenia Gravis medicine Mucositis Humans Immunology and Allergy Neoplasm remission on steroids Aged business.industry Remission Induction Thymus Neoplasms RC581-607 thymoma mucocutaneous lesions medicine.disease Dermatology Myasthenia gravis 030220 oncology & carcinogenesis Female Immunologic diseases. Allergy Differential diagnosis business paraneoplastic autoimmune multiorgan syndrome (PAMS) |
| Zdroj: | Frontiers in Immunology Frontiers in Immunology, Vol 12 (2021) |
| ISSN: | 1664-3224 |
| DOI: | 10.3389/fimmu.2021.584703 |
| Popis: | In up to 34% of cases, thymoma, itself a rare neoplasm, is accompanied by autoimmune disorders, two of which are thymoma-associated multiorgan autoimmunity (TAMA) and paraneoplastic autoimmune multiorgan syndrome (PAMS). Unfortunately, differential diagnosis between these two entities can be challenging since no strict PAMS definition exists and PAMS can overlap with a subgroup of TAMA patients with skin lesions as leading presentation. We present a case of a 68-year-old woman with a diagnosis of thymoma accompanied by myasthenia gravis, hypothyroidism and GvHD-like mucocutaneous lesions that initially could account to both TAMA and PAMS diagnosis. However, following the exclusion of humoral autoimmunity against components of epithelial cells junction, TAMA was finally established. Interestingly, the introduction of corticosteroid therapy for TAMA symptom management resulted in unexpected partial remission of thymoma with no impact on mucocutaneous lesions. Our case study is an example of two extremely rare phenomena accompanying thymomas: unprecedented TAMA presentation with GvHD-like mucositis, which as we postulate should be placed in the spectrum of TAMA, and tumor remission on steroids. |
| Databáze: | OpenAIRE |
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