Remission of Thymoma on Steroid Therapy in a Patient With Atypical Thymoma-Associated Multiorgan Autoimmunity: A Case Report and Literature Review.

Ewa Wrona,Marta Pastuszka,Sylwia Dębska-Szmich,Rafał Czyżykowski,Piotr Potemski,Marcin Braun

doi:10.3389/fimmu.2021.584703

Abstract

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.

Highlights

Thymoma is described as a rare neoplasm that constitutes roughly 0.2% to 1.5% of all malignancies, with an annual age-standardized incidence rate of 0.13 cases per 100 000 population [1]
The term ‘thymoma-associated multiorgan autoimmunity’ (TAMA) emerged in 2007 to describe patients with Graftversus-Host Disease–like (GvHD-like) dermatitis, enterocolitis or hepatitis with underlying thymoma and no prior haematopoietic stem cell (HSCT) nor organ transplantation [8]. This contrasted with the term ‘paraneoplastic autoimmune multiorgan syndrome’ (PAMS), which was proposed in 2001 for cases characterized by mucocutaneous lesions often extending to the aerodigestive tract with bronchial involvement, humoral autoimmunity against cellular adhesion molecules, poor response to treatment and high mortality rate [9, 10]
Twenty six patients presented with GvHD-like dermatitis, clinically manifested as erythematous scaly papules disseminated over the whole body (Table 1)

Summary

INTRODUCTION

Thymoma is described as a rare neoplasm that constitutes roughly 0.2% to 1.5% of all malignancies, with an annual age-standardized incidence rate of 0.13 cases per 100 000 population [1]. The term ‘thymoma-associated multiorgan autoimmunity’ (TAMA) emerged in 2007 to describe patients with Graftversus-Host Disease–like (GvHD-like) dermatitis, enterocolitis or hepatitis with underlying thymoma and no prior haematopoietic stem cell (HSCT) nor organ transplantation [8] This contrasted with the term ‘paraneoplastic autoimmune multiorgan syndrome’ (PAMS), which was proposed in 2001 for cases characterized by mucocutaneous lesions often extending to the aerodigestive tract with bronchial involvement, humoral autoimmunity against cellular adhesion molecules, poor response to treatment and high mortality rate [9, 10]. Detailed timeline on the patient’s diagnostic process and treatment was presented in the Supplementary Figure 1

DISCUSSION

38 Female

15 Early 50s

Findings

ETHICS STATEMENT